Congenital Heart Desease

Congenital
Heart Disease
Morphological and
Functional Assessment
Hideaki Senzaki
Satoshi Yasukochi
Editors
123
Congenital Heart Disease
Hideaki Senzaki Satoshi Yasukochi

Editors
Congenital Heart Disease

Morphological and Functional Assessment
Editors
Hideaki Senzaki
Department of Pediatric Cardiology
Saitama Medical Center
Saitama Medical University
Kawagoe, Japan
Satoshi Yasukochi
Heart Center/Pediatric Cardiology
Nagano Childrens Hospital
Azumino, Japan
ISBN 978-4-431-54354-1
ISBN 978-4-431-54355-8 (eBook)
DOI 10.1007/978-4-431-54355-8
Springer Tokyo Heidelberg New York Dordrecht London
Library of Congress Control Number: 2014958569
Springer Japan 2015
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Preface
Anatomical or morphological abnormalities in congenital heart disease (CHD) are

generally accompanied by abnormal loading conditions, which in turn cause functional ventricular and vascular impairments that may also exist independently of
anatomical abnormalities, however. Interactions between morphological and functional abnormalities are important determinants of underlying pathophysiology and
cause clinical symptoms in CHD. Therefore, detailed and precise assessment of
morphology and function is essential to better understand and treat this disease.
Recent advances in technology have provided useful tools for this purpose, and
novel ndings are accumulating.
This book, entitled Congenital Heart Disease: Morphological and Functional
Assessment, comprehensively covers the latest information about ventricular
vascular morphology and function in CHD, as evaluated by numerous innovative
methodologies, including echocardiography, magnetic resonance imaging (MRI),
computed tomography (CT), and cardiac catheterization.
In Chaps. 14 (Part I), the concept of morphological assessment by threedimensional echocardiography, MRI, and high-resolution multi-slice CT scanning
is introduced by world-leading Japanese experts. Chapters 1 and 2 describe echocardiographic assessment, using various different approaches, of intra-cardiac anatomy, with particular focus on valvular anatomy, in detail. Three-dimensional CT
and MRI are highly useful tools for assessing extra- and intra-cardiac structures and
their anatomical relationships, as outlined in Chaps. 3 and 4. In Chaps. 58 (Part II),
new frontiers in the assessment of cardiovascular function in CHD are presented by
world-renowned experts in the eld. While cardiac catheterization provides detailed
information about cardiovascular function based on pressure measurements, as
discussed in Chaps. 5 and 6, echocardiography and MRI provide detailed owbased as well as myocardial motion-based information on cardiovascular function,
as described in Chaps. 7 and 8.
The information contained in each chapter will provide researchers and clinicians with invaluable knowledge of this eld, and should help to deepen their
understanding of CHD. It is my great honor to edit this book with my respected
v
vi
Preface
friend, Dr. Satoshi Yasukochi, and to invite world-leading Japanese pediatric

cardiologists as authors for each chapter.
Finally, as a token of affection and gratitude, I dedicate this book to my father,
Dr. Mamoru Senzaki, who died peacefully on April 6th, 2012, surrounded by his
family. Arigatou, Otousan.
Kawagoe, Japan
May 20, 2014
Hideaki Senzaki
Contents
Part I
1
Real-Time Three-Dimensional (3D) Echocardiography

for Diagnosis and Treatment of Congenital Heart Diseases
in Practical Medicine: Transepicardial and Transesophageal
Approach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Kiyohiro Takigiku
Assessment of Atrioventricular Valve Anatomy and Function
in Congenital Heart Diseases Using Three-Dimensional
Echocardiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Masaki Nii
21
Assessment of Intracardiac Anatomy by Magnetic

Resonance Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Satoshi Yasukochi
43
Assessment of Extracardiac and Intracardiac Anatomy

by MD-CT . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Kenji Waki
71
Part II
5
Morphological Assessment of Congenital Heart Disease
Functional Assessment of Congenital Heart Disease
Assessment of Ventricular Function Using the Pressure-Volume

Relationship . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Satoshi Masutani and Hideaki Senzaki
97
Assessment of Vascular Function by Using Cardiac

Catheterization . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127
Hirofumi Saiki and Hideaki Senzaki
vii
viii
Contents
Assessment of Ventricular-Vascular Function

by Echocardiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 143
Manatomo Toyono
Assessment of Hemodynamics by Magnetic

Resonance Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 177
Masaya Sugimoto
Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 205
Part I
Morphological Assessment of Congenital

Heart Disease
Chapter 1
Real-Time Three-Dimensional
(3D) Echocardiography for Diagnosis
and Treatment of Congenital Heart Diseases
in Practical Medicine: Transepicardial
and Transesophageal Approach
Kiyohiro Takigiku
Abstract How to use real-time three-dimensional (3D) echocardiography for

diagnosis and treatment of congenital heart diseases? To obtain morphological
details of complicated intracardiac structure is one of the most effective usages,
when planning for cardiac surgery, such as an intracardiac rerouting through
ventricular septal defect in patients with double-outlet right ventricle, valvuloplasty
for complicated atrioventricular valve regurgitation, or release for intracardiac
stenotic lesions. Especially, intraoperative transpericardial 3D echocardiography
performed by putting the 3D probe on the pericardium directly enables us to get the
good quality images with a high S/N ratio in pediatric patients less than 20 kg of
body weight, for whom transesophageal 3D echocardiography cannot be applied.
This approach also enables both cardiovascular surgeons and cardiologists to share
the surgeons view in the operating room quickly. Moreover, transesophageal 3D
echocardiography is also useful for the decision for the indication of catheter
intervention and monitoring the procedure of the percutaneous closure of atrial
septal defect, as well as to diagnose the abnormal morphology and guide the
intracardiac surgery in children more than 20 kg of body weight with congenital
heart disease.
Keywords Congenital heart disease Transesophageal 3D echocardiography
Transpericardial 3D echocardiography
K. Takigiku, MD, PhD (*)

Department of Pediatric Cardiology, Nagano Childrens Hospital, 3100 Toyoshina,
Azumino, Nagano 399-8288, Japan
e-mail: tackymr2@me.com
Springer Japan 2015
H. Senzaki, S. Yasukochi (eds.), Congenital Heart Disease,
DOI 10.1007/978-4-431-54355-8_1
1.1
K. Takigiku
Introduction
The ultrasonic diagnostic device, three-dimensional (3D) probe, and analysis workstation have developed their function, and then revolutionary advancement has
been achieved. The diagnostic device has a sophisticated image processing capability and the probe is equipped with functions such as the matrix array, multi-wave
transmission and reception, and multi-focusing, which enable a simple and sensitive 3D reconstruction. These are the major factors that helped the real-time 3D
echocardiography increase the use opportunities in clinical practice.
The 3D workstation echo scan in the 1990s used the following methods: the
ECG-gated multi-cross-sectional images were obtained rst, which were then
consolidated to reconstruct the volume data and divided into an optional cut plain
to visualize the intracardiac structure that was necessary for diagnosis [1]. Thus, it
took several tens of seconds to obtain the multi-cross-sectional images (Fig. 1.1).
Regarding children, moreover, it was problematic in terms of quality of images
even after collecting volume data over time and reconstructing based on them
because of their fast heat rate and difculty of breath-holding compared with adults.
In other words, the stitches caused by heartbeat synchronization and the gaps
caused by respiratory uctuation can affect the quality of the images signicantly.
In addition, enormous amount of time was necessary for off-line image analysis
(the right lower panel in Fig. 1.1). Therefore, 3D echocardiography was rarely used
for the diagnosis of a complicated form of congenital heart disease in the actual
clinical practice.
However, recently, high-resolution volume datasets have to be collected in any
direction from a single to a few heartbeat datasets, when using the real-time 3D
echocardiography. The development of the high-frequency 3D probe for children
and the improvements of image quality, even if, by using the low-frequency probe
have contributed to the issue. In addition, since the performance improvement of
the analysis workstation has enabled the volume data analysis in the extremely short
period of time, it has become possible to visualize the optimum cross section
as well.
The following are the good examples of clinical applications of the real-time
3DE for congenital heart diseases.
1. As the guide for surgical repair: Intracardiac route creation via ventricular septal
defect (VSD) with double-outlet right ventricle and valvuloplasty for the complicated atrioventricular valve insufciency
2. As the guide and monitoring for the percutaneous catheter closure of atrial septal
defect or ventricular septal defect
3. As the 3D functional analysis of volume and wall motion both in the left and
right ventricle and quantitative evaluation of the dynamic morphological of the
atrioventricular valve leaets in congenital heart disease
Once the methodology is established, when it comes to children whose echo
windows are easy to obtain and have relatively clear images, it is evident that the
1 Real-Time Three-Dimensional (3D) Echocardiography for Diagnosis . . .
Fig. 1.1 3D echocardiography by ECG-gated rotational device. The upper gures show how to
obtain the actual image by using ECG-gated rotational device. The probe is placed on the patient
from subxiphoid window. The lower right gure shows the 3D echocardiography in a case with
complete atrioventricular septal defect by the system that rotates by 2 s and collects the images of
every heartbeat to reconstruct. RA right atrium, RV right ventricle, ASD(I ) primum atrial septal
defect, CAVV common atrioventricular valve
real-time 3D echocardiography would become even more useful for the understanding of the complicated anatomical structure than the adults cardiovascular
diseases.
In this chapter, I would like to discuss mainly how to use the transpericardial
real-time 3D echocardiography as a guide for surgical repair in the actual clinical
practice. In addition, I also would like to outline the usage of the transesophageal
real-time 3D echocardiography as the guide and monitoring for the percutaneous
catheter closure.
1.2
3D Display of the Intracardiac Structure
Roll as a tool for detailed diagnosis and a guide for surgery.

Since 3D display of the intracardiac structure enables to understand the anatomically abnormal ndings, it is an extremely useful method for considering the
hemodynamic status and the operative procedure for intracardiac surgical repair
K. Takigiku
[2]. It can be utilized for various surgeries such as closure operation for the multiple
or complicated VSDs, creation of the intracardiac route via ventricular defect with
double-outlet right ventricle and transposition of the great arteries, release for
stenotic or obstructive lesions such as the left of right ventricular outow tract
and the pulmonary vein, and the atrioventricular valvuloplasty for congenital heart
disease. One major point of the 3D display as a guide of the congenital heart disease
is how to present it to surgeons. Creating the images from the surgeons standing
point, i.e., surgeons view, would serve as the base of communication between
cardiologists and cardiac surgeons and also help surgeons understand with ease.
1.2.1
Transpericardial 3D Echocardiography
Although transthoracic 3D echocardiography has a certain level of diagnostic

accuracy, we perform intraoperative transpericardial 3D echography with the aim
to construct the good quality of images that would have more diagnostic accuracy
and would be helpful for surgery [3]. In adult patients having severe mitral
regurgitation, it is difcult to perform the detailed guide for mitral valvuloplasty
by using transthoracic 3D echocardiography, while the transesophageal 3D echocardiography is better than that. Since the body weight of the most children with
congenital heart disease, who undergo intracardiac repair that needs a cardiopulmonary bypass, is less than 15 kg, it is impossible to insert the probe of the current
transesophageal 3D echocardiography. For the children for whom the
transesophageal probe cannot be used, the transpericardial approach is probably
the best 3D echocardiography currently because it produces best images and high
sensitivity. Specically, the images can be obtained by applying the 3D probe
directly on the pericardium or heart under thoracotomy. Volume data will be
obtained at the full-volume mode using 3D probe by temporarily shutting off the
articial respirator only when the breathing movement is inuential. Of course,
clearer images with higher resolution and better S/N ratio than transthoracic 3D
echocardiography will be obtained (Fig. 1.2).
1.2.2
Accommodation of Images
Collecting high-quality volume data is the key to obtain the 3D images with higher
quality. The rst step is to select the appropriate probe. For the children with body
weight less than 20 kg, it might be better to use a 3D probe with as high frequency
as possible (more than 7 MHz). For those with body more than that, a 3D probe with
5 MHz frequency should be used. First, capture the 2D images. Then, decide where
to put the center for collecting the 3D images. It is important to put the probe from
the window that can best visualize the target lesion. It might be better to conrm
whether the whole target sites are visualized properly by the biplane mode, the
Fig. 1.2 The actual procedure of 3D pericardial echocardiography volume dataset can be acquired
to put the 3D probe directly on the pericardium under thoracotomy, using 3D probe by temporarily
shutting off the articial respirator only when the breathing movement is inuential. Then good
quality of reconstructed images with higher resolution and better S/N ratio compared to the images
by transthoracic 3D echocardiography can be obtained
multi-slice mode, and tilting the probe. Adjustment such as gain and dynamic
range should be controlled on the 2D images. One of the keys to success is to put
the probe on the pericardium tightly.
1.2.3
Volume Data Collection
After adjusting the 2D images, start to work on the 3D image collection. It is

important to capture the images considering what kind of 3D images you would like
to compose at the end. For example, suppose that the disease is VSD. Figure 1.3 is
the schema to observe the interventricular septum that was cut out from the right
atrium and the right ventricular free wall at the frontal plane. Based on the
anatomical relationship among the defect, the tricuspid valve, the pulmonary
valve, and the aortic valve, it can be classied into perimembranous VSD, doubly
committed VSD, trabecular VSD, and inow septal VSD. If the 3D volume data is
cut similarly as in this gure, it would help to determine what kind of approach
would be appropriate to close the VSD, or whether it should be performed from the
pulmonary artery or the tricuspid valve, or under which leaet of tricuspid valve the
K. Takigiku
Subpulmonary
Doubly committed
Inflow septal
Outlet muscular
Perimembranous
Trabecular
Inlet muscular
Fig. 1.3 Type of the ventricular septal defects
Fig. 1.4 The comparison between the transpericardial 3D image with the ventricular septal defect
and the surgical nding. RA right atrium, PA pulmonary artery, RV right ventricle, VSD ventricular
septal defect
defect exists. It might be better to understand the anatomical knowledge and

representative operative procedures regarding the VSD before data acquisition.
Figure 1.4 shows the comparison between the transpericardial 3D image with the
VSD and the surgical nding. Similarly as in the surgical ndings, the defect is seen
in perimembranous portion, nearly the upper part of the septal leaet of the
tricuspid valve. In addition, the chorda tendinea of the tricuspid valve appears to
cross over the defect, which can be clearly conrmed by the 3D echocardiography.
To close the defect, it used to be necessary to make a resection avoiding the chorda
tendinea [3]. In the assessment of the double-outlet right ventricle and the VSD that
is porous and more complicated, the positional relationship among the pulmonary
valve, the aortic valve, the tricuspid valve, and the abnormal chorda tendinea in
addition to the size or the number becomes even more important in order to
determine the method of closing the defect and forming the intracardiac route.
Therefore, the volume data should be collected so that not only the VSD but also all
the surrounding large vessels and atrioventricular valves would be included. The
volume rate at the time of collecting should be over 40 Hz when the heart rate is
around 100 bpm. Since a wide angle becomes necessary due to the necessity of
including the surrounding structures, capturing in the full-volume mode integrated
with ECG-synchronized multiple slices (heartbeats) could maintain the beamline
density higher than capturing the single heartbeat with low volume rate.
1.2.4
Cropping
The next step is to create the images viewed from the surgeons position, so-called
surgeons view. To that end, it is necessary to understand the anatomical features of
the disease and representative operative procedures and keep in mind how to
proceed with the cropping to make the cut-plane. Here is an example of the actual
cropping case of the double-outlet right ventricle. Figure 1.5 shows a case with
VSD in double-outlet right ventricle. The 2D echography reveals that the aorta is
located in the right posterior and the pulmonary artery in the left anterior while the
VSD exists subpulmonary. In Fig. 1.6, the 3D image by cropping of the right
ventricular free wall of the transpericardial volume data in this case is visualized.
An abnormal muscle bundle that separates the large VSD into the right and left
halves exists from the center of the VSD to the right ventricular free wall. It was
diagnosed as the subpulmonary VSD and the subaortic VSD, so-called multiple
VSDs. Figure 1.6 is the view of the VSD from the pulmonary artery side, which is
the surgeons view of the opened pulmonary artery. The VSD under the aortic valve
is invisible due to the abnormal muscle bundle. The surgical ndings shown in
Fig. 1.7 are completely consistent with the preoperative echo ndings when the
pulmonary artery is opened. The VSD that is inserted with forceps was the one
under the pulmonary valve, while the other one is not accessible being blocked by
Fig. 1.5 2D echocardiography in a case with double-outlet right ventricle. Ao aorta, PA pulmonary artery, LA left atrium, RA right atrium, LV left ventricle, VSD ventricular septal defect
10
K. Takigiku
Fig. 1.6 3D transpericardial echocardiography in a case with double-outlet right ventricle. The
view of the ventricular septal defect above the pulmonary valve. Ao aorta, PA pulmonary artery,
TV tricuspid valve, LV left ventricle, VSD ventricular septal defect, IVS interventricular septum
Fig. 1.7 The surgical ndings in a case with double-outlet right ventricle. The relation between
subpulmonary VSD and muscle band is completely consistent with the preoperative echo ndings
in Fig. 1.6, when the pulmonary artery was open. PA pulmonary artery, VSD ventricular septal
defect
11
the muscle bundle as expected. The VSD under the aortic valve is approached from
the tricuspid valve side and a route is created from the left to the right ventricle. On
the other hand, patch closure is performed for the VSD under the pulmonary valve
from the pulmonary valve side. If the two VSDs have not been found at the
preoperative diagnosis, they could not have been closed completely and the patient
could not have been disconnected from the cardiopulmonary bypass. Thus, when
the VSD is multiple or the shapes of the defect and the surrounded structures are
complicated, anatomically detailed diagnosis by optimal cropping of the transpericardial 3D volume data would be very useful for the surgery practically.
1.2.5
Case Presentation
In this chapter, I would like to discuss some actual cases for which the transpericardium 3D displays were effective.
1.2.5.1
Postoperative Left Ventricular Outow Tract Stenosis
This is a case in which a left ventricular outow tract stenosis occurred after
intracardiac repair for the double-outlet right ventricle (Fig. 1.8). This 3D image
was the one that was cropped looking up the left ventricular outow tract from the
left ventricle side. The brous structure sticking out under the aortic valve from the
anterior mitral leaet side and the patch used to close the VSD (the highly bright
structure extending from the interventricular septum to the aortic valve) have
narrowed the subaortic site and formed a high degree of stenosis. In the surgery,
Fig. 1.8 Transepicardial 3D echocardiography in a case with left ventricular outow tract stenosis
after intracardiac repair of the double-outlet right ventricle. Ao aorta, MV mitral valve, TV tricuspid
valve, LV left ventricle, VSD ventricular septal defect
12
K. Takigiku
the brous structure was resected from the aorta side and the patch was removed
and reapplied in a boat-like shape to secure the wide outow tract again. Thus, not
necessarily only surgeons view but also the cross section observed from the angle
that is difcult to obtain from the usual 2D images can be utilized as a guide of
surgical procedures.
1.2.5.2
Atrioventricular Valvuloplasty
In children with atrioventricular valve disease, it is also very important for

valvuloplasty to analyze of the several mechanism of valvular regurgitation, by
using the 3D assessment/diagnosis, such as extension of the ap, tethering, contraction of the ap, extension and rupture of the chorda tendinea, and abnormal
adhesion of papillary muscle. Collecting volume data enables to cut the valve and
the valvular structure as one complex at an optimal cross section or continuous
cross sections, and then, the abnormal structure would be grasped accurately. It also
works as a guide for surgical repair to identify the abnormal area, the area of the tobe-resected valve, and the need of the articial chorda tendinea, lling of the valve
leaet, and adaptation of articial valve replacement surgery. Adult patients mainly
undergo the detailed diagnosis using transesophageal 3D echocardiography; however, morphological evaluation of the atrioventricular valve complicated with
congenital heart diseases using transpericardial 3D echography is very useful
because the valvuloplasty in most patients is performed in infancy, for whom
transesophageal 3D echocardiography cannot be applied, and moreover the quality
of the transthoracic 3D echocardiographic image is not yet satisfactory.
Figure 1.9 shows the transpericardial 2D echocardiographic image of congenital
mitral regurgitation while Fig. 1.10 shows the transpericardial 3D echocardiography. When being observed from the left atrial side, the anterior leaet is short and
Fig. 1.9 Transpericardial 2D echocardiography in congenital mitral regurgitation. Ao aorta, LA

left atrium, LV left ventricle
13
Fig. 1.10 Transpericardial 3D echocardiography in congenital mitral regurgitation
the coaptation with the posterior leaet is poor during the period from the time of
mitral valve closing to the end-systole. When cut in the sagittal direction at the short
anterior leaet, it is evident that almost no prolapse of the anterior leaet is observed
at the left atrial side, but a major gap is seen because the posterior leaet is tethered
and cannot be lifted up. Additionally, the anterior mitral leaet lacks support
because it is not connected with the chorda tendinea. In a case of a child who had
difculty during ring annuloplasty, valvuloplasty was performed successfully by
extending the anterior leaet by adding a glutaraldehyde-treated autopericardium to
the leaet and by attaching the articial chorda, because the anterior leaet cannot be
coapted well to the posterior leaet only using the articial chorda. Reconstructed
3D image as in Fig. 1.10 allows us to see the overall bad coaptation area, which
enables to infer the approximate extendable area of the anterior leaet.
Figure 1.11 shows a case of asplenia syndrome and single right ventricle that
presented with severe common atrioventricular valve regurgitation. When observed
from the atrium side, it is evident from the transpericardial 3D echocardiographic
images that the superior leaet and the inferior leaet among the four leaets of
common atrioventricular valve are small and the right and left leaets are large.
This corresponds with intraoperative ndings fairly well. When observed from
the ventricle side, regurgitation is developing from between the right lateral and the
superior leaet and between the right lateral and the inferior leaet, judging from
the location where aliasing of Doppler color ow imaging is occurring (Fig. 1.12).
14
K. Takigiku
Fig. 1.11 Asplenia syndrome and single right ventricle with severe common atrioventricular
valve regurgitation. The surgical ndings and transpericardial 3D echocardiography. CAVV
common atrioventricular valve
Fig. 1.12 Doppler color image of transpericardial 3D echocardiography. CAVV common atrioventricular valve
Since the body weight of this patient was also less than 10 kg, valvuloplasty was
performed to suture the upper and lower commissure of the right lateral leaet
without ring annuloplasty, because it was expected that the valve would not grow if
the articial ring was used and cause valve stenosis in the future.
1.2.5.3
Pulmonary Venous Obstruction
Figure 1.13 shows the transpericardial 2D echographic image of a case that

developed stenosis in the bilateral pulmonary veins after the intracardiac repair of
total anomalous pulmonary venous returns. In Fig. 1.14 the 3D echography
15
Fig. 1.13 Transpericardial 2D echography in a case that developed bilateral pulmonary venous
obstruction after the intracardiac repair of total anomalous pulmonary venous connection. rPV
right pulmonary vein, lPV left pulmonary vein
Fig. 1.14 Transpericardial 3D echography in the same case of Fig. 1.13. LA left atrium
revealed the stenotic pulmonary vein orice observed from the left atrial side.
When observed closely by 2D and 3D echocardiography, it was found that the
stenosis of the right and left pulmonary veins are formed by the surrounding brous
ridge and that the orice was slit-like especially in the left pulmonary vein. It was
also found out that the middle part between the brous ridges of the right and left
pulmonary vein ostium was bulging remarkably. The stenosis was released by
resecting that lesion.
16
1.2.5.4
K. Takigiku
Truncus Arteriosus
This is the transpericardial 3D echocardiographic image of truncus arteriosus.

Differentiation as to Collett-Edwards classication 1 or 2 was difcult to make
by transthoracic 2D (subcostal) echocardiography because the presence of the
central pulmonary artery could not be diagnosed (Fig. 1.15). As shown in
Fig. 1.16, transpericardial 3D echocardiography revealed clearly that the common
arterial trunk had four valves and the pulmonary arteries on both sides are originating from the common arterial trunk, which turned out to be Collet-Edwards
classication 2. A useful information for the plasty of pulmonary artery was
obtained.
Fig. 1.15 Transpericardial 2D echocardiography from subcostal view in a case with truncus
arteriosus. TrV truncal valve, LPA left pulmonary artery, RPA right pulmonary artery, RV right
ventricle
Fig. 1.16 Transpericardial 3D echocardiography from subcostal view in a case with truncus
arteriosus. TrV truncal valve, LPA left pulmonary artery, RPA right pulmonary artery
17
Fig. 1.17 Transpericardial 3D echocardiography of coronary arteries in transposition of the great

arteries. Ao aorta, LAD left ascendance branch, LCX left circumex branch, RCA right coronary
artery
1.2.5.5
Coronary Artery in Transposition of the Great Arteries
To diagnose for the morphology of coronary artery in transposition of the great

arteries, using transpericardial echocardiography is also easy. Figure 1.17 shows the
2D echocardiographic image of Shaher classication type I. The left coronary
artery is originating from the left coronary artery sinus 1 of the aortic valve and
the main stem divides into the anterior descending branch and the circumex
branch. The right coronary artery is also originating from sinus 2. The transpericardial 3D echocardiography revealed that the right and left coronary arteries are
originating from each Valsalva when observed from the inside, which helped to
construct exactly the same cross-sectional image of the aorta as seen from the
surgeons view. Transthoracic 3D echocardiography cannot visualize this much
clear and steric image of the coronary artery.
1.2.5.6
Transposition of the Great Arteries After Conotruncal Switch

Operation
This is a case of type III transposition of the great arteries (complicated with VSD
and PS) after conotruncal switch operation. Figure 1.18 shows the transpericardial
2D and 3D echocardiographic images. Conotruncal switch operation (Fig. 1.19) is a
surgery to rotate the conical portion of the aortic valve and the pulmonary valve
180 to make an arterial switch [4]. The transpericardial 3D echocardiographic
image can observe it toward the depth direction from above the aortic valve in
addition to the longitudinal direction and reveals that the outow tract was created
without any 3D torsion.
18
K. Takigiku
Fig. 1.18 Transpericardial 2D echocardiography with transposition of the great arteries after
conotruncal switch operation and surgical scheme. LV left ventricle, Ao aorta
Fig. 1.19 Transpericardial 3D echocardiography with transposition of the great arteries after
conotruncal switch operation. LV left ventricle, Ao aorta, LA left atrium
1.3
Decision of Indication of Catheter Intervention

for Congenital Heart Disease and Intraoperative Guide
Using Transesophageal 3D Echocardiograpy
Transesophageal 3D echocardiography can be used to make a detailed morphological diagnosis and as a guide for children with a body weight more than 20 kg as
similarly as the transpericardial echocardiography. In this chapter, I would like to
discuss the actual usage of transesophageal 3D echocardiography for atrial septal
defect closure by Amplatzer septal occluder (hereafter, ASO), one of the catheter
interventions for intracardiac structural abnormality of congenital heart disease.
Transesophageal 2D echocardiography and intracardiac echography are used as
a guide of ASO before/during operation; however, transesophageal 3D echocardiography can also be considered as one of the useful methods because of the increase
19
in the image quality. In a common ASO, transesophageal 2D echocardiography is

used to measure the size of the atrial septal defect and the distance between the
defect and the surrounding structures (superior vena cava, pulmonary vein, inferior
vena cava, coronary sinus, right pulmonary vein, atrioventricular valves, and
posterior wall), i.e., the length of the margin, which enables to determine the size
of the placeable device and the safety of device implantation. When the margin
except the aorta is shorter than 5 mm or its coverage is wider, it can be considered as
likely to induce erosion (cardiac perforation) or device migration and therefore the
device will not be placed sometimes. Transesophageal 3D echography can observe
ASD facing directly from the right atrium and the left atrial side and can grasp the
relationship with other structures in a short period of time. Theoretically, 2D
transesophageal echocardiography can measure neither the longest/shortest diameter of the atrial septal defect nor the shortest length of the margin from all the
structures correctly. Figure 1.20 is the 3D echocardiographic image of the atrial
septal defect observed from the right atrium side, which shows that the aortic
margin is short but the distance from the other structures is well over 5 mm.
Fig. 1.20 Transesophageal 3D echocardiography with atrial septal defect. The left lower panel
shows optimal cropped plane in which the accurate aortic rim with atrial septal defect is observed
clearly. ASD atrial septal defect, SVC superior vena cava, IVC inferior vena cava, Ao aorta
20
K. Takigiku
Fig. 1.21 Transesophageal 3D echocardiography during closure atrial septal defect by Amplatzer
septal occluder (ASO). Upper images showed the device implantation viewed from left atrium, as
well as lower images viewed from right atrium. ASD atrial septal defect, RA right atrium, LA left
atrium
Measuring an arbitrary cross section by cutting it out from the volume data enables
to measure the precise size and even determine the area of the shorter part of the
margin. This can be utilized as preoperative information in occlusion surgery for
selecting a device or a specic insertion method. It can be considered that combination usage with high-resolution 2D echography would inuence the decision on
the indication of ASO as well as on the success of implantation [5]. As is shown in
Fig. 1.21, which shows the serial images of the device motion and disc opening
during the actual implantation, it is evident that the device is being placed onto the
appropriate position of the septum.
References
1. Roelandt JRTC, Salustri A, Mumm B et al (1995) Precordial three-dimensional echocardiography with a rotational imaging probe: methods and initial clinical experience. Echocardiography 12:243252
2. Chen G, Huang G, Tao Z et al (2008) Value of real-time 3 dimensional echocardiography
sectional diagnosis in complex congenital heart disease evaluated by receiver operating characteristic analysis. J Am Soc Echocardiogr 21(5):458463
3. Kajimura I, Genngi S, Yasukochi S et al (2008) Pericardial 3D echocardiography. J
Echocardiogr 62:3945
4. Yamagishi M et al (2003) Half-turned truncal switch operation for complete transposition of the
great arteries with ventricular septal defect and pulmonary stenosis. J Thorac Cardiovasc Surg
125:966968
5. Simpson JM, Miller O et al (2011) Three-dimensional echocardiography in congenital heart
disease. Arch Cardiovasc Dis 104(1):4556
Chapter 2
Assessment of Atrioventricular Valve

Anatomy and Function in Congenital Heart
Diseases Using Three-Dimensional
Echocardiography
Masaki Nii
Abstract The function of the atrioventricular valve (AVV) complex is one of the
most important determinants of prognosis in patients with congenital heart disease.
However, the anatomy of the AVV complex is complicated, especially in patients
with congenital heart disease, which hampers precise preoperative assessment.
Moreover, AVV function is maintained by a very delicate balance of the forces
generated by the ventricle, atrium, papillary muscles, and blood ow. The AVV
leaet billows with the increment of the hydrostatic pressure in the ventricle and
closes by making coaptation with the adjacent leaets using this balance of force.
The shape and size of the annulus or position and function of the papillary muscles
are also very important factors in maintaining the effective coaptation of the leaets
with minimal stress on the leaet and chordae. If a congenital abnormality of the
leaet or valvular apparatus or an incorrect surgical repair causes an imbalance of
the forces at work in this delicate system, the result can be valve failure. Since the
advent of real-time three-dimensional echocardiography (3DE), we are able to
assess the precise anatomical and functional features of this complicated system.
Keywords Atrioventricular valve Congenital heart disease Mitral valve
Three-dimensional echocardiography Tricuspid valve
2.1
2.1.1
Anatomical Aspects of the Atrioventricular Valve

Development of the Atrioventricular Valve
During the embryonic development of the heart, the looping of the heart tube is
followed by the development of the atrioventricular valve (AVV) in the
M. Nii, MD, PhD (*)
Cardiac Department, Shizuoka Childrens Hospital, 860 Urushiyama, Aoi-ku,
Shizuoka 420-8660, Japan
e-mail: NII3MYSY@gmail.com
Springer Japan 2015
DOI 10.1007/978-4-431-54355-8_2
21
22
M. Nii
atrioventricular canal starting with the formation of endocardial cushions by an

epithelial to mesenchymal transformation. The proliferation of mesenchymal cells
at the base of the embryonic leaet contributes to its elongation. The patterning of
extracellular matrix proteins in the leaet is accomplished 1 week after birth, with
glycosaminoglycan and versican on the atrial side and collagen bers on the
ventricular side. Finally, the nodular thickening of the coaptation zone is formed
by the end of the second week after birth [1].
In the mature heart, the mitral valve (MV) leaet consists of four histological layers,
the atrialis, spongiosa, brosa, and ventricularis, ordered from the atrial aspect to the
ventricular side. The atrialis is the surface layer adjacent to the atrium, which is
composed of elastic and collagen bers covered with overlying endothelium. The
spongiosa largely consists of extracellular matrix, proteoglycans and glycosaminoglycans, and elastic bers. The spongiosa is the major component of the free edge and acts as
a cushion to protect the leaet from the impulse of valve closure. Beneath the spongiosa
is the brosa, which is comprised of collagen bers and is located close to the ventricular surface extending to the chordae tendineae. It works as the major load-bearing
layer during valve closure. The nal layer adjacent to the ventricle is the ventricularis,
which is covered by endothelial cells that overlie elastic and collagen bers [2].
2.1.2
Normal Anatomy of the Mitral Valve
Figure 2.1 shows a normal MV specimen from a neonate. The MV is comprised of two
leaets (the anterior and posterior leaets), the annulus, chordae tendineae, and
papillary muscles. The anterior leaet hangs like a curtain, dividing the inlet and
outlet portions of the left ventricle (Fig. 2.1). The anterior leaet (also called the aortic
leaet) occupies a third of the annular circumference and has a brous continuity with
the aortic valve. The right and left ends of this brous continuity are demarcated by the
right and left brous trigone. The right trigone together with the membranous septum
forms the central brous body. Although the annulus is recognized as the hinge line of
the leaet on an echocardiogram, from the pathological point of view, the distinctive
ringlike brous cord that supports the base of the leaets does not always surround the
entire area of the leaet base. Moreover, a well-formed brous cord is frequently
absent at the annulus opposite to the brous continuity [3, 4]. The posterior leaet (also
called the mural leaet) occupies two thirds of the annulus and has three or more
scallops, which are referred to as the anterolateral (P1), middle (P2), and
posteromedial sections (P3). Figure 2.2 shows an en face image of a normal MV
constructed by transesophageal 3DE. The three sections of the posterior leaet and
counterparts of the anterior leaet are well visualized by 3DE. The corresponding
sections of the anterior leaet are labeled A1, A2, and A3, respectively. The area of the
anterior leaet is slightly bigger than that of the posterior leaet, and the combined
surface area of the two leaets is twice that of the mitral annulus, which provides
sufcient area for the creation of a coaptation zone of appositional leaets.
2 Assessment of Atrioventricular Valve Anatomy and Function in Congenital. . .
23
Fig. 2.1 Normal mitral valve anatomy in a neonate. (a) Mitral valve. The posteromedial papillary
muscle usually consists of multiple papillary muscle bundles. On the other hand, the anterolateral
papillary muscle usually consists of a single muscle bundle. Note the prominent strut chordae from
the top of each papillary muscle inserting on the rough zone of the anterior leaet. Asterisks
indicate the left and right brous trigone. (b) Fibrous continuity of the mitral and aortic valve.
Arrows indicate brous continuity of the anterior mitral leaet and aortic valve. AL anterior leaet,
ALPM anterolateral papillary muscle, L left coronary cusp, LA left atrium, LT left trigone, LVOT
left ventricular outow tract, N noncoronary cusp, PFO patent foramen ovale, PL posterior leaet,
PMPM posteromedial papillary muscle, R right coronary cusp, S strut chordae
Fig. 2.2 An en face image of a normal mitral valve by three-dimensional echocardiography. The
left panel shows a closed mitral valve and the right panel shows an opened valve image. The three
scallops of the posterior leaet are referred to as the anterolateral (P1), middle (P2), and
posteromedial (P3) sections. The corresponding sections of the anterior leaet are labeled A1,
A2, and A3, respectively. AO aorta, CFB central brous body, MV mitral valve, TV tricuspid valve
24
M. Nii
The chordae tendineae are classied into three groups: (1) rst-order chordae
(also called marginal/free-edge chordae), which insert on the free edge of the
leaet, (2) second-order chordae (also called rough zone chordae) that insert on
the ventricular surface of the leaet beyond the free edge, forming the rough zone of
the leaet, and (3) third-order chordae (also called basal chordae), which are unique
to the posterior leaet and arise directly from the ventricular wall or from
trabeculations and insert on the basal zone of the posterior leaet. The two distinctive thick and strong second-order chordae of the anterior leaet are called strut
chordae and arise from the tip of the papillary muscle and insert on the rough zones
(Fig. 2.1). Because of their distinctive morphology of chordal branching that
resembles the ribs of a fan, the chordae that insert into the commissure are called
fan-shaped chordae (also called commissural chordae).
Two groups of papillary muscles are located beneath the commissures, occupying anterolateral and posteromedial positions. The anterolateral papillary muscle is
usually a single muscle bundle, while the posteromedial papillary muscle consists
of two or three papillary muscle bundles (Fig. 2.1). Both papillary muscles usually
have separate heads and the number and shape of the papillary muscle bundles vary
among individuals.
2.1.3
Normal Anatomy of the Tricuspid Valve
Figure 2.3 shows the normal tricuspid valve (TV) of a neonate, the same specimen
shown in Fig. 2.1. Although there are many variations to the normal morphology of
a TV, the TV is generally accepted to consist of three leaets: the anterior, septal,
and posterior leaets. Figure 2.4 shows an en face image of a normal TV
Fig. 2.3 Normal tricuspid
valve anatomy in a neonate.
The tricuspid valve consists
of three leaets. The septal
leaet is tethered by
chordae from the
ventricular septum and is
less mobile compared to the
other two leaets. AL
anterior leaet, APM
anterior papillary muscle,
CS crista supraventricularis,
MPM medial papillary
muscle, PAV pulmonary
artery valve, PL posterior
leaet, PPM posterior
papillary muscle, SL septal
leaet, TSM trabecula
septomarginalis
25
Fig. 2.4 An en face image of a normal tricuspid valve obtained using three-dimensional echocardiography. The left panel shows a closed tricuspid valve and the right panel shows an opened
valve image. The tricuspid valve consists of three leaets. AL anterior leaet, MV mitral valve, PL
posterior leaet, SL septal leaet, TV tricuspid valve
constructed by transesophageal 3DE. The leaet and chordae are thinner than those
of the MV. The anterior leaet (also called the superior leaet) is the largest of the
three leaets, is located in the anterosuperior position, and guards the orice of the
right ventricular outow tract. The septal leaet (also called the medial leaet) is
usually larger than the posterior leaet. Although most of the septal leaets basal
attachment is to the interventricular septum, its attachment sometimes extends to
the inferior wall. A small fold is frequently observed at the transition between the
septal and posterior leaets. The septal leaet has a chordal attachment to the
ventricular septum, which limits its mobility. These distinctive anatomical features
of the sepal leaet allow the TV to be distinguished from the MV. The posterior
leaet (also called the inferior leaet) is the smallest leaet and is located at the
inferior position. Compared to the MV, the leaet morphology of the TV is highly
variable, with many indentations of variable depth. The annulus of the TV usually
lacks a solid ringlike brous cord and is pathologically just a continuation of the
brous tissue of the leaet to the subendocardial ber. The annulus of the septal
leaet is especially indistinct because the anterior part of the leaet merges with the
membranous interventricular septum and is apically displaced from the atrioventricular junction. The commissure between the anterior and septal leaets
(anteroseptal commissure) is located at the most cranial position of the membranous septum and the fan-shaped chordae from the medial papillary muscle attach to
the septal and anterior leaets. The medial papillary muscle is on the bifurcation of
the anterior and posterior limbs of the trabecula septomarginalis or is sometimes
absent, in which case the chordae arise directly from the trabecula septomarginalis
or the crista supraventricularis. The commissure between the anterior and posterior
leaets (anteroposterior commissure) is located roughly at the acute margin of the
right ventricle, and beneath it is an anterior papillary muscle, which is the largest
papillary muscle, in the right ventricle and has a moderator band attached to its
26
M. Nii
base. The commissure between the posterior and septal leaets (posteroseptal
commissure) is located at the junction of the inferior and septal walls, and beneath
it is a posterior papillary muscle. The posterior papillary muscle is on the inferior
wall and at the most medial position. Its size varies considerably and is usually
small (Fig. 2.3) [5].
2.2
Anomalies of the Atrioventricular Valve
The etiologies of AVV disease are as follows: congenital, degenerative, inammatory, endocarditis, rheumatic, ischemic, cardiomyopathies, traumatic, and iatrogenic. These etiologies are associated with the anatomical abnormality or
malfunction of one or multiple components constituting the AVV complex, thereby
causing regurgitation or stenosis of the AVV. Carpentier et al. classied the
mechanisms of mitral regurgitation into three categories [7, 8]. This classication
is applicable to the TV or common AVV:
Type I: normal leaet motion (annular enlargement, leaet perforation, or cleft)
Type II: excessive leaet motion (ail leaet, ruptured chordae, prolapse, or
billowing)
Type III: restricted leaet motion:
(a) Short leaet or chordae
(b) Leaet tethering by the papillary muscle
In congenital AVV disease, the multiple pathologies outlined above usually
coincide and cause regurgitation and/or stenosis.
2.2.1
Mitral Valve Disease
The classication of congenital mitral valve anomalies is shown in Table 2.1 [6].
1. Isolated mitral valve cleft (Fig. 2.5): Figure 2.5 shows en face images of an
isolated MV cleft by transesophageal 3DE. This case was not associated with an
atrioventricular septal defect and the regurgitation was from a cleft. This anomaly was rst reported by Petitalot in 1987 [9], and the cleft is often oriented
towards the left ventricular outow tract rather than the ventricular septum, as is
usually seen in atrioventricular septal defects. However, in this particular
patient, the cleft points towards the ventricular septum.
2. Double orice mitral valve (Fig. 2.6): A double orice MV was rst described
by Greeneld in 1876 and is a rare AVV anomaly characterized by the presence
of two or more orices in the AVV leaet, each having an independent chordal
attachment to the papillary muscles [10]. A double orice MV rarely occurs as
27
Table 2.1 Congenital mitral valve anomalies

1. Anomalies of the leaet
2. Anomalies of the commissure

3. Anomalies of the chordae tendineae
4. Anomalies of the papillary muscle
5. The supramitral ring

6. Combination of the above anomalies
Cleft mitral valve

Double orice mitral valve
Excessive (accessory) mitral valve tissue
Ebsteins anomaly
Hypoplasia/dysplasia/atresia of leaet
Commissural fusion
Short chordae
Chordal elongation
Chordal rupture
Parachute mitral valve
Mitral arcade/hammock mitral valve
Obstruction by abnormal papillary muscle
Supramitral ring/membrane
Fig. 2.5 Isolated mitral valve cleft. En face images of a mitral valve at the closed (a) and opened
(b) positions obtained using transesophageal three-dimensional echocardiography. The arrow
indicates the mitral valve cleft. AO aorta
an isolated form and is usually associated with an atrioventricular septal defect,

ventricular septal defect, truncus arteriosus, pulmonary stenosis, coarctation or
interruption of the aortic arch, a bicuspid aortic valve, tetralogy of Fallot, or
Ebsteins anomaly. A partial atrioventricular septal defect is most commonly
seen, accounting for 41 % of associated anomalous lesions. Mitral regurgitation
is the most common functional abnormality (43 % of patients), followed by
mitral stenosis (13 %) and their combination (7 %). Of note, no functional
abnormality of the MV is observed in 37 % of patients [11].
28
M. Nii
Fig. 2.6 Double orice mitral valve. (a) A transthoracic two-dimensional echocardiography
image. (b) An en face image obtained using transesophageal three-dimensional echocardiography.
LVOT left ventricular outow tract
Fig. 2.7 Rheumatic mitral valve disease. Transthoracic three-dimensional images of a mitral
valve at the closed (a) and opened (b) positions. Note signicant commissural fusion (b)
3. Rheumatic mitral valve disease (Fig. 2.7): Figure 2.7 shows a transthoracic 3DE
image of a 12-year-old Afghan boy suffering from rheumatic fever with commissural fusion causing signicant MV stenosis. The rheumatic process causes
leaet thickening and fusion of commissures, resulting in limited leaet movement and a narrowing of the mitral orice. The chordae tendineae are also
involved in fusion, shortening, brosis, and calcication, leading to restricted
leaet movement, leaet malcoaptation, and regurgitation [12].
2.2.2
29
Tricuspid Valve Disease
TV anomalies are shown in Table 2.2 [13].

The functional abnormality of TV disease most commonly presents as tricuspid
regurgitation. Isolated TV stenosis is very rare and is observed in countries where
rheumatic heart disease is prevalent. Rather, TV stenosis commonly presents as
combined lesions of stenosis and regurgitation.
The most common causes of congenital tricuspid regurgitation are Ebsteins
anomaly and TV dysplasia. These two entities are clinically similar but anatomically different. Ebsteins anomaly is characterized by an inferior displacement of
the proximal hinge point of the septal and posterior leaets from the atrioventricular
junction and the existence of an atrialized ventricle (Fig. 2.8a,b). On the other hand,
the basal attachment of the TV is normal in TV dysplasia. Aaron described the
characteristic features of TV dysplasia as follows: [1] focal or diffuse thickening of
the leaets; [2] decient development of the chordae tendineae and papillary
muscles, most often binding down or tethering the valve margin; [3] improper
separation of valve components from the ventricular wall; and [4] focal agenesis of
valvular tissue [14].
Table 2.2 Tricuspid valve anomalies
1. Congenial
2. Right ventricular disease
3. Acquired
4. Right ventricular dilation
Ebsteins anomaly
Tricuspid valve dysplasia
Tricuspid valve hypoplasia/atresia
Tricuspid valve cleft
Double orice tricuspid valve
Unguarded tricuspid valve orice
Straddling of chordae tendineae
Arrhythmogenic right ventricular cardiomyopathy
Uhls disease
Endocardial broelastosis
Increased right ventricular pressure
Annular dilation
Left-sided valvular heart disease
Endocarditis
Trauma
Carcinoid heart disease
Rheumatic heart disease
Tricuspid valve prolapse
Iatrogenic (radiation, drugs, biopsy, pacemaker, ICD)
Pulmonary hypertension
Atrial septal defect
Anomalous pulmonary venous return
Pulmonary valve insufciency
30
M. Nii
Fig. 2.8 Ebsteins anomaly. (a) The simultaneous orthogonal 3 planes of a tricuspid valve. Note
the signicant plastering of the septal and posterior leaets. Arrows indicate the hyphenated distal
attachment of the anterior leaet to the right ventricular free wall. (b) An en face image of a
tricuspid valve in the closed position. (c) An en face image of a tricuspid valve in the opened
position. AL anterior leaet, AO aorta, ARV atrialized right ventricle, LA left atrium, LV left
ventricle, LVOT left ventricular outow tract, PL posterior leaet, RA right atrium, RV right
ventricle, RVOT right ventricular outow tract, VS ventricular septum
1. Ebsteins anomaly (Fig. 2.8): The crucial feature of Ebsteins anomaly is the
rotational displacement of the hinge point of the TV leaet, with maximal apical
displacement occurring at the junction of the septal and posterior leaets and no
displacement of the anterior leaet. This apical displacement creates the
atrialized portion of the basal right ventricle. The anterior leaet is usually
large, with normal annular attachments at the atrioventricular junction. However, it is commonly associated with restricted motion. The restriction of the
anterior leaet is caused by short chordae and the expansion of the anterior
papillary muscle onto the ventricular surface. In its severe form the chordae
tendineae are absent, and linear or hyphenated distal attachment of the leaet
edge to the ventricular wall is observed [15, 16].
2. The TV of a patient with hypoplastic left heart syndrome (Fig. 2.9): In patients
with hypoplastic left heart syndrome, tricuspid regurgitation is one of the most
important risk factors for mortality and/or ventricular dysfunction [17]. Figure 2.9
shows severe tricuspid regurgitation in a patient with hypoplastic left heart
syndrome after a Norwood operation. This patient had mild regurgitation before
the Norwood operation. However, as the dysfunction and dilation of the right
ventricle progressed, tethering of the septal leaet by the chordae from the
septum became prominent, and regurgitation deteriorated.
31
Fig. 2.9 Tricuspid valve in hypoplastic left heart syndrome. (a) An en face image of a tricuspid
valve in the closed position obtained using transthoracic three-dimensional echocardiography.
(b) A corresponding image to that in panel A obtained using color Doppler three-dimensional
echocardiography. (c) A four-chambered view showing tethering of the septal leaet and severe
tricuspid valve regurgitation. The arrow indicates the tethering chordae from the ventricular
septum to the margin of the septal leaet. AL anterior leaet, AV aortic valve, PL posterior leaet,
SL septal leaet
2.2.3
Common Atrioventricular Valve
1. Common AVV
The essential features of an atrioventricular septal defect (AVSD) are the defect
of the atrioventricular septum and the abnormalities of the AVV. There are four
subtypes of AVSD: complete, intermediate, transitional, and partial AVSD
[18]. In complete and intermediate AVSD, there is a single annulus, although
in intermediate AVSD there are two separate right and left orices divided by a
tongue of tissue that connects the superior and inferior bridging leaets
(Fig. 2.10). Complete AVSD is subdivided into three types according to the
anatomy of the superior bridging leaet (Rastelli classication). Figure 2.11
shows representative 3DE images of Rastelli type A and C defects. Rastelli A
accounts for 60 % of all complete AVSDs, Rastelli C accounts for 35 %, and
Rastelli B is rare and accounts for less than 5 % of cases. Partial and transitional
AVSDs have distinct right and left AVV annuli, and the left AVV invariably has
32
M. Nii
Fig. 2.10 Intermediate atrioventricular septal defect. (a) An en face image of an atrioventricular
valve in the closed position. (b) An en face image of an atrioventricular valve in the opened
position. Arrows indicate tongue tissue connecting the superior and inferior bridging leaet and
dividing the orice into two. AL anterior leaet, AOV aortic valve, IBL inferior bridging leaet,
LAVV left atrioventricular valve, LL lateral leaet, RAVV right atrioventricular valve
Fig. 2.11 Complete atrioventricular septal defect. (a) An en face image of Rastelli type A. (b) An
en face image of Rastelli type C. AL anterior leaet, AO aorta, IBL inferior bridging leaet, LL
lateral leaet, OS outlet septum, SBL superior bridging leaet
a cleft (Fig. 2.12). Transitional AVSD is a subtype of partial AVSD and is

associated with a small or restrictive inlet VSD.
2. Common-Inlet Atrioventricular Connection
The common-inlet atrioventricular connection is characterized by the connection of both atria to a single ventricular chamber by a common AVV. This type
of atrioventricular connection is usually associated with a common atrium and is
predominantly observed in patients with heterotaxy syndrome, especially in
those with right atrial isomerism (RAI). The connection of the common AVV
and the underlying ventricle is classied into three types, and this classication
is used in Shizuoka Childrens Hospital (Shizuoka, Japan) (Fig. 2.13). Type A
33
Fig. 2.12 Partial atrioventricular septal defect after repair. (a) An en face image in the closed
position. Arrows indicate the sutured cleft and the cleft is oriented towards the middle of the
ventricular septum. (b) An en face image in the opened position. (c) A corresponding image to that
in panel A by color Doppler three-dimensional echocardiography. Signicant regurgitation is seen
from the residual cleft and the commissure between the inferior and lateral leaets. AO aorta, IBL
inferior bridging leaet, LAVV left atrioventricular valve, LL lateral leaet, RAVV right atrioventricular valve, SBL superior bridging leaet
Fig. 2.13 Classication of atrioventricular connections in heterotaxy syndrome (Shizuoka Childrens Hospital Classication). AO aorta, L left ventricle, R right ventricle
34
M. Nii
Fig. 2.14 Common-inlet atrioventricular connection in right atrial isomerism (type B). (a) An en
face image in the closed position. (b) An en face image in the opened position. (c) Severe
atrioventricular valve regurgitation. AO aorta, CA common atrium, LL lateral leaet, LV left
ventricle, RV right ventricle
refers to a balanced connection, with the common AVV communicating equally

with both underlying ventricles, and accounts for 46 % of patients with RAI.
Type B is an unbalanced connection, with the common AVV communicating
predominantly with the unilateral ventricular chamber, and accounts for 19 % of
RAI patients. Type C is the univentricular connection, with the common AVV
communicating solely with the unilateral chamber, and accounts for 35 % of
RAI patients. Figure 2.14 shows 3DE and 2DE images of type B with severe
AVV regurgitation. In patients with RAI, the grade of AVV regurgitation is
closely related to prognosis [19].
2.3
2.3.1
Functional Assessment of the Atrioventricular Valve

by Three-Dimensional Echo
Assessment of the Shape and Area of the Annulus
in the AVV
The AVV leaet is exposed to large uid shear stresses, hydrostatic pressure, and
large in-plane tensions during the systolic phase. Under these stresses, the AVV
35
Fig. 2.15 The effect of stress reduction by leaet billowing in a computer model. (a) Stress on
leaet without billowing of leaet. (b) Stress on leaet with billowing of leaet. Note the
signicant stress reduction (blue color) resulting from the billowing curvature of the leaet
under the same saddle shape conditions. Leaet stress is calculated based on the von Mises
distortion energy theory. A warm color is associated with higher stress (Salgo et al. [22]; with
permission)
leaet expands its area by as much as about 50 % by stretching its undulated

collagen bers [20, 21]. The leaet expands to its limit very quickly at the
beginning of valve closure. This expansion is followed by a plateau phase and a
dramatic increase of leaet stiffness to prevent further leaet deformation. To cope
with leaet stress, the leaet is congured into a curved surface during the systolic
phase, which is called billowing. The annulus is known to form a saddle shape
during systole. The saddle shape of the annulus and the billowing of the leaet are
the main components for the leaet curvature formation that contributes to the
reduction of leaet stress (Fig. 2.15) [22]. Like the MV annulus, the TV annulus
also forms a saddle shape (Fig. 2.16) [23]. Salgo et al. showed that leaet stress is
minimized when the ratio of saddle height to commissural width, which is a
surrogate of annular saddle-shaped nonplanarity, is over 20 % [22]. A 3DE study
found that in normal human adults this ratio is 24 5 % [24]. Furthermore, the
saddle shape of the annulus optimizes force distribution on the chordal system as
the load is divided more evenly among the chordae with a saddle-shaped annulus
than with a at annulus [25]. The close relationship between the attening of the
annulus and the worsening of MV prolapse due to the elongation or rupture of the
chordae has been reported [24]. On the other hand, leaet stress also works as a
leaet coaptation force for the prevention of regurgitation. For example, the
anterior leaet of the MV is positioned parallel to systolic ow in the LV outow
tract, and the force exerted by the blood stream on the anterior leaet works as a
coaptation force, and also it expands the aortic valve annulus and enhances the
saddle shape of the MV annulus during systole. Therefore, stress on the leaet
works both ways, and as long as the stress on the leaet and the tension on the
chordae are balanced, the leaet functions normally. However, if a congenital
abnormality of the valve or valvular apparatus or an incorrect surgical repair causes
abnormally increased leaet stress, it leads to valve failure.
36
M. Nii
Fig. 2.16 Saddle shape of the mitral and tricuspid valves. The bending angle becomes most acute
in early diastole in the mitral and tricuspid valves. AO aorta, IC isovolumic contraction, IR
isovolumic relaxation, MV mitral valve, RVOT right ventricular outow tract, SEM standard
error of the mean, TV tricuspid valve
In normal children, the saddle shape becomes the most prominent at early
diastole in both the MV and TV (Fig. 2.16) [23], and the grade of the saddle
shape is closely related to ventricular function and the grade of regurgitation
(Fig. 2.17) [26]. The annulus becomes at when there is signicant AVV regurgitation or reduced ventricular function, placing more stress on the leaets and
leading to further worsening of regurgitation [24, 26]. The area of the annulus
also has an important role in maintaining normal AVV function. It has been known
that, in adults, the annular area of the MV becomes small during systole to support
good leaet coaptation and reduce leaet stress and that during diastole it expands
to reduce resistance of the annulus to blood inow [27, 28]. However, this pattern of
annular area change during the cardiac cycle is not always the case in children. In
the majority of children, the annular area of the MV expands during systole and
reaches maximum before the opening of the MV. This pattern is similar to the
change of left atrial volume during the cardiac cycle, suggesting the inuence of left
atrial volume on the annular area [23]. In the TV, the annular area becomes small
during systole and expands during diastole in children, which is the same pattern as
that observed in adults [29]. The reduction of annular area by ventricular septal
37
Fig. 2.17 Saddle shape and

grade of tricuspid valve
regurgitation in hypoplastic
left heart syndrome. The
saddle shape of the tricuspid
valve becomes at with the
progression of
regurgitation. IC
isovolumic contraction, IR
isovolumic relaxation, TR
tricuspid valve
regurgitation, TV tricuspid
valve
bowing towards the right ventricle during systole is especially important for
retaining good leaet coaptation, as the mobility of the septal leaet of the TV is
limited compared to that of the other two leaets. Atrial contraction is also an
important factor in the reduction of the annular area in advance of the beginning of
systole; the MV reduces its area by about 10 % during atrial contraction, and the TV
reduces its area by about 16 %, suggesting a greater dependence of areal reduction
on atrial contraction in the TV [23].
2.3.2
Papillary Muscle and Chordal Position in 3DE
The position and function of the papillary muscles are also very important factors
for maintaining normal AVV function. To cope with the force on the leaets, the
papillary muscles generate tension and shorten during systole so as not to cause
prolapse of the leaets. The contraction and shortening of papillary muscle occurs
as follows: isometric contraction of the papillary muscle coincides with isovolumic
contraction of the ventricle, and the shortening of the papillary muscle begins at the
early ejection phase and continues throughout the ejection phase and also during the
isovolumic relaxation phase [30]. Although the contraction of the papillary muscle
is important for normal AVV function, the position of the papillary muscles has
been elucidated as an even more crucial factor for AVV function through enthusiastic investigations of functional mitral regurgitation in adults after myocardial
infarction. Three-dimensional echocardiography (3DE) enabled us to assess the
spatial relationship among the papillary muscles, annulus, and leaets [24, 31]. The
lateral displacement of the papillary muscle from the annulus due to LV enlargement or myocardial infarction causes the abnormal tethering of leaets and
an imbalance of the force distribution on the chordae, leading to regurgitation
[31, 32]. The lateral displacement of the papillary muscle is also a cause of TR in
patients with HLHS or left AVV regurgitation in AVSD after repair [26, 33].
38
2.3.3
M. Nii
Prolapse and Tethering Analysis in 3DE
The commercially available software MVQ (QLAB Cardiac 3DQ; Philips Medical
Systems, Andover, MA) allows for quantitative geometrical measurement of AVV
based on acquired transesophageal 3DE data [34]. This software assists in the stepby-step creation of a three-dimensional AVV model, proceeding through the
annulus, coaptation line, leaets, and tips of the papillary muscles, and the created
AVV model can be manipulated in a three-dimensional space and overlaid on three
simultaneous orthogonal planes (Fig. 2.18). Based on the created three-dimensional
AVV model, MVQ is able to measure the following parameters: the intercommissural and anteroposterior annular diameters, the annular area, the area, length,
and angle of each leaet, the tethering height, the coaptation leaet angle, and the
angle between the aortic valve annulus and AVV annulus. The 3DE data also
provide quantitative information on tethering, prolapse, and billowing of the leaet.
Takahashi and Smallhorn analyzed tethering and prolapse volume of leaets and
showed a close relationship between tethering or prolapse volume and grade of TR
in patients with HLHS [35]. They also showed that prolapse is closely related to
annular dilation and age, suggesting that prolapse is mostly a secondary change due
to increased stress on the chordae and leaet over the years. On the other hand,
tethering is more frequently seen in younger patients, suggesting an intrinsic
Fig. 2.18 Three-dimensional mitral valve model created by MVQ. (a) The simultaneous orthogonal planes of the mitral valve based on transesophageal three-dimensional echocardiography
volume data. (b) A three-dimensional mitral valve model. The leaet area colored in red signies
prolapse, and the area colored in blue signies tethering. A anterior, AL anterolateral, P posterior,
PM posteromedial
39
Fig. 2.19 Interrelation of atrioventricular valve regurgitation and ventricular dilation and/or
dysfunction. EDP end-diastolic pressure, PM papillary muscle
abnormality of the chordae and/or papillary muscles. It may also be related to the
hemodynamic condition of the palliative stage. The volume overload and coronary
insufciency caused by systemic to pulmonary artery shunting sometimes lead to
ventricular dilation and/or dysfunction [17]. The ventricular dilation leads to the
enlargement of the annulus, ventricular dysfunction leads to a at annular shape,
and both ventricular dilation and dysfunction lead to a lateral displacement of the
papillary muscles and leaet tethering, which are all causative of increased leaet
stress and maldistribution of force on the chordae and, hence, of chordal elongation
or rupture and prolapse of the leaet. These factors are all interrelated and form
feedback loops, sometimes causing a vicious cycle (Fig. 2.19).
2.3.4
Quantitative Assessment of Regurgitation
The quantitative assessment of AVV regurgitation by echocardiography remains

challenging. Although several echocardiographic methods have been proposed, a
true gold standard evaluation is still lacking. Measurement of the effective
regurgitant orice area (EROA) and regurgitant volume is currently recommended
as a quantitative assessment. The proximal isovelocity surface area method or the
pulsed Doppler-derived ow volume method is used to estimate EROA or
regurgitant volume. However, both methods are indirect measurements based on
several imprecise assumptions. The well-accepted surrogate to the direct measurement of EROA is the vena contracta width, which is the narrowest cross section of
the regurgitant jet. However, as the EROA is not always circular, it cannot be
40
M. Nii
assessed by a vena contracta width in a single dimension by 2DE. 3DE enables the
direct measurement of EROA by placing the cropping plane perpendicular to the jet
direction at the narrowest area of the jet stream. The EROA is then measured by
manual planimetry of the color Doppler jet signal. This method is reportedly
accurate and the regurgitant volume can be estimated as the vena contracta area
multiplied by the velocity time integral of the regurgitant jet on the continuouswave Doppler [36]. However, the low temporal resolution of color Doppler 3DE
due to the slow volume rate is a limitation of current 3DE systems.
Conclusion
3DE offers new insights into AVV anatomy and function. An en face view of
the AVV from the atrial side created by 3DE approximates the surgeons
view, making it easier for echocardiologists to communicate the anatomy and
pathology of the AVV to surgeons prior to the operation. Moreover, 3DE
ushered in a new era of functional assessment of the AVV by enabling the
creation of a three-dimensional computer model. This modality has an enormous potential to deepen our knowledge and may ultimately lead to virtual
AVV repair if three-dimensional anatomical information is combined with
pulsed Doppler-derived 3D ow dynamics information.
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2:12451252
Chapter 3
Assessment of Intracardiac Anatomy

by Magnetic Resonance Imaging
Satoshi Yasukochi
Abstract Recent advances of magnetic resonance imaging (MRI) technology
enable us to visualize more ne and accurate structures of intracardiac anatomy
and morphology in patients with congenital heart disease (CHD) before and after
the surgical interventions.
The basic concept to make a diagnosis of CHD by every imaging modality is
based on the same segmental approach, which consisted of three-step evaluation of
the cardiac segments: atrial situs, ventricular loop, and the position of the great
arteries. Then two-step evaluation of relations or connections among the three
cardiac segments, which is between the atria and the ventrclem and between the
ventricle and the great vessels. This ve-step segmental approach is a standard
technique to complete the morphological assessment of CHD in the rst place.
For this purpose, the sequential multi-slice cross-sectional imaging data
obtained by MRI are particularly useful to undergo the segmental-approach evaluation of CHD. In some cases, the other imaging protocol such as a three-point
planning or as a double oblique technique is prescribed to obtain optimal cut plane
to observe each specic lesion of interest of CHD by the observers preference
without any interference of adjacent organs and add more detailed information
about the morphology.
We could use these sequential gap-less multi-slice cross-sectional imaging data
to reconstruct the ne three-dimensional intracardiac anatomy, which could provide
more clinically useful information for the further diagnosis and treatment. Recent
advances of computer imaging technology enable us not only to reconstruct the
intracardiac 3D morphology of complex CHD with adjacent organ structures but
also to simulate the surgical approach or virtual cardiotomy for planning the realworld cardiac surgery by software or by 3D anatomical articial model. The more
the technology advances, the better the understanding of the intracardiac anatomy
and the surgical outcome can be promised.
Keywords Magnetic resonance imaging Congenital heart disease Intra-cardiac
anatomy Segmental approach Three-dimensional reconstruction
S. Yasukochi, MD (*)
Heart Center/Pediatric Cardiology, Nagano Childrens Hospital, 3100 Toyoshina,
Azumino, Nagano 399-8288, Japan
e-mail: mapleyasukochi@gmail.com
Springer Japan 2015
DOI 10.1007/978-4-431-54355-8_3
43
44
3.1
S. Yasukochi
Basic Principle of Magnetic Resonance Imaging

for Intracardiac Structure
Magnetic Resonance Imaging (MRI) is a powerful imaging tool to dissect intra- and
extracardiac anatomy without using ionizing radiation or contrast medicine. This
imaging technique and computed tomography can be applied for all-age patients
with congenital heart disease (CHD) at all angles, irrespective of surrounding
structures or air. Since MRI delivers multi-slice or true three-dimensional images,
it is easy to reconstruct the three-dimensional morphology of the cardiovascular
system and its topographic relationships to the extracardiac structures, such as the
trachea and bronchus [1, 2].
Recent advances of MRI technology, together with those of softwares to reconstruct images, remarkably improve the spatial and temporal resolution of the
images which enables to clarify the ne two-dimensional and three-dimensional
intracardiac morphology. Moreover, we could use these real three-dimensional
intracardiac images to simulate the surgical procedure and to plan treatment
strategy.
3.2
MRI Imaging Protocol
The examination begins with a series of static scout images of the thorax and
abdomen in three orthogonal body planes. All subsequent sequences for detailed
examination are planned using this scout as well as subsequent obtained cine
images as a reference [1] (Fig. 3.1).
As a principle of prescription, an imaging plane is dened as an axial view
perpendicular to the body axis and the images are acquired by the serial multiple
axial thin slice (1.8 mm thick interpolated to 0.9 mm) to cover the whole heart from
the upper abdomen up to the neck vessels. To visualize the 3D-twisted structures
like aortic arch or coronary arteries, an imaging plane is also dened unequivocally
either by three points, so-called three-point planning, or by how it dissects two
separate images previously obtained, the so-called double oblique technique [1].
These images are obtained by ECG gating or vector cardiac gating for compensating heart movement and by navigator gating with prospective slice correction for
compensating respiratory motion [3].
3.2.1
Whole-Heart Protocol
To dene the intracardiac anatomy without contrast medicine, the vector cardiac
real-time navigator-echo technique with prospective slice correction is used to
compensate for respiratory motion. A ow-insensitive T2-weighted preparatory
3 Assessment of Intracardiac Anatomy by Magnetic Resonance Imaging
45
Fig. 3.1 Work ow for anatomical and cine imaging. Each scan plane is prescribed starting from
two differently angled reference images, using the double oblique technique for the four-chamber
view. Starting from the 3 orthogonal scout plane images: axial (a), coronal (b), sagittal (c), cine
imaging begins with a vertical long-axis or pseudo-two-chamber view (d). From (d), serial shortaxis imaging planes (e) is prescribed parallel to the atrioventricular groove. The four-chamber
view, (f) and (g), cuts through the tricuspid and mitral valves and through the ventricular apex. The
three-chamber view (h) cuts through the aortic valve to obtain the left ventricular outow view (i)
46
S. Yasukochi
pulse for contrast enhancement without the use of contrast material was followed by
a localized anterior saturation preparatory pulse, a navigator echo, a spectrally
selective fat-saturation pulse (spectral presaturation by inversion recovery), and a
3D-segmented k-space gradient-echo sequence (TR range/TE range, 4.35.0/2.2
2.5; ip angle range, 90100 ; radial k-space sampling technique). These sequences
were followed by whole-heart imaging with eight phase-encoding steps per cardiac
cycle, so-called bright-blood imaging. Slices 1.8 mm thick (interpolated to 0.6 mm)
were acquired with a 180200 mm eld of view and were reconstructed with a
512 360 matrix (in-plane voxel size, 0.35 0.35 mm). The parallel imaging
technique of sensitivity encoding was used, usually with accelerator factors 1.3 in
the phase direction and 1.0 in the slice direction [3, 4].
The bright-blood protocol demonstrates the intracardiac chamber or blood
space as a bright white as opposed to the myocardium as a grey using a 3D steadystate free precession (SSFP) MRI sequence (Fig. 3.2). By this imaging protocol, a
whole-heart imaging from the multi-slice axial images is obtained as to understand
the three-dimensional anatomy of CHD by the sequential and systematic diagnosis
of the previously reported segmental approach. The border of the myocardium and
blood can be clearly demarcated, which is very benecial to plan the reconstruction
of three-dimensional intracardiac anatomy, just like an ECG-gated contrastenhanced cardiac computed tomography [2, 6].
Fig. 3.2 The sequential multi-slice imaging of MRI by whole-heart protocol (bright-blood
imaging)
3.2.2
47
Black-Blood Protocol
There are several imaging sequences of black-blood protocol. The 2D blackblood sequence by T1-weighted spin-echo/echo-planar imaging was previously
used, but the data was not isotropic and time-consuming. Recently 3D volumetric
black-blood angiography and vessel-wall imaging is proposed by using 3D rapid
acquisition with relaxation enhancement (RARE) or turbo spin-echo sequence
(TSE). This RARE technique has a sequence-endogenous ow-void effect and
used with variable or low ip refocusing enables acquisition of 3D T2-weighted
imaging [5]. This imaging data is proton density-weighted acquired volume isotropic T2-weighted voxel data which can be extracted from any angle cut-plane
images of intracardiac anatomy as a ne black-blood angiography. This protocol
is also called as 3D volume isotropic T2-weighted acquisition: VISTA
(Fig. 3.3) [5].
This black-blood protocol is very useful to visualize the intracardiac morphology or vessel wall if there is a signicant ow-void lesion like stenosis or regurgitation to cause a signal loss by dephasing in a gradient-echo sequence.
Fig. 3.3 VISTA imaging: from voxel data, three orthogonal sequential multiplane images are
expanded
48
S. Yasukochi
Fig. 3.4 Intracardiac artifact: ow void. Flow void or dephasing the signal by turbulent ow
sacrices the imaging data of intracardiac morphology in cine MRI. This case has the signicant
subaortic stenosis due to subaortic conus septum and abnormal membranous structures (a);
however, the T2-weighted black-blood demonstrated the clear image of subaortic anatomy (b)
3.2.2.1
Cine MRI
This is the moving image of the beating heart. The sequence of cine MRI is an
ECG-triggered turbo eld-echo SSFP (one signal acquired per R-R interval; heart
rate phase, 80; cardiac synchronization, retrospective gating). The sequence parameters were as follows: 4.2/1.88; ip angle, 60 ; eld of view, 220 mm. A 192 154
matrix with cartesian k-space sampling yielded an in-plane resolution of approximately 1.15 0.87 mm (reconstructed 256 256 matrix, 0.8 0.8 mm). This cine
MRI gives blood as a white signal and myocardium as a grey with clear border [1, 3, 4].
A serial multiplane short-axis cine image is used for calculating ventricular
volume and myocardial masses, which is widely accepted as a gold standard for
ventricular volumetry, since this method does not require the geometric assumptions as opposed to echocardiography or cineangiography.
Because of the TFE sequence, turbulent ow either by stenosis or by regurgitation ow causes loss of signal from dephasing and can be identied as dark streak
within the bright-blood pool [1] (Fig. 3.4). This could sometimes interfere the
interpretation of the acquired images for intracardiac morphology because of the
signal loss due to the lesion of stenosis or regurgitation in the case of cine MRI and
whole-heart protocol.
3.3
How to Obtain Cardiac Imaging by MRI

in Clinical Setting
Since MRI examination takes a longer time to acquire the imaging data, younger
children and infants must be sedated to avoid the motion artifact during the
examination. In some cases, general anesthesia with or without intratracheal
49
Fig. 3.5 Abdominal-belt technique. Navigation tracing for diaphragmatic motion
intubation is necessary to secure the respiration and hemodynamic stability to

obtain a good MRI imaging [1, 3, 5]. Because of the necessity of sedation or
anesthesia, the patients need to be monitored by MRI-applicable heart rate and
saturation monitor with video monitoring.
One of our tips to reduce the patients respiratory motion is abdominal-belt
technique, which a towel or blanket belt wraps the patients abdomen before
applying MRI coil to restrain. This could reduce the diaphragmatic motion to
improve the navigation tracking (Fig. 3.5).
Prior to any study, absolute and relative contraindications of MRI study must be
addressed [1]. Pacemakers and other electronic implanting devices such as an
implanted pump for drug infusion are considered as absolute contraindication.
The metallic implantation of non-ferromagnetic devices such as stent, coils, and
surgical wires is not the contraindication and is tested for MRI even just after
implantation.
3.4
Basic Approach for Making a Diagnosis of CHD
The basics of making a diagnosis for complex CHD are a segmental approach.
Before starting the intracardiac anatomical assessment, one should understand the
whole cardiac structure and anatomy by this classical but effective systematic
diagnostic method [712].
50
3.4.1
S. Yasukochi
What Is Segmental Approach/Analysis?
The segmental analysis of CHD was introduced about 35 years ago and is now used
worldwide [712], not only in pediatric cardiology but also in adult cardiology with
CHD [13, 14]. This approach is exible and applicable to any imaging modality and
thus particularly useful in clinical practice. In the segmental approach, the cardiac
anatomy is assessed rst by dividing the heart into three distinct segments. These
segments are the visceroatrial situs, which is evaluated in step 1; the ventricular
loop, evaluated in step 2; and the position of the great vessels, evaluated in step
3. These segments are fundamental building blocks of the cardiac anatomy, and the
morphological and anatomical features specic to each segment are assessed
separately. The understanding of these morphological features of each segment is
a key to make a precise diagnosis in the segmental analysis of CHD.
After three-step diagnosis of cardiac segments, two steps evaluated the relationships between the cardiac segments (blocks) at the atrioventricular (step 4) and
ventriculoarterial levels (step 5). Finally, associated abnormalities in individual
segments are assessed and diagnosed. The notation system developed by Van
Praagh (a series of three letters, separated by commas, within parentheses) may
be used in conjunction with this approach, as a segmental description [1517].
3.4.2
Step 1: Determining the Visceroatrial Situs: Atria
There are three types of situs: solitus (S,,), inversus (I,,), and ambiguus (A,,).
By denition, the type of situs is determined by the relationship between the atria
and the adjacent organs. The rst step in the assessment of the cardiac anatomy is to
locate and identify the left and right atria. Anatomically, the atrial chamber
differentiation is based on the morphological aspect of the atrial appendages.
The atrial appendages are earlike extensions of the atria. Typically, the right
atrial appendage is broad and blunt (triangular), whereas the left atrial
appendage is narrow, pointed, and tubular (ngerlike) (Fig. 3.6). The inferior
vena cava (IVC) is often used as a landmark for locating the anatomical right
atrium [1012].
The relationship between the right and left bronchi and pulmonary arteries
provides a reliable determinant of situs such as eparterial bronchus (bronchus
above the pulmonary artery) as a right-side structure and hyparterial bronchus
(bronchus below the pulmonary artery) as a left-side structure [1012].
The position of the heart in the thorax and the orientation of the cardiac apex are
also important as determined by the orientation of the cardiac base-apex axis:
dextrocardia, mesocardia, and levocardia but not determinative of the situs.
51
Fig. 3.6 The morphology of the atrial appendage. Right atrial appendage shows broad and blunt
triangular or baseball glove-like shape, while the left atrial appendage is narrow and tubular like an
index nger shape
3.4.3
Step 2: Determining the Ventricle Loop: Ventricle
The ventricular loop or ventricular situs may tend rightward (dextro-loop; hereafter,
d-loop) (, D, ) or leftward (levo-loop; hereafter, l-loop) (, L, ). The
cardiac structures are identiable on the basis of their specic morphological
features [1012]. The shape of the right ventricle (RV) is usually triangular and
crescent, while that of the left ventricle (LV) is a bullet shape.
The morphological characteristics of the RV are the presence of an apical
moderator band and the subvalvular conus, which is a muscle that demarcated the
tricuspid and pulmonary valve (no brous continuity of tricuspid-pulmonary junction). The tricuspid valve is usually attached lower at the more apical site of the
interventricular septum than the mitral valve (Fig. 3.7) [1113].
The trabeculae of RV septum is coarse, while that of the LV is smooth. In
addition, the papillary muscles of the RV are attached to both the interventricular
septum and the free wall, whereas the two papillary muscles of the LV are attached
only to the free wall (Fig. 3.8) [18, 19].
These basic morphological features of RV are preserved even if it is a pulmonic
RV (supporting the pulmonary circulation) or systemic RV (supporting the systemic circulation), although the entire shape of each RV is different as shown in
Fig. 3.9. The entire shape of pulmonic RV is triangular and crescent, while that of
systemic RV is an ellipsoid like a morphological LV [19]. However, in complex
cases, it may be difcult to determine which ventricle is the morphological right
ventricle and which is the morphological left ventricle. In such cases, the identication may be based on the assumption that in the presence of a right-sided aortic
52
Fig. 3.7 The morphology of the right ventricle
Fig. 3.8 The morphology of the left ventricle
S. Yasukochi
53
Fig. 3.9 Overall morphology of pulmonic RV and systemic RV
valve, the right ventricle is located to the right of the left ventricle (d-loop), and in
the presence of a left-sided aortic valve, the right ventricle is located to the left of
the left ventricle (l-loop). This is known as the loop rule [20].
3.4.4
Step 3: Determining the Position of the Great Arteries
Several variants may be observed with regard to the positions of the great vessels.
The vessels may be in normal position (solitus) (, , N(S)), inverted position
(inversus) (, , I), D-transposition (, , D), or L-malposition (, , L). In normal
heart, the aorta (Ao) is located posterior to and right of the pulmonary artery
(PA) (normal position) and runs crossing each other in spiral relation. If this
location shows mirror image of its position and running course, it is called as the
inverted position (, , I) or inverted normal position (, , IN). The position of the
great arteries is dened as where the aorta is located in relation to that of pulmonary
54
S. Yasukochi
artery. If the aorta is located anterior to and the right of the pulmonary artery, the
position is nominated as D-transposition. If the aorta is located anterior to and the
left of the PA, the nomination is L-transposition or L-malposition [712,
1517, 21].
Besides the spatial position of the great arteries, subvalvular conus anatomy is
also important. There are four type of conal anatomy: subpulmonary conus (normal), subaortic conus, bilateral conus, and bilaterally absent conus [21].
3.4.5
Step 4: Determining the Atrioventricular Connection
There are ve types of atrioventricular connection: concordant (normal), discordant, ambiguous, double inlet, and absent right or left connection. With a normal or
concordant connection, the right atrium drains into the morphological right ventricle, and the left atrium drains into the morphological left ventricle. With a discordant connection, the right atrium drains into the morphological left ventricle, and
the left atrium drains into the morphological right ventricle. Malposition of the
great vessels frequently occurs in association with discordant atrioventricular
connection [1012]. In cases of heterotaxy, the connection is described as ambiguous. Concordant, discordant, and ambiguous may be used to describe the connections when two ventricles are present, whereas double inlet and absent right (or left)
connection are used for a univentricular heart. For more precise description, the
anatomy and the position of the atrioventricular valve annuli also may be described
[1012, 1517].
3.4.6
Step 5: Determining the Ventriculoarterial Connection
Besides permanent truncus arteriosus, four types of ventriculoarterial connection

may develop: (1) concordant connection (the pulmonary artery arises from the right
ventricle, and the aorta arises from the left ventricle); (2) discordant connection,
which is synonymous with transposition of the great vessels (the pulmonary artery
arises from the left ventricle, and the aorta arises from the right ventricle); (3) double outlet right ventricle (the one and a half of two great vessels arise from the right
ventricle); and (4) double outlet left ventricle (the one and a half of great vessels
arise from the left ventricle) [1012, 1517].
In the light of ventriculoarterial connection, subvalvular conal anatomy is also
important. Typically, subaortic conus without subpulmonary conus is found in
cases of D- or L-transposition [10, 1517].
3.4.7
55
How to Do the Segmental Approach for Systematic

Diagnose of Complex CHD
By the segmental approach in this 3-year-old case (Fig. 3.10), the apex of the heart
directs to the right (dextrocardia) and the IVC drains to the right-side atrium which
is a morphological right atrium (solitus). The ventricle is a single chamber having
two inlets (black arrows) with subaortic conus, which is the morphological right
ventricle. There is a rudimentary LV located posterior to the right ( l-loop). The
aorta arises from the most anterior outow of RV and located that is anterior and to
the left of pulmonary trunk (L-malposition). The atrioventricular connection is
double inlet and ventriculoarterial connection is double outlet right ventricle.
Therefore, the segmental approach of this case is dextrocarida [S,L,L], double
inlet right venricle (DIRV), double outlet right ventricle(DORV), pulmonary atresia, and post surgical status of extracardiac total cavo-pulmonary connection.
Fig. 3.10 Case: 3-year-old girl having diagnosis of dextrocardia, [S.L.L] double inlet of RV,
DORV, PA, LSVC, post-TCPC
56
3.5
S. Yasukochi
Three-Dimensional Reconstruction of Intracardiac

Anatomy in Congenital Heart Disease
For many years preoperative planning in CHD has relied on the abilities of surgeons
to convert two-dimensional imaging information to three-dimensional mental
models and surgical strategies [6, 2228]. Recently the 3D echocardiography
could provide more accurate 3D/4D images of cardiac valves and intracardiac
anatomy in CHD; however, it has limitations because of echo-window. The accurate
preoperative assessment of the intra- and the extracardiac morphology not only for
the local lesion but also for the whole heart is essential for the cardiac surgeons to
make their surgical plan and to determine the right incision to access the lesions to be
corrected. These assessments denitely need for the patients with more complex
anomalies of CHD in order to achieve better surgical outcome and prognosis.
For this purpose, the 3D-reconstructed MRI images for assessing the intracardiac
anatomy are very benecial because of their direct visualization of the 3D anatomy.
These images are easily reconstructed from the gap-less multi-slice 3D-SSFP
sequence (white-blood protocol) and sequential VISTA images (black-blood
protocol).
3.5.1
Artifact of the Intracardiac Implanted Devices
Intracardiac device implanted by catheter intervention sometimes causes the artifact to interfere the magnetic eld to lose the anatomical signals. The degree of
losing signals depends on the MRI imaging protocol. In a case after implantation of
Amplatzer device which is made of nitinol (nonmagnetic material), the imaging
signal of atrial septum around the device is lost in 3D-SSFP sequence and in VISTA
but less in cine MRI (Fig. 3.11).
Fig. 3.11 Intracardiac device artifact. Amplatzer septal occluder (made of nitinol) causes the loss
of imaging signal around the device in whole-heart protocol of 3D-SSFP sequences (a) and 3D
black-blood angiography (b) but less in cine MRI (c)
57
Fig. 3.12 Intracardiac device artifact. Stainless stent causes the signal loss and ghost in the
pulmonary artery in T1-weighted spin-echo imaging (a), which can be visualized well in an
enhancement computed tomography (b). In (a), the stainless wire for sternal closure also causes
the signal loss at the center of the sternum (white arrow)
Stent and sternal wires made of stainless steel (nonmagnetic material) also cause
the signal loss and ghost in the limited area of the vessel lumen, which is well
visualized in an enhancement computed tomography (Fig. 3.12).
One must consider the presence of these materials and turbulent ow when
assessing the intracardiac morphology, especially the lesion next to these causes of
the artifacts.
3.5.2
The Inner View of the Right Ventricle of Normal Heart
The right ventricle is composed of three anatomical and functional subunits, which
include the inlet portion extending from the tricuspid valve to the insertions of the
papillary muscles onto the ventricular wall, the trabecular portion involving the RV
body and apex (the fundamental component of the pump mechanism), and the
outow or infundibular portion extending to the pulmonary valve that is generally
free of trabeculations [13]. The overall shape of pulmonic RV is triangular and
crescent-shaped and wrapped around the left ventricle. The myocardial ber orientation of the RV consisted of two layers which showed more longitudinal direction
in the wihch showed the more longitudinal directions in the inlet and in the
trabecular sinus, however demonstrates the more circumferential orientation in
the infundibulum.
The characteristics of the right ventricle are structures of muscle band on the
interventricular septal surface. There is an anterior papillary muscle attached to the
apical septum from where the moderator band bridges to connect to the trabecula
septomarginalis (TSM) [1113]. TSM rises up toward to crista supraventricularis
and branches off to anterior and posterior limb below the pulmonary valve.
58
S. Yasukochi
These morphologies can be visualized by 2D sequential slice image in Fig. 3.13;

however, the more detailed and 3D spatial recognition is easier in the
3D-reconstructed MRI images from 3D-SSFP whole-heart protocol by the commercially available software as in Fig. 3.14 [2, 3, 6, 22].
Fig. 3.13 The morphology of right ventricle from VISTA imaging of MRI: coronal sequential
sections
Fig. 3.14 The 3D morphology of the right ventricle reconstructed from whole-heart imaging
of MRI
3.5.3
59
The Inner View of the Left Ventricle
The interventricular septum of the left ventricle is smooth and compacted without
the trabeculated muscle band in the normal heart (Fig. 3.15). The two papillary
muscles attach to the free wall of the left ventricle. In a case with noncompaction of
the left ventricle, massive trabeculations are found at the apical portion of the
left ventricle, which usually appears as negative-contrast structures in cine
MRI. The 3D-reconstructed images provide three-dimensional distribution of
such trabeculation in the left ventricle (Fig. 3.16).
Fig. 3.15 The surface of the interventricular septum viewed from both sides of the RV and
LV. The position of membranous septum is also indicated by the marked area
Fig. 3.16 Case with LV noncompaction. Massive trabeculation in the apex of the left ventricle is
observed and occupied at lower half of the LV. This is also found as negative-contrast structures in
cine MRI
60
3.5.4
S. Yasukochi
Ventricular Septal Defect
The ventricular septal defect (VSD) is a defect of interventricular septum. In many

cases of VSD, the location of the defect lies around the membranous ventricular
septum (MVS), but could be at any sites of interventricular septum. The MVS is
located at the indicated area viewed from both sides of the interventricular septum.
The MVS is a thin brous membrane, about 1 cm long, which extends upward and
to the right from the muscular ventricular septum to the adjacent part of the aortic
brous annulus that also gives attachment to the right posterior (noncoronary) and
anterior (right coronary) aortic valve cusps. It is of considerable clinical importance
that there lies between the muscular ventricular septum and the membranous
septum the atrioventricular (AV) bundle of the cardiac conduction system. The
membranous septum has an irregular quadrangular form and has right and left
surfaces. The location of the VSD is easy to be visualized by the 3D-reconstructed
MRI images as well as the surrounding structures of the RV (Fig. 3.17).
3.5.5
Intracardiac Anatomy of Double Outlet Right Ventricle
The 3D spatial relation between the position of VSD and conal septum anatomy
with both great arteries is extremely important when one plans to undergo the
denitive surgery. The number and size of VSD defect may alter the surgical
Fig. 3.17 The ventricular septal defect at the perimembranous portion is indicated in the cine MRI
and in the 3D-reconstructed MRI image viewed from RV side
61
Fig. 3.18 3D-SSFP sequential multi-slice. Case: DORV with subaortic VSD and subpulmonary
stenosis (whole-heart protocol)
treatment strategy. Although a conus is easy to dene as the presence of muscle

between a semilunar valve and atrioventricular valve, muscle strip may vary from a
few millimeters to a few centimeters wide. The aorta is to the right and side by side
with the pulmonary trunk. The location of VSD is often described as relational
categories such as subaortic, subpulmonary, doubly committed, and noncommitted
or remote VSD.
The surgeon must realize the 3D spatial relations of the position of VSD, the
conus, and the both great arteries. They have to convert the sequential multi-slice
2D MRI images to reconstruct the 3D anatomy in their mind, which is sometimes
difcult (Figs. 3.18 and 3.19). By the segmental approach in this case, the IVC
drains to right side which is the right atrium (solitus) and the right-sided ventricle
has a moderator band in the apex which means the RV. Since RV is located at
anterior to the right of LV, the ventricular loop is d-loop. The great arteries sit side
by side, but the pulmonary trunk is located at the slight anterior to the left of the
aorta which forms an arch (solitus). VSD located at the subaortic position and RV
outow tract shows a stenosis at the subpulmonary level. Therefore the diagnosis of
this case is (S, D, N or S) double outlet right ventricle with subaortic VSD and
subpulmonary stenosis.
In order to elucidate the 3D intracardiac anatomy of this case, 3D images are
reconstructed and viewed from RV side (Fig. 3.20a) and from LV side (Fig. 3.20b).
The intracardiac structures including trabecular muscles and conus as well as VSD
are well visualized in size, position, and its running course with their mutual spatial
relations.
62
S. Yasukochi
Fig. 3.19 Case: DORV with subaortic VSD and subpulmonary stenosis (VISTA black-blood
protocol)
Fig. 3.20 (a) 3D intracardiac anatomy of DORV with subaortic VSD and subpulmonary stenosis
viewed from anterior aspects of right ventricle. (b) 3D intracardiac anatomy of DORV with
subaortic VSD and subpulmonary stenosis viewed from posterior to the left of the left ventricle
3.5.6
63
The Assessment of the Intracardiac Anatomy

of Subaortic Stenosis by 3D-Reconstructed MRI
Images from Sequential T1-Weighted Spin-Echo
Images
In most of the cases with CHD, 3D MRI images of intracardiac anatomy can be well
reconstructed from 3D-SSFP whole-heart protocol; however, in some cases with
intracardiac stenosis causing signicant turbulent ow, the 3D reconstructions are
difcult because of the signal loss by ow void or dephasing. In such case, the
sequential images obtained by black-blood protocol or VISTA can be used for
reconstruction [3, 4].
This particular case is a 15-year-old girl diagnosed with [S, L, X] dextrocardia,
mitral atresia, double outlet right ventricle, pulmonary stenosis, and subaortic
stenosis. The exact morphological assessment of subaortic stenosis is rather difcult by the sequential 2D MRI images either of axial (Fig. 3.21a) or from coronal
Fig. 3.21 (a) The sequential axial section images of T1SW/EPI in a 15-year-old patient with
diagnosed as [S, L, X] dextrocardia, mitral atresia, double outlet right ventricle, pulmonary
stenosis, and subaortic stenosis. After reconstructing 3D MRI, viewed from the bottom demonstrating the intracardiac anatomy of subaortic stenosis. (b) The sequential coronal section images
of T1SW/EPI in a 15-year-old patient with diagnosed as [S, L, X] dextrocardia, mitral atresia,
double outlet right ventricle, pulmonary stenosis, and subaortic stenosis. After reconstructing 3D
MRI, viewed from the bottom demonstrating the membranous structure subaortic stenosis
64
S. Yasukochi
Fig. 3.21 (continued)
section (Fig. 3.21b). However, 3D-reconstructed intracardiac morphological

images provide more ne and detailed structures below the aortic valve together
with outow geometry. These 3D images can be used for the future surgical
approach and for planning the surgical treatment strategy.
3.5.7
To Visualize the Intracardiac Anatomy Unseen

in 2D Images
The other benet of 3D reconstruction of intracardiac anatomy is to visualize the

structures unseen or difcult to imagine from the sequential 2D cross-sectional
images. This 20-year-old patient has a situs solitus, concordant crisscross heart,
double outlet right ventricle, and pulmonary stenosis which was treated by modied
Fontan procedure (lateral tunnel method). The position and shape of VSD is hardly
estimated from the 2D cross-sectional sequential images (Fig. 3.22); however,
3D-reconstructed extra- (Fig. 3.23) and intracardiac (Fig. 3.24) images enable us
to demonstrate the clear shape and position of VSD with surrounding papillary
muscles and structures in the ventricle. Moreover, by observing the VSD from
multiple angles, 3D-reconstructed images give more detailed and precise 3D spatial
recognition. This could be for the educational use to understand the characteristics
of VSD in a case with crisscross heart.
65
Fig. 3.22 Case: Situs solitus, concordant crisscross heart, DORV, PS, post-Fontan procedure. The
sequential axial section images of 3D-SSFP MRI using whole-heart protocol from the abdominal
level to pulmonary artery level. The images are arranged from the left upper corner at the level of
diaphragm to the right lower corner at the level of pulmonary artery
Fig. 3.23 3D anatomy of concordant crisscross heart after Fontan procedure. 3D-reconstructed
whole anatomy in a 20-year-old man with a situs solitus, concordant crisscross heart, double outlet
right ventricle, and pulmonary stenosis having a modied Fontan procedure. The small left
ventricle is located that is lower and to the right of the right ventricle. The aorta arose from the
left-superior-located right ventricle. The blue-colored part is a venous chamber of Fontan route
66
S. Yasukochi
Fig. 3.24 3D intracardiac anatomy of concordant crisscross heart after Fontan procedure, viewed
from the left upper aspects to visualize VSD with trabecula and surrounding structures
3.6
Future Direction: Simulation and Virtual Surgery

from MRI
A precise understanding of the anatomical structures of the heart, especially

intracardiac anatomy, is essential for successful treatment of CHD [2328]. Recent
advances in high-resolution 3D MRI provide a new means to virtually reconstruct
the patient-specic intracardiac morphology which can allow the visualization from
any angles of view or perspective and be integrated in the preoperative planning
process [23, 2628].
However, just a look for the reconstructed intracardiac anatomy from MRI on
screen may have some discrepancies between the reconstructed images and the
real-world structures in the surgery in the light of detailed anatomy and textures.
Several people reported a simulating system for preoperative planning in complex CHD from the reconstructed 3D images with high delity and resolution either
by enhanced computed tomography or MRI.
67
Shiraishi et al. proposed tangible replicas of complex CHD by stereolithography,

which is a rapid prototype technology whereby an ultraviolet laser beam selectively
polymerizes and solidies photosensitive and polymeric liquid plastic. They
invented a soft model of CHD made of rubberlike urethane [23]. This rubberlike
urethane biomodels are applied for the purpose of anatomical diagnosis and simulation for preoperative surgical planning and surgery. The delity of this biomodel
has been improving to replicate the almost-real 3D spatial intra- and extracardiac
structures and wall thickness but the heart valves with their accessory apparatus
such as tendineae and papillary muscle because of the limitation of an ECG-gated
enhanced computed tomography they use.
Srensen et al. proposed a virtual cardiotomy and surgical simulation based on
3D MRI for preoperative planning and surgical practice [2628]. After obtaining
the voxel data set by the isotropic state free precession acquisition protocol, they
reconstructed a virtual model of the myocardium and vessel borders using Virtual
Reality Heart software and custom software (Fig. 3.25). After adding the information of elastic properties of the modeled tissue, they invented a surgical simulating system providing the exact 3D position and orientation with tangible
sensation and force feedback simulated as a real surgical procedure.
Fig. 3.25 A virtual cardiotomy from 3D MRI reconstruction. Cited from Sorensen et al. [27]. A
case with complete atrioventricular septal defect, DORV, transposition of the great arteries,
valvular and subvalvular pulmonary stenoses, and a left superior vena cava. (a) Virtual reconstruction looking into the right ventricle and left ventricle from the apex, which has been cut away.
The outow tracts of the aorta and main pulmonary artery are visualized relative to the two
ventricular septal defects. (b) Virtual cardiotomy; the surgeons view. An incision is made from
the root of the aorta through the right ventricle revealing the exact location and course of the two
ventricular septal defects. AO aorta, LSVC left superior vena cava, LV left ventricle, MPA main
pulmonary artery, RA right atrium, RV right ventricle, SVC superior vena cava, VSD ventricular
septal defects. A 3-year-old girl with complete atrioventricular septal defect, DORV, transposition
of the great arteries, valvular and subvalvular pulmonary stenoses, and a left superior vena cava
68
S. Yasukochi
By their simulating system, a virtual surgical incision and cardiotomy can be

applied by the operators preference at any place and angles. The intracardiac
anatomy can be viewed and replicated just like a real surgery from the imaginary
incision in the simulation model [27, 28].
They can also use this simulation model for educational purpose. This simulation model system enables to illustrate various elements of surgical procedures and
allow surgeons to rehearse these elements virtually.
3.7
Summary
Recent advanced technology and software of MRI opens a door for the new horizon
of three-dimensional assessment of intracardiac anatomy in a complex CHD, not
only from the morphological point of view but also from the practical point of view
as a simulation.
We must take advantage of this highly powerful imaging tool to dissect each
segment of intracardiac anatomy of the heart in a patient-specic base, with care of
an artifact.
We could foresee this modern imaging technology providing a new surgical
procedure in near future for untreatable complex CHD at present.
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19. Haddad F, Hunt SA, Rosenthal DN, Murphy DJ (2008) Right ventricular function in cardiovascular disease, Part I: anatomy, physiology, aging, and functional assessment of the right
ventricle. Circulation 117:14361448
20. Vanpraagh R, Vanpraagh S, Vlad P, Keith JD (1964) Anatomic types of congenital
dextrocardia: diagnostic and embryologic implications. Am J Cardiol 13:510531
21. Van Praagh R, Van Praagh S (1966) Isolated ventricular inversion: a consideration of the
morphogenesis, denition and diagnosis of nontransposed and transposed great arteries. Am J
Cardiol 17(3):395406
22. Srensen TS, Beerbaum P, Korperich H, Pedersen EM (2005) Three-dimensional, isotropic
MRI: a unied approach to quantication and visualization in congenital heart disease. Int J
Cardiovasc Imaging 21:283292
23. Shiraishi I, Tmagishi M, Hamaoka K, Fukuzawa M, Yagihara T (2010) Simulative operation
on congenital heart disease using rubber-like urethane stereolithographic biomodels based on
3D datasets of multislice computed tomography. Eur J Cardiothorac Surg 37:302306
24. Greil GF, Wolf I, Kuettner A, Fenchel M, Miller S, Martirosian P, Schick F, Oppitz M,
Meinzer HP, Sieverding L (2007) Stereolithographic reproduction of complex cardiac morphology based on high spatial resolution imaging. Clin Res Cardiol 96:176185
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25. Riesenkampff E, Rietdorf Y, Wolf I, Schnackenburg B, Ewert P, Huebler M et al (2009) The

practical clinical value of three-dimensional models of complex congenitally malformed
hearts. J Thorac Cardiovasc Surg 138:571580
26. Srensen TS, Greil GF, Hanse OK, Mosgaard J (2006) Surgical simulationa new tool to
evaluate surgical incisions in congenital heart disease? Interact Cardiovasc Thorac Surg
5:536539
27. Srensen TS, Beerbaum P, Mosgaard J, Rasmusson A, Schaeffer T, Austin C, Razavi R, Greil
GF (2008) Virtual cardiotomy based on 3-D MRI for preoperative planning in congenital heart
disease. Pediatr Radiol 38:13141322
28. Srensen TS, Mosgaard J, Greil GF, Miller S et al (2007) Virtual cardiotomy for preoperative
planning. Circulation 115:e312
Chapter 4
Assessment of Extracardiac and Intracardiac

Anatomy by MD-CT
Kenji Waki
Abstract Due to recent advances in multidetector computed tomography
(MD-CT) technology, MD-CT images allow evaluation of both extracardiac and
intracardiac anatomy. The images provide high-quality spatial and temporal resolution and are highly reproducible irrespective of acoustic window parameters.
MD-CT has been shown to be very useful in the eld of congenital cardiovascular
malformations, especially in complex anomalies. Furthermore, it can less
invasively provide information about not only target lesions but also their surrounding structures including airways and their spatial relationships.
Nowadays, MD-CT provides surgeons and catheter interventionalists with precise diagnostic information as well as important information for both preoperative
and postoperative management and catheter intervention.
The clinical role and usefulness of MD-CT in the evaluation of children and
adults with congenital heart disease for both preoperative and postoperative management and catheter intervention are discussed and illustrated with several
examples.
Keywords Cardiac anatomy Catheter intervention Congenital heart disease
MD-CT Perioperative management
4.1
Background
Multidetector computed tomography (MD-CT) is an emerging imaging modality

for cardiovascular diagnosis in congenital heart disease [15]. Although echocardiography is still a primary, noninvasive imaging technique for the evaluation of the
anatomy of patients with congenital heart disease, it cannot always provide clear
images, especially for the evaluation of extracardiac structures, due to poor acoustic
windows [24]. On the other hand, MD-CT allows evaluation of the spatial
relationships between target lesions and their surrounding structures, including
airways, and can depict stenotic lesions in medium- or small-sized vessels, with
K. Waki, MD (*)
Department of Pediatrics, Kurashiki Central Hospital, Miwa 1-1-1, Kurashiki,
710-8602, Japan
e-mail: kw6169@kchnet.or.jp
Springer Japan 2015
DOI 10.1007/978-4-431-54355-8_4
71
72
K. Waki
high spatial resolution as well as high reproducibility. Furthermore, MD-CT images

can be reformatted in two and three dimensions [1, 6, 7]; therefore, surgeons or
catheter interventionalists can develop a more denitive strategy for surgical
operations or catheter interventions in patients with congenital heart disease [1, 8].
This chapter discusses and illustrates the role of MD-CT in the evaluation of
children and adults with congenital heart disease in pre- and postoperative management and catheter intervention with several examples.
4.2
4.2.1
Assessment of Extracardiac Anatomy

Aortic Arch Anomaly
MD-CT is extremely useful in the diagnosis of aortic arch anomaly. In the preoperative evaluation of coarctation of the aorta (Figs. 4.1 and 4.2), it is necessary to
assess not only the coarctation site but also the diameter of proximal and distal arch
and the isthmus. Such information is essential for surgeons to perform aortic arch
repair, such as extended end-to-end anastomosis [7, 9]. In the diagnosis of interruption of aortic arch (Fig. 4.3), MD-CT can provide valuable information about
which site is interrupted and the distance between the proximal and distal aortic
arch and the presence of branch anomalies [10, 11].
Double aortic arch is one of the vascular rings that encircle the trachea and
esophagus. It causes extrinsic airway obstruction (Figs. 4.4 and 4.5) that can be
Fig. 4.1 Coarctation of the aorta in a neonate is shown by 3D volume-rendered (VR) MD-CT
images. (a) VR image shows coarctation of the aorta and a long, hypoplastic arch between the left
common carotid artery and left subclavian artery. (b) VR image shows severe coarctation of the
aorta and hypoplastic distal arch and isthmus. Note that aortic arch anatomy is quite different
among patients
4 Assessment of Extracardiac and Intracardiac Anatomy by MD-CT
73
Fig. 4.2 A 1-month-old

infant with ventricular
septal defect presented with
tachypnea and failure to
thrive. The 3D volumerendered image reveals
coarctation of aorta (arrow)
and mildly hypoplastic
distal arch
Fig. 4.3 A neonate with

interruption of the aortic
arch and ventricular septal
defect. Coronal MPR
images clearly show
interruption of aortic arch
between the left common
carotid artery (LCA) and
left subclavian artery
(LSCA). Ao ascending
aorta, BCA brachiocephalic
artery, PA main pulmonary
artery
noninvasively and accurately diagnosed with MD-CT [12]. It is important for

surgeons to know which arch is dominant, because thoracotomy is usually
performed on the side of the more hypoplastic arch. An aberrant origin of the left
subclavian artery associated with the right aortic arch can be missed on echocardiography; however, it can be easily diagnosed with MD-CT (Fig. 4.6), especially
with 3D reconstructed images.
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K. Waki
Fig. 4.4 A 7-month-old infant with stridor. Transaxial MD-CT images show the dominant right
aortic arch (RAo) and smaller left aortic arch (LAo), with moderate tracheal narrowing
Fig. 4.5 3D volume-rendered images of the same patient as in Fig. 4.4, viewed from the anterior
(a) and the posterior (b) perspectives. Note that the right aortic arch is larger than the left, which is
not fully opacied because of an atretic portion
4.2.2
Pulmonary Artery
The pulmonary circulation in patients with pulmonary atresia is dependent on a

patent ductus arteriosus (Fig. 4.7). Therefore, the Blalock-Taussig (BT) shunt
operation is necessary during the neonatal period to maintain pulmonary blood
ow. For preoperative evaluation, precise characterization of the pulmonary artery,
including presence of juxtaductal pulmonary artery coarctation, is necessary. If
juxtaductal pulmonary artery coarctation is present or likely to develop after BT
shunt, plasty of the pulmonary artery should be performed at the same time as BT
75
Fig. 4.6 Right aortic arch with aberrant origin of the left subclavian artery. 3D volume-rendered
images show the left subclavian artery (LSCA) originating from the most dorsal part of the aortic
arch, viewed from the anterior (a) and the posterior (b) perspectives. LCA left common carotid
artery, RCA right common carotid artery, RSCA right subclavian artery
pulmonary atresia and
ventricular septal defect. 3D
volume-rendered image
clearly shows patent ductus
arteriosus (asterisk) and
pulmonary arteries. No
juxtaductal pulmonary
artery coarctation is
demonstrated. LA left
atrium, LPA left pulmonary
artery, RPA right pulmonary
artery
shunt with the patient on cardiopulmonary bypass [13, 14]. In contrast, if

juxtaductal pulmonary artery coarctation is not present, only the BT shunt operation
is performed, and cardiopulmonary bypass is no longer necessary. Evaluation by
MD-CT allows selection of the optimal surgical strategy. Some patients with
pulmonary atresia have nonconuent pulmonary arteries (Fig. 4.8). MD-CT,
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K. Waki
Fig. 4.8 A neonate with pulmonary atresia, nonconuent pulmonary artery, and univentricular
heart. 3D volume-rendered images show discontinuity (arrow) of bilateral pulmonary arteries and
tortuous bilateral patent ductus arteriosus (asterisk), viewed from the posterior (a) and superior (b)
perspectives. LPA left pulmonary artery, RPA right pulmonary artery
Fig. 4.9 A 1-month-old infant with pulmonary atresia and univentricular heart who underwent
Blalock-Taussig shunt. 3D volume-rendered images, viewed from the anterior (a) and posterior (b)
perspectives, clearly show discrete stenosis of the right pulmonary artery (RPA)
especially 3D reconstruction, can provide information about the distance separating

the two pulmonary arteries and their spatial relation with the surrounding structures.
Thus, MD-CT makes it easier for surgeons to develop a preoperative strategy for
pulmonary artery plasty.
MD-CT is useful for noninvasive assessment of pulmonary arterial stenosis and
growth after corrective surgery or BT shunt (Figs. 4.9 and 4.10). It can indicate the
77
Fig. 4.10 A 12-year-old boy with tetralogy of Fallot who underwent intracardiac repair. 3D
volume-rendered images show bilateral pulmonary stenosis. Ao ascending aorta, LPA left pulmonary artery, LV left ventricle, mPA main pulmonary artery, RPA right pulmonary artery
exact site length and severity of stenosis. Echocardiography does not always
provide sufcient images because of a limited echo window. Therefore, precise
characterization of target lesions by MD-CT allows optimal preparation for surgery
or catheter intervention.
4.2.3
Pulmonary Vein
MD-CT is a very useful diagnostic tool in total anomalous pulmonary venous

connection (TAPVC). Although TAPVC can only be diagnosed by echocardiography, it may be difcult to determine the precise structures of pulmonary veins,
especially in mixed type (Fig. 4.11) or when TAPVC is associated with heterotaxy
syndrome (Figs. 4.12 and 4.13). In such cases, MD-CT 2D images as well as 3D
reconstruction can provide accurate anatomical information [2, 15, 16]. It is an
excellent diagnostic tool for preoperative evaluation and for guidance of the
surgical operation, because the drainage site of the pulmonary vein can be exactly
determined. Furthermore, MD-CT can provide interventionalists with important
information. In a neonate with TAPVC, stent implantation may be one of the
treatment options to relieve stenosis, which is often found at the drainage site of
pulmonary vertical veins [17]. Interventionalists must pay attention not only to the
stenotic site but also to the surrounding structures (e.g., bronchi, coronary arteries,
branches of the pulmonary vein, systemic veins, etc.) [1820]. After repair of
TAPVC, pulmonary vein stenosis may develop in some patients (Fig. 4.14), and
MD-CT can give useful information about the site and severity of stenosis.
78
K. Waki

mixed type total anomalous
pulmonary venous
connection. 3D volumerendered images show the
right lower pulmonary vein
(RLPV) and left upper
(LUPV); the left lower
pulmonary veins (LLPV)
connect to a horizontal
conuence, which leads into
a vertical vein (VV). The
VV drains into the
innominate vein (InnV).
The right upper pulmonary
vein (RUPV) drains into the
superior vena cava (SVC)
Fig. 4.12 A neonate with supracardiac total anomalous pulmonary venous connection. 3D
volume-rendered MD-CT images viewed from the posterior perspective show all four pulmonary
veins connect to a vertical vein (VV), which drains into the superior vena cava
79

infracardiac total
anomalous pulmonary
venous connection. 3D
volume-rendered MD-CT
images viewed from the
posterior perspective show
all four pulmonary veins
connected to a vertical
conuence, which leads into
a vertical vein (asterisk).
The vertical vein descends
and drains into the
portal vein
Fig. 4.14 A 2-month-old infant with total anomalous pulmonary venous connection, who developed pulmonary venous obstruction after surgical repair. (a) Transaxial MD-CT image shows
severe stenosis of the left pulmonary vein (arrow). (b) 3D volume-rendered MD-CT images ( from
behind) demonstrate the obstructed left pulmonary vein (arrow). LA left atrium, LPV left pulmonary vein, RPV right pulmonary vein
80
4.2.4
K. Waki
Coronary Arteries
MD-CT can provide us with precise images concerning the origin of coronary
arteries. In patients with anomalous origin of the left coronary artery from the
pulmonary artery (ALCAPA), the diagnosis can often be made only by echocardiography; however, this is not always the case. The left coronary artery rarely
originates from the right pulmonary artery in ALCAPA (Fig. 4.15) [21]. Such
cases may be difcult to diagnose by echocardiography only, because the left
coronary artery looks as if it originates normally from the left coronary cusp on
echocardiography. MD-CT is a very useful tool for the diagnosis of such cases.
In the eld of pediatric cardiology, echocardiography is a noninvasive and very
useful tool to visualize the coronary arteries. Echocardiography is useful in the
diagnosis of enlarged lesions; however, there are some limitations, such as the
diagnosis of stenotic lesions in the coronary arteries. Furthermore, it is more
difcult to obtain clear images in adolescents or patients with chest deformities
such as pigeon chest. However, MD-CT can depict small structures such as the
coronary arteries with improved spatial resolution because of increased numbers of
detector rows [2225]. Multiplanar reformatted (MPR), maximum-intensity projection (MIP), and 3D volume-rendered (VR) images are powerful tools that
provide additional information on the nature and extent of disease and accurately
illustrate anatomical relationships (Figs. 4.16, 4.17, 4.18, 4.19, and 4.20).
Fig. 4.15 A 2-month-old infant presented with severe congestive heart failure. (a) Transaxial
MD-CT image shows an anomalous origin of the left coronary artery (arrow) from the right
pulmonary artery (RPA). (b) 3D volume-rendered MD-CT image clearly shows an anomalous
origin of the LCA from the RPA
81
Fig. 4.16 A 14-year-old boy presented with chest pain on exercise. (a) Transaxial MD-CT image
demonstrates discontinuity between the ascending aorta (Ao) and left coronary artery (LCA),
which is hypoplastic. He was diagnosed with congenital ostial atresia of the LCA. (b) Curved
multiplanar reconstruction image shows an intact right coronary artery
Fig. 4.17 3D volume-rendered image shows the status of the patient in Fig. 4.16 after a left
internal mammary artery bypass graft (arrow) to the diagonal coronary artery
82
K. Waki
Fig. 4.18 A 28-year-old man with tetralogy of Fallot and pulmonary atresia who underwent
Rastelli operation. (a) Slab maximum-intensity projection (MIP) image shows stenosis at the
origin of right coronary artery and left anterior-descending coronary artery (LAD), which anomalously originates from the right coronary cusp and has an interarterial course between the aorta
(Ao) and Rastelli conduit (in blue). (b) 3D volume-rendered image also shows the interarterial
course of the LAD
Fig. 4.19 Curved multiplanar reconstruction images (the same patient as in Fig. 4.18) show the
entire course of the right coronary artery (in (a)) and left anterior-descending coronary artery
(in (b)). Note that only the origins of both coronary arteries are stenotic
83
Fig. 4.20 An 18-year-old man with transposition of the great arteries who underwent arterial
switch operation. (a) Transaxial multiplanar reformatted CT image shows obstruction of main
trunk (arrow) of the left coronary artery (LCA). (b) 3D volume-rendered CT image ( from above).
Ao ascending aorta, RCA right coronary artery
4.2.5
Systemic Venous Return
Anomalies of systemic venous return may be associated with congenital heart

disease [26]. The general population without congenital heart disease may have a
left superior caval vein (LSVC); however, it is more prevalent in individuals with
congenital heart disease. The LSVC usually drains into the coronary sinus; however, it may drain into the left atrium or atrial roof (Fig. 4.21). It is very important to
evaluate its drainage site and presence of the innominate vein for intracardiac
repair. When the LSVC drains into the left atrium, atrial septation results in
desaturation. MD-CT can illuminate the precise site of LSVC drainage.
4.2.6
Airways (Spatial Relationship with the Great Arteries)
In patients with congenital heart disease, anomalous vessels and previous surgical
operations may cause airway obstruction [3, 6, 27, 28]. MD-CT can provide information on not only abnormal arteries or veins but also the structures of the trachea,
bronchi, and coronary arteries. Pulmonary artery sling (anomalous origin of the left
pulmonary artery from the right pulmonary artery) causes compression of the trachea
by the anomalous origin of the left pulmonary artery (Fig. 4.22) [3, 6, 28]. 3D
images superimposed on the obstructed airway show the spatial relationships.
In patients with tetralogy of Fallot and absent pulmonary valve, the airway is compressed and obstructed by the dilated main and peripheral pulmonary arteries
84
K. Waki
Fig. 4.21 A 9-month-old infant with partial atrioventricular septal defect. (a) Coronal multiplanar
reformatted MD-CT image shows the left superior vena cava (arrow) draining not into coronary
sinus but into the roof of left atrium (LA). (b) Oblique sagittal 3D volume-rendered CT image. LV
left ventricle
Fig. 4.22 A 1-year-old girl with pulmonary artery sling. (a) Transaxial MD-CT image clearly
shows the left pulmonary artery originating from the right pulmonary artery and surrounding and
compressing the trachea (arrow). (b) 3D volume-rendered image provides accurate spatial relationships between the pulmonary arteries and airway (in blue). Ao ascending aorta, mPA main
pulmonary artery, LPA left pulmonary artery
(Fig. 4.23) [3]. The site of airway obstruction can be clearly visualized with MD-CT,
especially 3D images superimposed on images of the airway (Fig. 4.24). In patients
with double aortic arch, the airways are surrounded and compressed by both aortic
arches (Fig. 4.25).
85
Fig. 4.23 A 6-month-old infant with tetralogy of Fallot with absent pulmonary valve. (a)
Transaxial MD-CT image shows marked dilation of the pulmonary artery, especially the left
pulmonary artery (LPA). The left main bronchus (arrow) is compressed by the dilated LPA. (b)
Coronal 3D volume-rendered MD-CT image (viewed from the posterior perspective) clearly
shows the dilated pulmonary artery
Fig. 4.24 A case of tetralogy of Fallot with absent pulmonary valve. (a) Coronal 3D volumerendered MD-CT image (viewed from the posterior perspective) shows dilated pulmonary arteries.
(b) The site of airway obstruction can be clearly visualized with 3D volume-rendered MD-CT
images superimposed on images of the airway (in blue). LPA left pulmonary artery, RPA right
pulmonary artery
4.2.7
Catheter Intervention
MD-CT images are extremely useful for planning catheter interventions, because
they can provide interventionalists with information about whether the planned
catheter intervention is indeed necessary, the technical feasibility of the
86
K. Waki
Fig. 4.25 Double aortic

arch (same patient as in
Figs. 4.4 and 4.5). Cropped
3D volume-rendered image
superimposed on an image
of the airways. Note that the
trachea is surrounded and
compressed by the double
aortic arch. RL right lung,
LL left lung
Fig. 4.26 An 11-year-old

boy with univentricular
heart who underwent total
cavopulmonary connection.
Transaxial MD-CT image
shows a long segment of
stenosis in the left
pulmonary artery (arrow).
Dilation of left pulmonary
artery by transcatheter stent
implantation should not be
performed, because it is
likely to compress the left
bronchus (LB)
intervention, and the type of catheters or devices that should be used. Furthermore,
MD-CT images can provide interventionalists with important information about the
spatial relationship of the target lesion with its surrounding structures. If the
trachea, bronchus, or coronary arteries are near the lesion, extrinsic compression
may cause lethal complications [1820]. When extrinsic compression by stent
implantation is expected, surgical repair should be recommended rather than
catheter intervention (Fig. 4.26). Furthermore, the use of MD-CT before the
procedure may shorten the uoroscopy time as well as the total time required for
the procedure.
87
Fig. 4.27 An 8-year-old girl with extracardiac total cavopulmonary connection. Filling defects
are shown by a transaxial MD-CT image (arrow in (a)) and oblique sagittal multiplanar
reformatted MD-CT image (arrow in (b)). In-stent stenosis was suspected; however, no stenosis
was demonstrated by angiography
4.2.8
Limitations
There are limitations in the use of MD-CT for diagnosis in patients with right heart
bypass operations, such as bidirectional Glenn shunt or Fontan operations. MD-CT
images of pulmonary arteries may be misdiagnosed as pulmonary stenosis, because
they may not be fully opacied with contrast media due to the lack of an appropriate
venous mixing chamber (Fig. 4.27) [29, 30]. For the diagnosis of atretic vessels
such as ligaments by MD-CT, it should be recognized that such vessels cannot be
opacied with contrast media [3].
4.3
4.3.1
Assessment of Intracardiac Anatomy

Situs Anomalies with Complex Congenital Heart
Disease
MD-CT can be used to identify visceroatrial situs, looping of the great arteries, or
the position of the atrial appendages (Fig. 4.28). 2D-reformatted and
3D-reconstructed images can provide more accurate information about anatomy
and spatial relationships (Fig. 4.29).
88
K. Waki
Fig. 4.28 A 17-year-old man with congenitally corrected transposition of the great arteries. (a)
and (b) Transaxial MD-CT images show atrial situs inversus, discordant atrioventricular connection, and a descending aorta on the right side of the vertebra. (c) Coronal multiplanar reformatted
MD-CT shows that the ascending aorta (AAo) originates from the morphological right ventricle
(RV) on the right side, and the pulmonary artery originates from the morphological left ventricle
(LV). DAo descending aorta, PA main pulmonary artery, RAA right atrial appendage
Fig. 4.29 A 52-year-old man with congenitally corrected transposition of the great arteries
presented with congestive heart failure. (a) Transaxial MD-CT image shows atrial situs solitus
and discordance of the atrioventricular connection. The ventricle on the left side is dilated, has
coarse trabeculations, and is considered a morphological right ventricle. (b) Coronal 3D volumerendered CT images can provide accurate spatial relationships of the structures
89
Fig. 4.30 Rupture of the aortic sinus of Valsalva in a 49-year-old man who presented with
congestive heart failure. Subarterial and doubly committed VSD and protruded right coronary
cusp with deformity are shown by sagittal oblique (a) and coronal oblique (b) multiplanar
reformatted MD-CT. Ao ascending aorta, PA main pulmonary artery, RV right ventricle
4.3.2
Ventricular Septal Defect
Subarterial and doubly committed VSD with aortic cusp prolapse may develop
rupture of the aortic sinus of Valsalva [31, 32]. Although this may be diagnosed by
echocardiography, it is not always possible in adult patients, especially in larger
patients because of the lack of an adequate acoustic window. MD-CT can accurately depict the location of protruded and ruptured aortic cusps regardless of body
size (Fig. 4.30).
In muscular VSD, especially apical VSD, clear images that show the size and
number of defects cannot always be obtained on echocardiography. MD-CT can
provide accurate images of muscular VSDs, even in the apex (Fig. 4.31) [4], and
enables surgeons to make a decision about whether the VSD should be closed and
the best surgical approach to achieve closure.
4.3.3
Postoperative Complications in the Fontan Operation
Various complications may develop after the Fontan operation such as arrhythmia,
pathway obstruction, right atrial enlargement (Fig. 4.32) and thrombus formation,
or right heart failure [3, 4]. MD-CT can provide information about patency of the
pathway including stenotic lesions or thrombus formation, which is shown by
lling defects.
90
K. Waki
Fig. 4.31 Muscular VSD. Transaxial MD-CT images show a large perimembranous VSD (asterisk in (a)) and interventricular communication in the muscular part of the interventricular septum
at the apex of the heart (arrow in (b))
Fig. 4.32 A 24-year-old man with tricuspid atresia and a classic Fontan operation who developed
syncope due to atrial tachycardia. Marked enlargement of the right atrium (RA) is shown by
transaxial MD-CT images (a) and by coronal oblique multiplanar reformatted MD-CT images (b)
4.3.4
Cardiac Electrophysiologic Study and Radiofrequency

Catheter Ablation (CARTO Merge)
With recent advances of technology in the eld of cardiac electrophysiology, CT

images can be merged with an electroanatomical mapping system (CARTO system). Transferring MD-CT images to the CARTO system allows reconstruction of
3D images, which can be rotated and viewed from any angle on a computer screen.
It is very useful for performing cardiac electrophysiology studies and
radiofrequency catheter ablation, especially in cases of complex congenital heart
disease (Figs. 4.33 and 4.34) [33, 34]. Furthermore, the cropping function allows
91
Fig. 4.33 A 38-year-old man with transposition of the great arteries, ventricular septal defect, and
pulmonary stenosis, who underwent the Rastelli operation. He developed atrial tachycardia.
Transaxial CT images show juxtaposition of right atrial appendage (asterisk) to the left atrial
appendage (LAA). LA left atrium, LV left ventricle, RA right atrium
Fig. 4.34 Electroanatomical mapping (CARTO) system (the same patient as in Fig. 4.33). (a)
3D-reconstructed image on the CARTO system screen by transferring and merging MD-CT
images. Note that the right atrial appendage (RAA) is located on the juxtaposition of the left atrial
appendage (LAA). (b) Propagation mapping of the right atrium (RA). The atrial tachycardia was
diagnosed as focal atrial tachycardia, with the focus in the lower lateral portion of the RA (in red)
the visualization of all cross sections; this makes it possible to understand the
spatial relationships between a VSD or ASD and the great arteries or atrioventricular valve annulus. In addition, these images make it easier for cardiac electrophysiologists to manipulate catheters during radiofrequency catheter ablation.
92
4.3.5
K. Waki
3D Biomodels Based on MD-CT Datasets
Because of improving spatial resolution by increasing numbers of detector rows and

temporal resolution by faster gantry rotation with MD-CT, high-contrast 3D images
have recently become available, even from the contracting heart. Therefore, some
studies [35, 36] have created plastic replicas based on MD-CT datasets. Shiraishi
et al. [36] reported that by using the stereolithographic technique, MD-CT 3D
volumetric image data could be converted into plastic models, which may be useful
in evaluating the complex anatomy of congenital cardiovascular malformations.
These models may have substantial impact on the accurate diagnosis, planning, and
practical simulation of surgical or catheter interventions and may also be used as an
instructive tool for patients with complex congenital heart disease (Fig. 4.35).
Fig. 4.35 MD-CT images ((a) and (c)) and manufactured biomodels ((b) and (d)). (a), (b) A
2-month-old infant with isolated coarctation of aorta. (c), (d) A 28-day-old neonate with doubleoutlet right ventricle associated with subpulmonary ventricular septal defect. aAo ascending aorta,
CoA coarctation of the aorta, dAo descending aorta, LA left atrium, LPA left pulmonary artery,
LPV left pulmonary vein, LV left ventricle, PA pulmonary artery, RA right atrium, RV right
ventricle, SVC superior vena cava (modied from Kim et al. [35])
4.4
93
Summary
MD-CT plays an increasing role in perioperative management and catheter intervention in congenital cardiovascular disease, because it provides high-quality
images and information about target lesions as well as their surrounding structures.
MD-CT provides images with superior spatial and temporal resolution that do not
depend on acoustic window parameters. Furthermore, the advancement of technology will shift two-dimensional visualization to three-dimensional, even for complex congenital cardiovascular disease. It should contribute to further improve
outcomes in these patients.
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Part II
Functional Assessment of Congenital Heart

Disease
Chapter 5
Assessment of Ventricular Function Using

the Pressure-Volume Relationship
Satoshi Masutani and Hideaki Senzaki
Abstract Pressure-volume relationships clearly demonstrate loading conditions
(preload and afterload) and cardiac function (systolic and diastolic functions) as
well as those relationships in a single plane. These advantages are greatly helpful to
understanding the hemodynamics of patients with congenital heart disease, in
which loading conditions as well as intrinsic heart functions may become greatly
altered on medical, catheter, or surgical interventions. Stroke volume and
end-systolic pressure (Pes) result from the balance between contractility and
afterload, i.e., between end-systolic elastance (Ees) and effective arterial elastance
(Ea) in a given preload. Ventricular-arterial coupling (Ees/Ea or Ea/Ees) is closely
related to heart energy. The position and the slope of end-diastolic pressure-volume
relation provide information about intrinsic myocardial stiffness, although it is also
affected by factors outside the left ventricle (external constraint). While obtaining
the actual pressure-volume loops requires invasive measurement, a noninvasive
pressure-volume assessment is possible upon consideration of all clinical symptoms
and history. This noninvasive approach also helps clinicians select an optimal
therapy and assess the consequent changes based upon the evaluation of each factor
and those interactions. Hence, the importance of understanding this classic
concept remains unchanged for pediatric cardiologists managing the complicated
hemodynamics of congenital heart disease.
Keywords Congenital heart disease Contractility Pressure-volume loops
Pressure-volume relation Ventricular-arterial coupling
5.1
Introduction
Most patients with congenital heart disease have abnormalities in loading conditions such as an abnormal pulmonary to systemic ow ratio, stenosis, or regurgitation. Such hemodynamics can change drastically in response to medical therapy,
mechanical ventilation, cardiac or noncardiac events, and surgical or catheter
S. Masutani, MD, PhD, FJCC, FAHA (*) H. Senzaki, MD, PhD, FJCC, FACC, FAHA
Department of Pediatric Cardiology, Saitama Medical Center, Saitama Medical University,
1981 Kamoda, Kawagoe-shi, Saitama 350-8550, Japan
e-mail: masutani@saitama-med.ac.jp
Springer Japan 2015
DOI 10.1007/978-4-431-54355-8_5
97
98
S. Masutani and H. Senzaki
interventions. Cardiac systolic and diastolic functions per se are intrinsic to the
heart itself and independent of loading conditions (preload and afterload) and heart
rate. The performance of the cardiovascular system depends on the interactions of
its components [1], which affect each other in complex ways. Thus, to better
characterize the pathophysiology of patients, it is essential to assess their cardiac
function and loading conditions in both separate and integrated manners. Such
assessments can be more clearly achieved by pressure-volume relationships. Thus,
an understanding of the pressure-volume relationship concept [2] is needed to
manage congenital heart disease, which may help clinicians provide optimal therapy in a tailor-made manner according to an understanding of each factor and their
interactions.
5.2
What Does a Single Pressure-Volume Loop Show?
Ea=ESP/SV
Ejection
EDP
PMVO
Pmin
SW
Filling
B
Isovolumic
Contraction
Relaxation
LV Pressure
SP
ESP
DP
LV Pressure
LV Volume or Area
A single pressure-volume loop represents one cardiac cycle (Fig. 5.1). The x-axis
displays the volume and the y-axis displays the pressure. One cardiac cycle is
shown as one counterclockwise loop. One cardiac cycle consists of four elements
that correspond to each side of the loop rectangle. The starting point of the QRS
complex in an electrocardiographic recording indicates the end of diastole and the
E
EDV
ESV
SV
EDV
LV Volume
EF=SV/EDV
AB
CD
systolic diastolic
Fig. 5.1 Pressure-volume counterclockwise loop during one cardiac cycle. See the text for details
5 Assessment of Ventricular Function Using the Pressure-Volume Relationship
99
beginning of systole (Fig. 5.1, Point A). One heart cycle can be divided into
isovolumic contraction (between Points A and B), ejection (between Points B and
C), isovolumic relaxation (between Points C and D), and ventricular lling
(between Points D and A). The former two comprise systole, while the latter two
comprise diastole (blue and red, respectively, in Fig. 5.1, right panel).
In systole, left ventricular (LV) pressure rst increases straight up to Point B
without changing LV volume because the mitral and aortic valves are closed during
the isovolumic contraction. When the LV pressure exceeds the aortic pressure, the
aortic valve opens and blood begins ejecting from the LV to the aorta. During this
ejection phase (between Points B and C), LV volume decreases and the pressurevolume curve is convex upward. The movement of blood from the LV to the aorta
ceases when the aortic valve closes (Point C; upper left-hand corner of the LV
pressure-volume loop) after the LV pressure decreases to less than the aortic
pressure. The LV pressure then decreases without a change in LV volume
(isovolumic relaxation: Point C to D). When the LV pressure decreases to the
level of the left atrial (LA) pressure (Point D), the mitral valve opens (MVO), LV
lling starts, and the LV volume begins to increase. Figure 5.2 shows the relationship between LV and LA pressures and LV lling [3]. LV relaxation persists after
the MVO; thus, the LV pressure continues to decrease despite increased LV
volume. After the LV pressure reaches its minimum (Fig. 5.1, Point E), LV pressure
Fig. 5.2 Relationship

between pressures in the left
ventricle (LV), left atrium
(LA), and lling. LV and
LA pressure and the LV
volume rate (dv/dt) were
obtained in conscious
chronically instrumented
dogs. The dv/dt shows LV
lling and LV ejection in
each phase. See the text for
details. Reprinted with
permission from Masutani
et al. [3]
100
and volume increase to the end of diastole, when the lling by atrial contractions
ceases (Point A).
The pressure-volume loop of one cardiac cycle provides useful and important
hemodynamic information. The x-axis of Points C and D represents the end-systolic
volume, while the x-axis of Points A and B represents the end-diastolic volume. The
x-axis distance between lines AB and CD represents the stroke volume (SV), while
the SV/end-diastolic volume (EDV) represents the ejection fraction (EF).
The slope of the thick-dashed line in Fig. 5.1 represents the effective arterial
elastance (Ea) [4], which indicates the relationship between the SV and the Pes. Ea
is an integrated measure of LV afterload [5, 6]. The slope of the line between Points
E and A, which is calculated by dividing the change in the pressure from the time of
minimal LV pressure to the end-diastolic pressure by the change in the volume
during this period, is dened as the LV chamber stiffness and greatly affects
ventricular lling [7, 8]. The area surrounded by one cardiac pressure-volume
loop shows stroke work (or external work) during one cardiac cycle. The dimension
of stroke work is that of energy (force times length) because it is equal to the
product of pressure (force/area) and volume (volume).
In summary, one single pressure-volume loop provides six kinds of pressure, two
kinds of volume, stroke volume, ejection fraction, stroke work, and Ea.
5.3
Noninvasive Estimation of a Single

Pressure-Volume Loop
According to the basal knowledge described in the previous section on what a

single pressure-volume loop involves, the opposite directional thinking enables
noninvasive estimation of a single pressure-volume loop (Fig. 5.3). We can use
echocardiographic measurements to determine the LV end-systolic and
end-diastolic volumes as well as the relative position of the pressure-volume loop
on the x-axis. Among the six kinds of pressure provided in Fig. 5.1, sphingomanometric pressure measurements provide systolic (top of slope between Points B
and C), mean (approximately y-axis of Point C), and diastolic blood pressure (y-axis
of point B). Pes approximately equals mean blood pressure. Doppler and tissue
Doppler echocardiographic measurements of E/e0 provide information about
whether end-diastolic pressure is elevated, although validation studies in children
were limited. Other two pressures, namely LV minimal pressure and LV pressure
on the MVO opening, cannot be determined noninvasively. Finally, with such
information taken together, the pressure-volume loop was noninvasively
constructed (Fig. 5.3). Although the inferior portion of the loop cannot be accurately estimated, such pressure-volume loops shown in Fig. 5.3 provide useful
assessments about loading conditions, functions, and those interactions in daily
bedside practice.

Fig. 5.3 Noninvasive
estimation of a single
pressure-volume loop. See
the text for details
101
, Ea=ESP/SV
SP
ESP
DP
''
----~~---' , , , : -- e .._--
------ -e:-
'
-~------
''
'
''
'
-----------'
''
''
'
'
EDP
------ _r: -
--------------- '-'-- -, ,
ESV
... .,. ...
~~ ,..
sv
EDV
' ,.'
EDV
) EF=SV/EDV
LV Volume
5.4
Multiple Loops Obtained by Changing Loads
The single-loop pressure-volume curve and the elements of the cardiac cycle are
well understood using a time-varying elastance model [9] in which the elastance of
the ventricle changes with time during the cardiac cycle. This model is analogous to
the elastic energy stored in a stretched spring; mechanical energy must be increased
within the time-varying elastance when the elastance increases within the ventricular wall according to the following equation:
Et
P t
;
V t V 0
where V0 is almost equal to the volume axis intercept of the Pes-volume relationship (ESPVR). The increasing slope of the ESPVR and, hence, the increasing
elastance during systole are analogous to a thickening spring within the ventricular
wall (Fig. 5.4) [9]. At the end of systole, the elastance generally reaches its
maximum (Emax) [9]. Although Emax is an important ventricular property, it is
somewhat difcult to use in the clinical setting. The slope of ESPVR of multiple
loops is called Ees, which is obtained independent of a timing of maximum
elastance and is more clinically useful. Thus, Ees and ESPVR rather than Emax
will be detailed in the following section as a relatively load-insensitive measure of
contractility.
It is difcult to gain load-insensitive measures of contractility or ventricular
stiffness using a single pressure-volume loop. To obtain those values, variably
loaded multiple pressure-volume loops are needed, which can be obtained by
102
Fig. 5.4 The increasing

elastance during systole is
symbolized by a thickening
spring within the ventricular
wall (time-varying
elastance model). Reprinted
with permission from Suga
et al. [9]
Fig. 5.5 Representative pressure-volume relationships during inferior vena cava occlusion before
and after the use of the calcium sensitizer levosimendan (LS), an inodilator, in a conscious
instrumented heart failure dog. Volume measurements were performed by ultrasonic crystals.
After inodilator administration, end-diastolic volume was decreased and end-systolic volume was
further decreased. Thus, stroke volume was increased. The end-systolic pressure-volume relationship was shifted to the upper left area and its slope was increased
preload or afterload modication. Figures 5.5 (experimental dog) [10] and 5.6a [11]
are examples of such multiple pressure-volume or area loops, respectively, that are
obtained by inferior vena cava (IVC) occlusion. As shown in Fig. 5.3a, the
trajectory of Points C and A in Fig. 5.1 represents the ESPVR and the end-diastolic
pressure-volume relationship (EDPVR), respectively. Ees is dened as the slope of
the ESPVR. ESPVR position and slope provide load-insensitive measures of
contractility. As shown in Figs. 5.5 and 5.6, increasing contractility induced by
inotropes causes the ESPVR position to shift upward and to the left and the Ees to
increase. In contrast, decreasing contractility causes the ESPVR position to shift
103
Fig. 5.6 Pressure-area relationships during vena cava occlusion before and after dobutamine and
contractility assessment. Similar to the pressure-volume relationship [19], the end-systolic pressure-area relationship, stroke work-end-diastolic area relationship, and dp/dt max-end-diastolic
area relationships are linear, and their slopes increase with dobutamine. Reprinted with permission
from Senzaki et al. [11]
lower and to the right and the Ees to decrease. Increased ventricular diastolic
stiffening causes a steep EDPVR in the physiologically working range regardless
of right or left position.
With respect to ESPVR nonlinearity, the Ees may differ between preload and
afterload manipulations [12]. More importantly, it cannot be overemphasized that
an understanding of the entire pressure-volume relationship is preferable to knowing just a single Ees value [12].
To change the afterload, drugs such as phenylephrine [13] have been sometimes
used in clinical settings. In light of ease, safety, quick recovery to the original state,
and repeatability, drug-induced modulations have signicant disadvantages over
transient IVC occlusion. Transient IVC occlusion seems to be the most easily
repeatable and, thus, suitable way to change load in clinical settings. In contrast
to the use of drugs to change load, IVC occlusion does not take a long time (usually
about 5 s of ination time) and balloon deation can be quickly completed. During
the simultaneous measurement of LV pressure and volume/area of catheterization,
transient IVC occlusion can be safely performed in both adults [14] and children
[11, 15].
Newly developed balloon catheters for IVC occlusion with a reasonable size
(57 Fr) for use in all pediatric and adult patients [11, 15] have contributed to the
safety and easy applicability of this procedure (Fig. 5.7). A balloon catheter is
usually introduced from the femoral vein through an appropriately sized sheath and
advanced into the right atrium under uoroscopic guidance. The balloon is inated
with CO2 gas in the right atrium and then withdrawn toward the IVC, thus
obstructing venous inow [15]. Blood pressure recovers quickly enough after the
procedure.
104
Fig. 5.7 Occlusion balloon catheter that can be inserted in a regular sheath for pediatric catheterization (57 Fr). Reprinted with permission from Senzaki et al. [15]
5.5
Pressure and Volume Measurement Methodology
For accurate pressure-volume analyses, LV pressure is preferably measured by a

micromanometer due to the considerable limitations in pressure waveforms
obtained by heparinized saline-lled catheters that are connected to the transducers
(Fig. 5.8). For pediatric catheterization, pressure transducers mounted on a 0.014-F
guidewire (RADI Medical Systems AB, Uppsala, Sweden), which can be placed in
a 3- to 5-F pigtail catheter, are useful for obtaining accurate measurements of LV
pressure [11]. Measurement by a pressure guidewire provides more accurate waveform information without false uctuations that may result in inaccurate analysis in
Ees, relaxation, and stiffness (Fig. 5.8).
On the other hand, obtaining accurate and continuous LV volume measurements
has been challenging. Although volumetry by cineangiograms has been used to
construct human pressure-volume loops, it takes huge amounts of time to measure
the LV volume by determining the border of the LV cavity frame by frame.
Moreover, the frame rate on cineangiograms is too low to enable precise
pressure-volume analyses. If the frame rate were increased, the radiation exposure
would be increased as well. The development of a conductance catheter has
overcome these issues and enabled a relatively feasible way to continuously
monitor LV volume [14] with simultaneous measurements of LV pressure. This
is currently one of the most frequently used tools for pressure-volume data acquisition in adult clinical settings. However, in small children, conductance catheters
cannot be used due to catheter size limitations. Moreover, based on the measurement principle and its geometric assumption of the conductance catheter, it should
a
120
Water
100
80
60
40
Wire
20
0
-20
110
110
90
90
Pressure (mmHg)
b
Pressure (mmHg)
Fig. 5.8 Comparison of

pressure measurements
using a pressure guidewire
and a saline-lled catheter.
(a) Analog recordings of the
left ventricular pressure
waveform using a pressure
guidewire (orange) and a
saline-lled catheter
(black). The waveform by
saline-lled catheter (black)
had unphysiological
uctuations. (b) The
pressure-area relationship
during inferior vena cava
occlusion by pressure
measurements using a
saline-lled catheter (left
panel) and a pressure
guidewire (right panel)
demonstrating the
usefulness of pressure
measurements using a
pressure guidewire
105
70
50
30
50
30
10
10
-10
70
-10
0
Area (cm2)
Area (cm2)
be difcult to accurately measure ventricular volume in patients with large ventricular septal defects or a single right ventricle.
Because of such difculties in continuous volume recordings in children,
LV areas have sometimes been used in clinical settings to generate pressure-area
loops. Although caution should be exercised, pressure-area relationships (Fig. 5.5)
[11] provide essentially the same physiological evaluations as pressure-volume
relationships in the physiological range as previously validated. Pressure-area
relationships, which can be less invasively applied to small children, elucidate
the complicated hemodynamics in complex congenital heart disease. This continuous measurement of ventricular area may overcome the aforementioned
limitations of conductance catheter measurement in children. Although volume
measurement on magnetic resonance imaging (MRI) is the best methodology since
it does not depend on geometrical assumptions, pressure-volume analyses
employing MRI [16, 17] are currently under development and await further
progress.
106
5.6
Assessment of Systolic Function
Indices of systolic function or ventricular contraction that can be derived from the
pressure-volume loop during one cardiac cycle, such as EF and dp/dtmax, are loaddependent indices and, thus, are not purely intrinsic systolic functions. In contrast,
three indices of the Ees, MSW (the slope of stroke work [SW] to end-diastolic
volume), the slope of dp/dtmax, and end-diastolic volume relationships [18], are
highly load-independent; hence, they are useful for assessing ventricular function
independent of loading condition [18]. These three relationships in pressurevolume correlations are linear in physiological ranges as well as the increases in
slope in response to increased contractility by dobutamine [19]. Similarly, as shown
in Fig. 5.6, these three relationships have also been obtained in pressure-area
relationships in children [11]. Among these three relationships, the strong points
of MSW are that its dimensions consist of mmHg in pressure-volume, pressure-area,
or pressure-dimension relationships and that adjustment by body size is unnecessary in contrast to the other two indices, which require body size correction. Among
these three indices, MSW is the most stable but the least sensitive to changes in
inotropic states, whereas the slope of dp/dtmax and the end-diastolic volume relationship are the most sensitive but most variable measures of the contractile state
[19]. Among these, Ees has advantages over the other two indices in that it can be
used to assess ventricular-arterial coupling, which is directly related to heart
energy, and predict systemic pressure/stroke volume responses to afterload or
preload reduction therapy [12, 20, 21]. This issue will be described in the next
section.
5.7
Assessment of Ventricular-Arterial Coupling
The LV pumps blood into the artery, which then delivers it to the tissues. To
effectively achieve this, the relationship between the ventricular and arterial system, or ventricular-arterial coupling, is an important determinant. This ventriculararterial coupling is quantied by the ratio between ventricular and arterial elastance
expressed as Ea/Ees (or Ees/Ea). Given the preload, which is dened as
end-diastolic volume (Fig. 5.1), the SV and the Pes result from the balance between
Ees (describing the ventricle) and Ea (describing the arterial system) [1].
The SW is the external work of the heart during one cardiac cycle (represented
by the shadowed area in Figs. 5.1 and 5.9). The pressure-volume area (PVA) is
dened as the area circumscribed by the ESPVR, EDPVR, and systolic segment of
the pressure-volume trajectory (Fig. 5.9). The area under the ESPVR and to the left
107
Ea:ESP/ SV
Ees
Ea=ESP/SV
Ees
LV Volume
PVA=PE +SW
Fig. 5.9 Concept of pressure-volume area (PVA). (a) PVA in schematic presentation and (b)
PVA in a real pressure-area relationship. The potential energy (PE) is dened as the area under the
end-systolic pressure-volume relationship (ESPVR), above the end-diastolic pressure-volume
relationship (EDPVR), and to the left of the pressure-volume loop. PVA is the sum of PE and
stroke work (SW) or external work (EW)
of the SW area is the potential energy (PE) and is expressed according to this
equation:
PVA SW PE:
PVA represents the total mechanical energy that is produced by the LV. The
efciency of the conversion of mechanical energy to external work of the heart is
calculated as SW/PVA [22]. The mechanical efciency (SW/MVO2) of the LV can
be expressed as the product of the ratio of PVA to MVO2 (the conversion of
metabolic energy to mechanical energy) and the ratio of SW to PVA (the conversion of mechanical energy to external work) as follows [23]:
SW=MVO2 PVA=MVO2 SW=PVA;
where SW is approximated by SV Pes. The efciency of SW/PVA and
ventricular-arterial coupling is a tight relationship expressed as follows:
SW=PVA 1=1 0:5 Ea=Ees:
This equation shows that the efciency of the conversion of mechanical energy
to external work of the heart is approximately determined by ventricular-arterial
coupling and explains its importance.
Suga et al. claried the close relationship between the metabolic energy of the
heart (MVO2) and PVA [9], which established the integrated concept of heart
108
energy in pressure-volume analyses based upon the following time-varying

elastance model:
MVO2 a PVA b a PVA c Emax d;
where a PVA corresponds to the PVA-dependent VO2 and b corresponds to the
PVA-independent VO2. Because b changes with Ees, b can be written as the sum of
c Ees + d. The variable a indicates the O2 cost of PVA, c indicates the O2 cost of
Ees, and d indicates basal metabolism [9]. Thus, PVA is a measure of the total
mechanical energy that is generated by each ventricular contraction. This PVA
concept is an important extension of the Ees concept.
The left ventricle and arterial system are optimally coupled to produce SW when
Ea/Ees 1.0. When Ees exceeds Ea (Ea/Ees < 1.0), SW remains nearly optimal,
but when Ea exceeds Ees (Ea/Ees > 1.0), SW falls and the LV becomes less
efcient [24]. In normal subjects, the LV and arterial system are optimally coupled
both at rest and during exercise [22, 25]. In contrast, in patients with systolic heart
failure, Ees is reduced and peripheral vascular resistance and Ea are increased, as
the LV and arterial system are suboptimally coupled (Ea/Ees > 1.0). Because Ea is
approximately equal to the peripheral vascular resistance times the heart rate, any
increase in heart rate will further increase Ea, making the coupling even worse
[26, 27].
5.8
Assessment of Diastolic Function
Diastole consists of isovolumic relaxation and lling. Thus, diastolic function is

assessed in two phases.
5.8.1
Isovolumic Relaxation
Early diastolic (from end systole to MVO; Fig. 5.1, Points C to D) function is
relaxation: how fast the LV can relax and the LV pressure can decrease. Early
diastolic function can be assessed by the time constants of relaxation. The LV
pressure of this phase is approximately tted to a monoexponential curve. Thus,
relaxation can be assessed by the time constant () of the monoexponential curve
with a zero asymptote and a nonzero asymptote [28]. To better t the LV pressure,
the logistic t has been developed to obtain an accurate and robust t [2931]. If LV
relaxation is severely impaired, it may develop a characteristic change in the
diastolic pressure-volume relationship [32]. However, it seems difcult to precisely
evaluate an abnormality in relaxation from an actual pressure-volume curve.
5.8.2
109
Filling
Late diastole (from MVO to end-diastole; Fig. 5.1, Points D and A) is the lling
duration. The late diastolic function of LV consists of how the LV can easily
receive blood from the left atrium. Such an ability is represented by compliance
(V/P). Stiffness (P/V ), the reciprocal of compliance, indicates how much
pressure is needed to increase the unit volume. The slope of the line between Points
E and A in Fig. 5.1, which is calculated by dividing the change in the pressure from
the time of minimal LV pressure to end-diastolic pressure by the change in the
volume during this period, is dened as LV chamber stiffness [7, 8]. LV chamber
stiffness can be noninvasively assessed by the deceleration time of early mitral
inow velocity (E wave) in echocardiography, which seems more useful in cases of
restrictive physiology with increased left atrial pressure. LV chamber stiffness may
have a greater impact on LV lling than the absolute position of the EDPVR curve
[33]. However, caution should be exercised since chamber stiffness is preload
dependent; that is, chamber stiffness increases with greater preload. In hypertrophic
cardiomyopathy, there may be a large disparity between at pressure-volume
relationships during lling and steep end-diastolic relationships [34].
EDPVR position and slopes indicate the LV stiffness, which can be obtained by
multiplying loaded pressure-volume loops by IVC occlusion as curvilinear trajectories (Fig. 5.9) of the end-diastolic point (Fig. 5.1, Point A). The EDPVR is
shallow in a compliant LV and steep in a stiff LV. Quantication of EDPVR is
obtained by tting to the exponential curve to calculate the stiffness constant ()
[35, 36]. Increased ventricular stiffening causes steep EDPVR values in physiologically working ranges regardless of right or left position.
Diastolic LV-right ventricular interaction (ventricular interaction) [37] is an
important factor of LV diastolic pressure-volume relationships because the LV
and right ventricle exist in the cavity in the pericardium and share both the
intraventricular septum and the outside layer of muscle (Fig. 5.10a). A substantial
RV
LV
Fig. 5.10 Ventricular interaction. See the text for details
110
proportion of the up and down positions of the diastolic pressure-volume relationship (Fig. 5.10b) stems from forces that are extrinsic to the LV rather than from
intrinsic diastolic stiffness in the LV itself. This is called pericardial (or external)
constraint [37, 38], and right-heart lling is one major factor of it. When the resting
diastolic pressure was >6 mmHg, almost 38 % of the pressure was due to external
factors [37]. Thus, in patients with high LV end-diastolic pressure, unloading of the
right ventricle would decrease LV diastolic pressure and improve LV lling.
5.9
Noninvasive Assessment of Diastolic Pressure-Volume

Relationships
In contrast to the systolic phase in which the blood pressure provides considerable
information about the LV pressure, it seems more difcult to noninvasively predict
the diastolic LV pressure-volume relationship. Diastolic pressure has been evaluated from the dynamics of LV lling [39] by evaluation of the mitral valve ow
velocity that is measured by Doppler echocardiography and mitral annular velocity
(LV long-axis lengthening) by tissue Doppler imaging. However, such echocardiographic indices may not provide specic information on intrinsic passive diastolic
properties since abnormal lling dynamics do not necessarily equate with intrinsic
myocardial diastolic dysfunction [40].
All echo-derived indices are affected by loading condition. In addition, each
index has its own limitations. Nevertheless, comprehensive echocardiography,
including Doppler and two-dimensional [33, 41] such as left atrial volume, as
well as chest radiography, physical examinations, and clinical symptoms such as
exertional dyspnea, may help us predict whether LV end-diastolic pressure
(Fig. 5.1, y-axis of Point A) is high or low or within acceptable ranges.
5.10
Hemodynamic Responses in Pressure-Volume

Relationships
The strength of the pressure-volume plane analysis, therefore, is separation and

integration of cardiac functions and loading conditions, as it provides therapeutic
implications based upon a clinicians understanding of the current hemodynamic
situation and prediction of the therapeutic effect. Although the simultaneous measurement of LV pressure and volume is needed to construct real pressure-volume
loops, such measurements are impossible in most clinical situations and are actually
not necessary in daily clinical practice. Instead, integrating the bedside clinical
information including vital signs, blood pressure, and echocardiographic measurements enables the prediction of pressure-volume loops and the qualication of Ees
111
Fig. 5.11 Fundamental hemodynamic responses to preload, afterload, and contractility changes.
These responses illustrate hemodynamic changes (pressure and stroke volume) without changes in
the other two factors. Among the three factors of (a) preload, (b) afterload, and (c) contractility, the
change of one factor will make the loop shift as shown. Blue and green indicate increases and
decreases in each factor, respectively. See the text for details
and diastolic relationships in addition to quantication of Ea. Such predictions will

help optimize the clinical decision-making process.
5.10.1 Basic Response

On the pressure-volume planes, it can be easily predicted how stroke volume and
blood pressure will be changed by preloads, afterloads, and contractility modications. As shown in Fig. 5.11a, increasing preload (end-diastolic volume, shown in
blue) without changes in the other two factors would increase stroke volume and
blood pressure and vice versa. Increasing afterload (Ea, shown in blue) without
changes in the other two factors would increase blood pressure but decrease stroke
volume (Fig. 5.11b). Increasing contractility (Ees, shown in blue) without changes
in the other two factors would increase blood pressure and stroke volume
(Fig. 5.11c).
5.10.2 Combination of Responses

Complex responses in the real circulation are understood as the sum of each factors
response. Increasing contractility by dobutamine (Fig. 5.6a) decreases the
end-diastolic volume (preload change) and further decreases the end-systolic volume; thus, stroke volume (the difference between the two volumes) increases.
ESPVR slope and position will change to the upper left area (contractility change).
112
Fig. 5.12 Pressure-area

analysis of the effect of the
inodilator milrinone, which
possesses inotropic and
vasodilator actions,
increases Ees, reduces Ea
(afterload reduction), and
improves ventriculararterial coupling. Reprinted
with permission from
Senzaki et al. [11]
Milrinone, which possesses inotropic and vasodilator actions (inodilator), increases

Ees, reduces Ea (afterload reduction), and improves ventricular-arterial coupling
(Fig. 5.12) [11].
5.11
Clinical Application of Pressure-Volume Loops

in Pediatric Cardiology Practice
Knowing the current situation relative to the therapeutic goal on pressure-volume

relationships may help clinicians select the optimal therapeutic target. Heart failure
is a syndrome in which the heart cannot effectively eject the amount of blood that
the body demands. Thus, the key target in treating heart failure is improving
ventricular-arterial coupling under optimal preload. Vasodilator therapy, which
lowers Ea, will bring the Ea/Ees ratio back down toward 1.0; inotropic therapy,
which increases Ees, will also improve the Ea/Ees ratio [1]. Which method is more
effective depends on each situation. In this section, assessment by the pressure-area
(volume) relationship in various clinical situations in pediatric/neonatal cardiology
is presented to provide insight into how to apply the concept of pressure-volume
relationships in daily clinical practice.
5.11.1 Systolic Heart Failure With or Without Hypertension

Compared to individuals normal heart function (Fig. 5.13a), patients with systolic
heart failure with LV dilation and poor contractility (Fig. 5.13b) should have
primarily increased afterload (Ea Pes/stroke volume) and will benet most from
vasodilation therapy. Afterload reduction (Fig. 5.13d) will increase the stroke
113
Fig. 5.13 Simulation of hemodynamic changes from normal condition (a) to the contractility
increase (c) or afterload reduction (d) in patients with hypertension and heart failure with reduced
ejection fraction (b)
volume but should not excessively reduce blood pressure because of low Ees,
supporting the usefulness of afterload reduction therapy in such patients. The
primary cause of hypertension (if it exists), such as coarctation of the aorta,
malignancy, and other conditions, should be optimally treated. However, if a low
ejection fraction is the only target, one may choose catecholamine. As shown in
Fig. 5.13c, such an attempt will worsen hypertension without successfully increasing stroke volume or improving ventricular-arterial coupling. Normotensive or
hypotensive patients with reduced ejection fractions should have signicantly
reduced contractility (reduced Ees, Fig. 5.14c); thus, inotropic therapy may rst
be indicated if the condition is critical because vasodilation in this condition
may result in hypotension accompanied by hypoperfusion of the major organs.
Figure 5.15 displays the pressure-volume relationship of a 3-year-old with
dilated cardiomyopathy compared to a control patient of the same age. However,
if the condition is relatively stable despite such a pressure-volume relationship,
chronic titration of an angiotensin-converting enzyme inhibitor is one therapeutic
option that will gradually reduce afterload and improve ventricular-arterial
coupling.
114
Fig. 5.14 Evaluation of contractility in patients with left ventricular dilation and a reduced ejection
fraction (c) in contrast to normal (a) and heart failure with reduced ejection fraction and hypertension (b). Normotensive or hypotensive patients with a reduced ejection fraction should have
lowered Ees and signicantly reduced contractility (as shown in (c))
relationships in a control
and a patient with dilated
cardiomyopathy. Both
patients were 3 years old.
Compared to the control
(green), the pressure-area
relationship in the patient
with dilated
cardiomyopathy was
markedly shifted to the
right. The slope of the
end-systolic pressure-area
relationship (Ees) was
attened, indicating
markedly reduced
contractility
5.11.2 Relative Hypertension and Afterload Mismatch

After Patent Ductus Arteriosus (PDA) Closure
in an Extremely Low-Birth-Weight Infant
Surgical closure of PDA causes abrupt changes in loading condition, including
reduced LV preload and increased LV afterload. The effect of general anesthesia
and opening the chest cavity is superimposed; some smaller infants develop heart
failure due to afterload mismatch after PDA closure. Figure 5.16 represents an
115
Fig. 5.16 Noninvasive assessment of the pressure-volume relationship in an extremely low-birthweight infant with a dilated heart, lowered ejection fraction, and relative hypertension. Echocardiography and blood pressure measurements provided estimated loops. As shown in (a),
carperitide infusion lowered afterload (Ea), decreased end-diastolic volume, increased stroke
volume, and normalized blood pressure. (b) In contrast, if this patient had normotension or
hypotension, decreased contractility (Ees) would be apparent, so inotropes would be indicated
prior to the vasodilator
estimated pressure-volume relationship in an extremely low-birth-weight infant

(650-g birth weight) one day after PDA surgery. Urinary output was markedly
reduced, although blood pressure was 63/33 mmHg, which was relatively high for
this extremely low-birth-weight infant. LV was distended despite PDA closure, and
LV wall motion was impaired due to the low ejection fraction of 32 % [42]. Higher
blood pressure and reduced stroke volume indicated higher Ea; hence, higher
afterload should be the primary cause of low cardiac output (afterload mismatch;
Fig. 5.16a, black rectangle). To improve cardiac output and urination, we administered carperitide, a vasodilator of atrial natriuretic peptide. Thereafter, the blood
pressure reduced to the normal range, LV distention and impaired wall motion were
improved (Fig. 5.16a, blue rectangle), and urination was restored.
If the dilated LV and decreased EF were the focus in this patient, catecholamine
might have been selected. In such a case, the gain in stroke volume would be
minimal because of high Ea, might worsen harmful hypertension in extremely lowbirth-weight infants, and would never reduce the excessive LV load. However, if
this patient were normotensive or hypotensive, poor ejection fraction and increased
end-systolic volume would suggest decreased contractility (Ees) (Fig. 5.16b, black
rectangle). In such a case, catecholamine may be a better rst choice to increase
contractility, after which a vasodilator would be an option after a blood pressure
reserve was observed.
116
5.11.3 Dilated LV and Poor Ejection Fraction with Large

Fluctuations in Blood Pressure
A markedly dilated LV and poor ejection fraction with hypertension in the awake
state persisted in a 7-month-old girl even after an emergent operation for coarctation of the aorta, which had not been previously diagnosed, due to the presentation
of shock. Increased Ea but decreased Ees (Fig. 5.14b) was initially suspected.
However, marked uctuation of the blood pressure was observed in this patient
from 70 mmHg in sedated sleep to 120 mmHg in an awake and irritable state. No
signicant difference of end-systolic volume or ejection fraction was observed
between these two states by echocardiography.
Such uctuations cannot be explained by a shallow Ees, which only causes
minor pressure responses to changes in Ea (Fig. 5.17a); rather, it suggests a steep
ESPVR with increased Ees and Vo in the right position. Figure 5.17b schematically
displays the estimated pressure-volume loops of this patient, which can explain the
hemodynamic observations. As discussed and displayed in pink triangles (the slope
is Ees) in Fig. 5.17, changes in blood pressure relative to changes in end-systolic
volume provide us an idea about whether Ees is high or low.
In such patients, the primary pathophysiology is increased afterload (Ea Pes/
stroke volume), ventricular stiffening (increased Ees), and LV remodeling
(increased Vo). With this understanding, no more increases of Ees or Ea are
warranted. However, if the decreased EF is focused, one may select catecholamines
to increase contractility. In such cases, it will worsen hypertension during the awake
state and create a trivial gain in stroke volume (Fig. 5.17c). The use of vasodilators
Fig. 5.17 Estimation of end-systolic elastance by large uctuation in blood pressure in the patient
with a dilated left ventricle and poor ejection fraction. See the text for details
117
is difcult because vasodilator-induced responses in blood pressure may be insufcient in the awake condition and cause hypotension during sleep. Even with
adequate afterload reduction, the gain in stroke volume will be minimal due to
the steep Ees (Fig. 5.17b). Because it takes a long time to normalize abnormal
hemodynamics in such patients, a -blocker and angiotensin-converting enzyme
inhibitor were initiated and titrated for reverse remodeling. This patient gradually
improved and was discharged after 3 months [43].
5.11.4 Heart Failure with Preserved Ejection Fraction

in Children
Heart failure with preserved EF exists in children [44]. Patients with heart failure
and preserved EF (Fig. 5.18c) have ventricular and arterial stiffening [44, 45]. In
these children, large amounts of diuretics are required to avoid congestion despite
normal renal function, indicating severe heart failure. However, chest X-ray and
echocardiographic examinations demonstrate only modest impairment in most
cases. Figure 5.19 shows the pressure-area relationship during abdominal compression, which increases preload [11], in children with this disease entity (after repair
of interruption of aortic arch complex). Ventricular (increased Ees) and arterial
(increased Ea) stiffening as well as diastolic ventricular stifng are clearly
displayed. Afterload reduction results in marked decreases of blood pressure and
slight increases of stroke volume (Fig. 5.18c). Similarly, a slight reduction in
preload results in hypotension, while a slight excess in preload results in hypertension and pulmonary congestion due to increased diastolic pressure (Fig. 5.19). We
can also predict hypertensive responses to exercise because exercise increases
preload, further increases both Ees and Ea, and increases diastolic ventricular
Fig. 5.18 Schema of the pressure-volume relationship in heart failure with a preserved ejection
fraction (HFpEF). Patients with HFpEF (c) have ventricular-vascular stiffening (increased Ees and
Ea) compared to individuals with normal function (a) and those with heart failure and a reduced
ejection fraction (HFrEF) (b). Thus, the response of afterload reduction results in a slight increase
in stroke volume but a large decrease in blood pressure
118
140
120
100
Pressure (mmHg)

relationship during
abdominal compression in a
patient with heart failure
and a preserved ejection
fraction. This compression
increased preload and
greatly increased
end-diastolic pressure in
this patient, showing a steep
end-diastolic pressure-area
relationship and unmasking
the severe diastolic
dysfunction
80
60
40
EDPAR
20
0
0
Area (cm2)
pressure due to diastolic stiffening, resulting in limited exercise capacity in these
patients.
These responses to treatment can be easily predicted with an understanding of
the pressure-volume loop concept, in which the stroke volume and Pes result from
the balance between Ees and Ea with a given preload. End-diastolic pressure in this
disease in children is only modestly elevated [44]. However, a preload increase by
abdominal compression produced markedly elevated end-diastolic pressure (from
10 to 20 mmHg in Fig. 5.19), suggesting the usefulness of pressure-volume analysis
with this simple intervention to elucidate the complete picture of the end-diastolic
pressure-volume relationship.
5.11.5 Atrial Septal Defect (ASD) Assessment by Diastolic

Ventricular-Ventricular Interaction
The ASD is a major congenital heart disease that affects individuals across the
lifespan. Due to the left-to-right atrial shunt, volume overload of the right side of
the heart and unload of the left side of the heart persist until defect closure occurs.
Figure 5.20 shows an example of the pressure-area relationship in the presence of
an ASD featuring a pulmonary to systemic ow ratio of 1.6 [11]. As shown, LV
end-diastolic volume is relatively smaller than in the control, and contraction and
119
Fig. 5.20 Representative

pressure-area relationship
in a patient with an atrial
septal defect. The solid line
indicates the patient with
the atrial septal defect and
the dashed line indicates the
control patient. See the text
for details. Reprinted with
permission from Senzaki
et al. [11]
afterload do not change signicantly. Device closure has been the main treatment
option for patients who meet the indication criteria. After the abrupt change of device
closure, some elderly patients can develop pulmonary congestion, whereas the
younger patients do not. The response (increase or decrease) of end-diastolic pressure
on ASD closure may vary on the balance between two factors; movement toward the
upper right area of the end-diastolic pressure-volume relationship by an increase in
lling volume and a downward shift of the end-diastolic pressure-volume relationship by right ventricular unload (ventricular-ventricular interaction) (Fig. 5.10).
Figure 5.21 displays the pressure-volume relationship before and after device
closure in a 9-year-old patient. In this example, the end-diastolic pressure-volume
relationship moved signicantly downward and the latter factor was dominant. Hence,
the end-diastolic pressure did not increase in this young patient. However, the response
to ASD closure appears differently in elderly patients, in whom end-diastolic pressure
increases to a greater extent after closure [13], causing difculty adapting to the
new hemodynamics. Future accumulation of data on this issue is warranted to further
clarify the mechanism of age-related responses to ASD device closure.
5.11.6 Right Ventricular Pressure-Volume Relationship

The pressure-volume relationship concept can be also applied to the right ventricle.
In the patients without a shunt, cardiac output in the right side of the heart equals
that in the left side of the heart. Thus, the problem in the right side of the heart may
reduce systemic output directly and indirectly via right ventricular-left ventricular
interactions. Figure 5.22 displays the pressure-area relationship of a 13-year-old
patient with peripheral pulmonary stenosis after intracardiac repair of pulmonary
120
Fig. 5.21 Pressure-volume relationship in a young patient with an atrial septal defect before and
after device closure. After device closure in this young patient, the end-diastolic pressure-volume
relationship shifted downward in a parallel manner, indicating reduction of the right ventricular
effect. Reprinted with permission from Masutani et al. [49]
atresia and a ventricular septal defect [46]. Stent implantation, which is an effective
alternative to surgery and balloon angioplasty for the treatment of stenotic vascular
lesions, reduced the pressure gradient between the main pulmonary artery and each
side of the peripheral pulmonary arteries (from 22 to 11 mmHg on the right and
from 30 to 12 mmHg on the left) [46]. As shown in Fig. 5.22, the effective arterial
elastance was reduced and the Pes-area relationship was shifted to the upper left
area after stenting, indicating an improved ventricular-vascular interaction in the
right side of the heart (right ventricular-pulmonary arterial coupling) and resulting
in an increased cardiac output from 3.4 to 4.1 L/min/m2.
5.11.7 Fontan Circulation-Hemodynamic Rest

and Chronotropic Reserve Impairment
Fontan operations have been performed to physiologically correct congenital heart
disease in patients for whom repair in a two-ventricle system is impossible
[47]. This kind of circulation lacks a ventricle to eject blood to the pulmonary
circulation; instead, central venous pressure is the driving force propelling the
121
Fig. 5.22 Right ventricular pressure-area relationship before and after stent implantation for
peripheral pulmonary stenosis. See the text for details. Reprinted with permission from Kohno
et al. [46]
Fig. 5.23 Representative pressure-area relationship in a Fontan patient, who had a relatively
higher afterload (Ea) resulting in suboptimal ventricular-arterial coupling and limited stroke
volume. Reprinted with permission from Senzaki et al. [48]
blood into the pulmonary circulation. Late complications as well as limited exercise
tolerance are known to occur after Fontan operations. An earlier study employing a
pressure-area relationship [48] elucidated some of the unique rest and reserve
functional impairments in the Fontan circulation. Figure 5.23 represents the
pressure-area loops of Fontan compared to the control [48]. In this resting state,
Fontan has similar end-diastolic area and Ees but signicantly higher Ea, resulting
in impaired ventricular-arterial coupling (lowered Ees/Ea) and a reduced cardiac
122
Fig. 5.24 Tachycardia-induced response of the pressure-area relationship in Fontan patients.

Compared with control (closed circle), in Fontan patients (open circle), increasing heart rate by
atrial pacing similarly increased afterload (Ea) but did not increase contractility (Ees), resulting in
a marked reduction in stroke area index. Reprinted with permission from Senzaki et al. [48]
index. These characteristics were enhanced upon examination during a fast heart
rate induced by atrial pacing (Fig. 5.24). Increasing the heart rate increased Ea in
both Fontan and control. In contrast, signicant increases of Ees were observed in
the control but not the Fontan. Thus, compared with the control, Ees/Ea and stroke
area index decreased in Fontan with increasing heart rate [48]. These characteristics
in part account for the exercise intolerance seen in Fontan patients.
5.11.8 Follow-Up After Intervention: Effect of Stent

Implantation in Adolescent Coarctation of the Aorta
The pressure-volume analysis is also useful in follow-up after surgical/catheter
intervention. Here we present one case of such follow-up. Stent implantation was
performed in a 13-year-old boy with native coarctation of the aorta (Fig. 5.25).
After stenting, Ea decreased and Ees increased. Although the ventricular-arterial
coupling (Ees/Ea) was normal, both Ees and Ea remained high after stenting
160
160
140
LV pressure (mmHg)
140
LV pressure (mmHg)
123
120
100
80
60
40
120
100
80
60
40
20
20
0
0
10
Area
(cm2)
0
0
10
Area
(cm2)
before stent
after stent
follow up
Fig. 5.25 Example of follow-up evaluation employing the pressure-area relationship. After stent
implantation in a patient with coarctation of the aorta ventricular-arterial coupling was improved
(a). One year after stent implantation, ventricular-arterial stiffening (high Ees and Ea) was
improved ((b), pink) as blood pressure normalized
(Fig. 5.25, left panel). Ventricular-arterial stiffening can cause a hypertensive

response during exercise [45]. One year after stent implantation (Fig. 5.25, right
panel), however, a pressure-volume analysis revealed that the ventricular-arterial
stifng had improved (reverse remodeling: reduced Ees and Ea from original
abnormally high status), resulting in an increased stroke volume and improved
hypertensive state.
Conclusion
Understanding the concept of the pressure-volume relationship enables the
accurate analysis of current hemodynamics, prediction of the response to
therapy, and appropriate assessment of follow-up with loading conditions/
intrinsic function in a separate and integrated manner. Thus, pressure-volume
relationships, even those assessed noninvasively, are still irreplaceable
despite the signicant development of other imaging modalities. Because
advantages in pressure-volume relationships are particularly attractive and
useful in the management of congenital heart disease, further application of
the pressure-volume concept is warranted in pediatric and neonatal
cardiology.
124
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Chapter 6
Assessment of Vascular Function by Using

Cardiac Catheterization
Hirofumi Saiki and Hideaki Senzaki
Abstract Despite marked advances in cardiovascular surgery and perioperative
management, children with congenital heart disease still experience many problems
in their adult life. One of the issues that should be resolved is progressive heart
failure toward adolescence. During cardiac catheterization, the parameters of cardiac function and vascular function, according to which the best strategy for
patients could be chosen, are obtained. These data are useful for elucidating the
hemodynamic features of specic structural heart disease and could clarify the
mechanisms of heart failure even in children. However, vascular function tends to
be overlooked as a factor for worsening heart failure in view of the long term, and
only a few comprehensive reviews are available in the eld of congenital heart
disease.
This chapter summarizes the currently available methods for evaluating vascular
function, especially based on catheterization laboratory examination in children.
The rst part of this chapter discusses the direct and load-independent arterial
characteristics of vessels, which provide convincing information for clinical study
and for predicting hemodynamic changes corresponding to changes in loading
status. In the latter part, indirect evaluation of vessels is presented, which can be
useful in real-time decision making in a catheterization laboratory. Last, we also
discuss the venous and minor vessel functions that can affect organ congestion and
dysfunction.
Keywords Arterial function Children Coupling Impedance Vascular
function
H. Saiki, MD (*)
Pediatric Cardiology, Saitama Medical Center, Saitama Medical University, Saitama, Japan
Cardiovascular Diseases, Mayo Clinic, 200 First street S.W., Rochester, MN 55905, USA
e-mail: Saiki.Hirofumi@mayo.edu
H. Senzaki, MD
Pediatric Cardiology, Saitama Medical Center, Saitama Medical University, Saitama, Japan
Springer Japan 2015
DOI 10.1007/978-4-431-54355-8_6
127
128
6.1
H. Saiki and H. Senzaki
Introduction
The vascular system is the center of systemic circulation in humans. It consists of a

variety of vessels, including the aorta, elastic arteries, arterioles, capillaries, and
veins. The arteries receive blood output from the heart, which converts pulsatile
ow into more continuous ow by storing systolic energy as potential energy, and
then forward the blood into the diastolic phase. This function contributes to the
reduction in the mechanical force as a stress to the endothelium of peripheral
organs, also decreasing the afterload to the heart [1, 2]. A damaged endothelium
worsens the functions of the organ, due to a signicant association between the
vascular system and the systemic organ function. In the heart, arterial characteristics work as the cardiac afterload [3]; in turn, cardiac output is also determined by
the cardiac preload depending on the venous function. As Starlings law states,
preload is the traditional but essential determinant of cardiac output.
In addition, activation of the renin-angiotensin-aldosterone system (RAA) activation involves the substrates of cardiovascular brogenesis [4], worsening of heart
failure, and cardiovascular stiffening with structural and functional changes [57].
Heart failure and stiffening of the great arteries are also associated with impairment
of the regulatory system homeostasis in humans, including the baroreceptors,
noradrenaline cascade, and RAA. These deviating activities of homeostasis induce
ventricular and arterial stiffening, resulting in the formation of a vicious cycle that
further worsens heart failure. Thus, vascular stiffening can be the treatment target to
stop this cycle.
The veins are the pathways of venous blood ow to the heart. Venous congestion
can deteriorate, owing to venous valve malfunction or congestive heart failure, and
is considered to also cause organ congestion, which degrades the functions of the
somatic organs [47]. Such end-organ dysfunction induced by congestion has been
reported in the kidneys in children [8], and there have also been many indirect
evidences about the association between venous congestion and pathologic conditions, such as plastic bronchitis and protein-losing enteropathy [9] often observed in
patients with Fontan circulation. Our recent study elucidated that venous congestion also reduces cerebral perfusion in Fontan patients, indicating a potential
association with the neurodevelopmental outcome.
Hence, both arterial and venous functions have important effects on the cardiac
output as changes in loading status to the heart, which can directly inuence
virtually all the end-organ functions from the brain to the peripheral organs.
Therefore, proper evaluation and management of arterial and venous function in
children can help preserve cardiac function and cardiac output later in life and
prevent worsening of systemic organ functions, which have a close correlation with
the outcome in patients with congenital heart disease.
In this chapter, we aim to describe the basic concepts of vascular characteristics
in humans and the evaluation of vascular function in children with heart disease by
using cardiac catheterization.
6 Assessment of Vascular Function by Using Cardiac Catheterization
6.2
129
Arterial Function
As noted above [1], evaluation of arterial function is important in the clinical

management and prevention of heart failure. There have been numerous publications about arterial functional abnormalities in patients with congenital heart
defects, including coarctation of the aorta (CoA) [10, 11] and tetralogy of Fallot
(TOF) [1214]. Because evaluation of arterial characteristics in a living human
body by extracting vessel tissues is infeasible, blood ow and pressure data
obtained by performing echocardiography, cardiac catheterization, or other noninvasive modalities are usually used for estimation [1517]. In this section, the
systemic concepts of analyzing arterial characteristics especially available for
heart disease in children are summarized.
6.2.1
Estimation of Arterial Characteristics Based

on the Windkessel Model
6.2.1.1
Input Impedance
The characteristics of the arterial system share similar concepts with an electric
circuit. The relation between pulsatile blood ow and the arterial system can be
simulated as the windkessel model (Fig. 6.1), which is described as an electric
circuit that has a resistance and condenser in parallel. In this model, part of the
output in systole accumulates in the condenser (windkessel) part and is then ejected
into the artery (resistance part) in the diastole. The characteristics of the circuit,
condenser, and resistance (impedance) independently determine the blood pressure
if cardiac output (electrical current) is provided. Therefore, similar to the electrical
alternating current, the impedance can be evaluated where the relation between
pressure (voltage) and blood ow (electric current) is known. The real arterial
system can be more precisely simulated by adding a component of characteristic
impedance, which represents proximal artery stiffness where the effects of arterial
wave reection are negligible. In this model, the so-called three-element
windkessel model, the arterial system can be simulated by using three elements:
Rc
C
Fig. 6.1 Schema of the
three-element windkessel
model
Rp
130
characteristic impedance (Rc), total arterial compliance (C), and peripheral resistance (Rp), as shown in Fig. 6.1 [18].
During cardiac catheterization, simultaneous measurements of blood ow and
pressure can be obtained by using a manometer-mounted pressure-ow wire
in vivo, and both waveforms are resolvable to a large number of sine curves,
whose frequencies are the products of the integral multiplication of the basic
frequencies (see equations below). Importantly, all of the resolved waves are
prime and independent of each other.
Pt P0 Pn sin 2fn t n
Ft F0 Fn sin 2fn t n
where Pn and Fn are the pressure and ow amplitude of the nth harmonic,
respectively; fn n*HR/60 (frequency of the nth harmonic); n, n pressure
and ow phase angle of the nth harmonic.
The quotient of the pressure waveform divided by the ow waveform gives the
dimension of resistance (Zn) and is expressed as the amplitude of each term. The
phase angles are calculated by subtracting the aortic pressure and ow components.
Zn Zo Zn sin 2fn t n n
Each coefcient of the terms is called the impedance modulus, indicating the
index of the vascular characteristics (Fig. 6.2, left).
The impedance can be interpreted as the transfer function by which blood
pressure waveforms are determined if the blood ow waveforms are input; thus,
the impedance is an independent characteristic of vascular function regardless of
the input to the arteries. Most important, input impedance coupled with the cardiac
function evaluated by using the pressure-volume relation (elastance model) can be
used to simulate blood ow and pressure waveforms in patient models. Input
impedance itself conveys a variety of vascular characteristics in a frequency
domain by which the specic mechanical properties of the artery can be evaluated.
6.2.1.2
Characteristic Impedance (Zc, Rc)
The averaged amplitude of high frequency is called the characteristic impedance,

indicating the impedance of the proximal aorta mainly acting as the cardiac
afterload. Characteristic impedance is the impedance to the forward traveling
electric current until electricity lls the lead, represents the property of the electric
leads independent of their length. Similar to the electric circuit, Zc in the arterial
system represents the property of the proximal aorta and is usually calculated as the
average of n 310 frequency domains [19, 20], which are relatively free from the
effects of reection waves originating from the distal vessels. These frequencyanalyzed data are validated by analyzing the early systolic pressure-ow (volume)
data of the aorta and arterial elastance [3, 21].
Control
131
TOF
100
Pressure (mmHg)
100
80
60
50
40
20
100
20
0
200
100
300
250
250
200
200
150
150
100
100
50
50
Hz
300
Time (ms)
Time (ms)
300
200
10
12
10
12
Hz
Fig. 6.2 Examples of input impedance in control (left) and patients with TOF (right). Zc,
represented by average modules of 310 Hz, is markedly increased in patients with TOF,
indicating increased proximal aortic impedance. In addition, Z1, represented by the fundamental
frequency modules, and uctuation in the high-frequency domain are also markedly increased in
patients with TOF
6.2.1.3
Impedance Modules in Zero Harmonic: Peripheral Arterial

Resistance (Rp)
Because the zero-frequency domain is free from the inuence of sine curve
components of ow and pressure formula, Z0 is equivalent to the peripheral arterial
resistance, which is free from the pulsatile components. Accordingly, Z0 is theoretically equal to the nonpulsatile arterial resistance calculated as the pressure
difference divided by the cardiac output (total arterial resistance, R).
The peripheral arteries are the most distal arteries from the heart, and they
usually accept a nonpulsatile ow of blood. These are considered the resistant
components of the electric circuit and comprise the total peripheral resistance
132
(TPR). TPR is usually calculated as the difference between the mean arterial
pressure and the right atrial pressure divided by the cardiac output:
TPR mBP mRAp = CI
where TPR is the total peripheral resistance, mBP is the mean blood pressure,
mRAp is the mean right atrial pressure, and CI is the cardiac index.
6.2.1.4
Reection Index
The pulsatile components of the cardiac output generate reex from heterogeneous
parts of the vessels, where impedance mismatch, measured as the uctuation of the
impedance modules, exists. The magnitude of the uctuation is considered as the
intensity of reex [22]; thus, the reection index is calculated as the difference
between the maximum and minimum values of impedance modulus at a frequency
of >3 Hz. The reection components are not included in the three-element
windkessel model, but are suspected as the determinants of enhanced systolic
pressure [23]. In addition, because low-frequency modules have large power, the
module of the rst harmonic can also be considered a representative of a
reection [24].
Senzaki et al. had reported about and emphasized the feasibility of input
impedance analysis in patients with TOF and Kawasaki disease [14, 15]. Figure 6.2
shows examples of input impedance analysis in patients with TOF.
However, because such a frequency domain approach requires specic equipments and extra time for the analysis, the time domain approach is often used in the
clinical evaluation of arterial function. These evaluations are presented in the latter
part of this chapter.
6.2.1.5
Total Arterial Compliance
Total arterial compliance (TAC) [25, 26] is a quantitative index that corresponds to
the characteristic of the condenser in the windkessel model. TAC represents the
elastic property of the aorta; it is inuenced by pulse wave transmission and
reection. Thus, it can be a comprehensive determinant of the cardiac afterload
against the pulsatile blood ow. TAC decreases in response to increased arterial
stiffness (e.g., due to aging) and is reported to be associated with essential hypertension [27] and the myocardial oxygen demand-supply balance [28].
The arterial pressure-volume relation and diastolic pressure decline in diastole had
been used to evaluate TAC. Because the time constant () of diastolic aortic pressure
decay is the product of peripheral resistance (Rp) and arterial compliance (TAC), total
arterial compliance can be obtained by the direct calculation of divided by arterial
resistance. Liu et al. reported a simplied estimation of compliance (C area) calculated
from the area under the curve of the aortic pressure waveform [29].
133
C area mL=mmHg SV=K ESAoP DAoP

with K As Ad=As
where K is the area coefcient, As is the systolic pressure wave area, and Ad is the
diastolic pressure wave area.
More recently, Chemla et al. found that the TAC calculation by using the area
method can be replaced with the following formula [30]:
C area SV=PP
where SV is the stroke volume and PP is the pulse pressure.
To summarize, arterial characteristics can be estimated, according to the concepts of the windkessel model, by using the data obtained during cardiac catheterization. These data can be relatively load-independent indices and, when combined
with the cardiac parameters, can be used to simulate a human circulation model.
6.2.2
Estimation of Arterial Function with Indirect Methods
As mentioned above, although impedance analysis provides comprehensive information about arterial hemodynamics and function, computing impedance is somewhat cumbersome. In this section, we will introduce an indirect method to evaluate
arterial stiffening during cardiac catheterization. This assessment can be performed
even noninvasively; however, a couple of limitations exist because of problems
specic to children.
6.2.2.1
Pulse Wave Velocity
Pulse wave velocity (PWV) is an index based on the principle that the pulse wave
transmits faster in rigid substances than in compliant substances. Thus, an increase
in PWV can be used as an indirect marker of arterial stiffening, which is associated
with an increase in afterload.
The PWV is dened by the following formula introduced by Korteweg-Moens:
PWV
p p p p
E h = D
where PWV is the pulse wave velocity, E is the Youngs modulus, h is the vascular
wall thickness, is the blood density, and D is the vessel radius diameter.
The formula represents the important inuence of vascular diameter and wall
thickness as determinants of the PWV, which should be kept in mind in the
evaluation of vascular stiffness with PWV.
134
tA
ECG
B
tB
ECG
PWV
B
DB-DA
tB-tA
Fig. 6.3 Schema of calculating the pulse wave velocity (PWV) during cardiac catheterization
The measurement of PWV during catheterization is shown in Fig. 6.3. During

catheterization, the pulse transmission time is calculated from the simultaneous
measurements of pressure waveform and electrocardiogram (ECG), and the distances of the two different parts are obtained as the length of the catheter outside the
body. Accordingly, the PWV is calculated as the distance divided by the traveling
time interval between the two points. This method requires catheter insertion to the
vessel; however, this PWV measurement is more accurate than the noninvasive
alternatives, especially in children. If catheter examination is planned with the
clinical requirement, then PWV can be evaluated without additional cardiovascular
risk even in extremely small children such as neonates.
The large cohort studies of adult heart disease emphasized the clinical importance of PWV in predicting cardiovascular events, including myocardial infarction,
unstable angina, heart failure, and stroke [17, 31]. In patients with congenital heart
disease, we found the signicant association between stiffening of the ascending
aorta and dilatation in TOF [12]. Because Niwa et al. had elucidated the close
correlation between aortic dilatation and arterial wall degeneration [32, 33], we
concluded that arterial stiffening in TOF is associated with arterial mid-wall
degeneration. In a similar-patients group, arterial stiffness, adjusted for
confounding factors such as blood pressure and age, was signicantly increased
in unrepaired TOF than in repaired TOF [13]. Therefore, early anatomical correction would be recommended to prevent arterial degeneration and dilatation in
patients with TOF. In addition, we also found that a similar correlation exists
between arterial stiffness and dilatation in patients with a single ventricular
135
circulation. Interestingly, the magnitude of the correlation between stiffness and

dilation in a single-ventricle heart is much smaller than that of TOF, suggesting
genetic background as an underlying mechanism of aortic dilation/stiffening
in TOF.
For a better understanding, the relation between input impedance analysis and
PWV should be addressed. As shown in the early part of this chapter, characteristic
impedance (Zc) is the index of arterial property that mainly reects proximal
arterial stiffness without the inuence of the reection wave. Therefore, Zc and
PWV should have a close correlation with each other. The relation between the two
parameters is described as follows:
Zc PWV=r 2 ;
where is the blood density, Zc is the characteristic impedance, PWV is the pulse
wave velocity, and r is the arterial lumen radius.
From this water-hammer formula and the Korteweg-Moens formula, it can be
seen that Zc is more inuenced by the arterial diameter than by the PWV.
6.2.2.2
Augmentation Index
The augmentation index (AI) represents the magnitude of blood pressure enhancement by the reected wave. The underlying principle is simple: if the artery is stiff,
the reection wave is enhanced and returns early to the proximal aorta. In this
concept, AI is a user-friendly marker evaluated by using only the arterial pressure
waveform without any other special device. In considering the clinical utility of AI,
its limitations should be elucidated, especially the following two points: First, AI is
easily inuenced by hemodynamic parameters. Studies about the association
between input impedance analysis and wave reection have shown that the variability of modules in high frequency, rather than those in low frequency, has
considerable effects on the increase in AI, implying the importance of reection
in determining AI, whereas the reection can also be inuenced by the mean blood
pressure [34, 35]. Second, systolic augmentation does not necessarily indicate the
early arrival of the reection wave. Mitchell et al. suggested that the enhancement
of the systolic wave might be the result of an impedance mismatch induced by the
small aortic size compared with the body size [36]. Therefore, AI is inuenced by a
variety of hemodynamic factors. Despite these limitations, inuences of the
decrease in TAC on the increase of reection are also reported, thus conrming
the usefulness of AI as an index of arterial stiffness [37]. We therefore propose that
in the vascular assessment with AI, the limitations of this index should be accounted
for and that it seems suitable to judge hemodynamic status only during cardiac
catheterization.
136
6.3
Evaluation of Venous Function by Using Cardiac

Catheterization
The venous system retains almost 75 % of the total amount of blood in the body and
mobilizes or reduces venous return in response to the bodys demands. This is the
basis of the preload reserve; thus, venous function can be considered as one of the
main components of circulation. This can be understood through the concept of
pressure-volume relation; if the preload is reduced, signicant increase of afterload
or dramatic hyper-contractility is required to maintain the bodys blood pressure.
Decreased preload reserve is a frequently encountered pathophysiology in pediatric
cardiology in the management of repaired TOF [38], pulmonary atresia with intact
ventricular septum [39], and Ebsteins anomaly [40]. The common fundamental
pathophysiology would be decreased pulmonary ventricular function, and the
Fontan circulation represented by a lack of the right ventricle is the extreme
example of this pathophysiology. In addition, heart failure gives rise to activation
of the RAA hormonal axis, leading to cardiac diastolic dysfunction [41]. Thus, the
preload is one of the easily disrupted mechanisms, especially in congenital heart
disease (which includes various types of right ventricular failure).
Despite the importance of venous capacity and thus preload reserve in the
cardiovascular system, assessment of venous properties is scarcely understood in
humans. Guyton et al. proposed the concept of venous return curves in animal and
theoretical experiments [4244], which represent the relation between right atrial
pressure and cardiac output (Fig. 6.4).
This model is too simple when considering the real circulation [45]; however, it
is valuable and useful in the clinical management of heart failure. The fundamental
concept is that the venous return (cardiac output) increases with the decrease in
right atrial pressure (RAp), because a reduction of RAp facilitates venous return.
If RAp decreases to less than zero, no further increase of the venous return and
cardiac output is observed because of venous collapse. Importantly, this establishes
the characteristic parameters of venous return resistance (VRR) and mean circulatory lling pressure (mcfP). VRR is the inverse slope of the venous return curve,
representing the venous characteristic. The mcfP is the intercept of the pressure
axis, representing the pressure when the blood ow is stopped and all of the vessels
are lled. The pressure difference between the mcfP and RAp is the driving
pressure of the venous return. If VRR and mcfP can be evaluated in the clinical
setting, novel ndings about the mechanisms of reduced preload, as in
decompensated heart failure or Fontan circulation, might be elucidated [46].
However, the clinical application of this kind of evaluation is extremely limited
because right ventricular inow obstruction is required to describe the venous
return curve. Therefore, most clinicians evaluate only the central venous pressure
(CVP) value in estimating the hemodynamic status of preload, whereas some
clinicians attempt to estimate venous function rather than performing simple
pressure measurements. To estimate the Rv and mcfP, simultaneous measurements
of CVP and venous return (cardiac output) are required without any inuence on
137
Venous return=Cardiac output

L/min/m2
venous return resistance venous return/ RAp
RAp
-4
mcfP
20 (mmHg)
Fig. 6.4 Guytons venous return curve. The mean circulatory lling pressure (mcfP) is the
intercept of the horizontal axis, representing the pressure where the total amount of blood was
stopped. The pressure difference between the mcfP and the measured right atrial pressure is the
driving pressure of the venous return. Venous return resistance is calculated as the inverted slope
of the venous return curve, representing the venous return characteristic
the cardiac function. To this aim, a variety of methods to modulate the venous
return and CVP during cardiac catheterization are proposed. Some researchers
applied and extrapolated experimental data obtained from animal studies
[47]. This area of venous characteristics needs further evaluation in the clinical
setting.
6.4
6.4.1
Integrated Measurements
Wave Intensity Analysis
Wave intensity analysis (WIA) is the method for delineating the hemodynamic
interaction between two organs at the specic location where the blood ow and
pressure data are obtained [4851]. WI is calculated as (dP/dt)*(dU/dt) [49, 52],
where dP/dt and dU/dt are the time derivatives of pressure (P) and ow velocity (U )
of the carotid artery. If WI is a positive value, the changes in pressure and velocity
caused by the forward-traveling wave from the ventricle are greater than those
caused by the backward-traveling wave from the peripheral circulation, and vice
versa. The increase in pressure is considered by the WI theory to be a result of
138
a compression wave and the decline in pressure a result of an expansion wave.

If the ow decreases, it has a nature of wave deceleration, whereas if the ow
increases, it has a nature of wave acceleration (Fig. 6.5). In WIA, the relation
between the heart and the central hemodynamics as well as peripheral perfusion can
be estimated. We had elucidated reduced cerebral perfusion in Fontan patients by
using WIA [53].
mmHg 140
120
100
Pressure
80
60
sec
40
cm/sec
50
40
30
Flow
20
10
sec
mmHgmsec-3*103 50
40
W1
30
Wave
Intensity
20
W2
10
0
10
NA
Fig. 6.5 Example of a wave intensity analysis (WIA) in the carotid artery
sec
139
Conclusions
In this chapter, we summarized the assessment of vascular function by using
cardiac catheterization. Coupled with hemodynamic evaluation by using the
pressure-volume relationship, these methods provide important information
about the pathophysiology of cardiovascular diseases. Although recent developments in noninvasive evaluation have reduced the necessity for cardiac
catheterization, assessment by using catheterization still provides irreplaceable information about vascular function. Hemodynamics in congenital heart
disease can be simulated by using the assessment of load-independent vascular function in conjunction with the ventricular pressure-volume relationship, yielding a precise estimation of the ideal postoperative morphology in
congenital heart disease. Because most of the congenital heart diseases
involve structural anomalies, the dynamic and drastic changes of hemodynamics are observed. If the treatment strategy is determined by the convincing and evidence-based cardiovascular function assessment, then it can
directly link to marked improvement of perioperative management and can
guide the proper intra- and postoperative procedures for better circulation and
improved patient outcomes.
We hope that this chapter provides information to help improve hemodynamic management and claries the appropriate application of cardiac catheterization, especially in children.
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Surg 97:13941399
Chapter 7
Assessment of Ventricular-Vascular Function

by Echocardiography
Manatomo Toyono
Abstract Reliable assessment of ventricular function is an essential for management of patients with heart disease. Noninvasive echocardiographic evaluation is
indispensable for repeated assessment of ventricular function in the clinical setting.
Alterations of left and right ventricular geometry and loading conditions are the
property of congenital heart disease; therefore, quantitative assessment of ventricular function is technically challenging. Systolic ventricular function is pump
activity for the generation of an adequate cardiac output with lling pressure as
low as possible. A wide variety of different echocardiographic parameters and
indices are developed for the assessment of systolic ventricular function; however,
no single parameter adequately provides all the necessary information. One should
integrate information from different parameters to comprehensively describe systolic function. Echocardiographic assessment of diastolic function is based on
Doppler method of mitral inow and the pulmonary veins with supplemental
assessment by tissue Doppler, strain, and strain rate. Although several indices are
available, no single indices adequately evaluate diastolic function. Therefore, a
comprehensive examination is mandatory as well as in systolic ventricular function.
This chapter will discuss traditional and newer echocardiographic techniques for
the evaluation of ventricular function and, in addition, vascular function in patients
with congenital heart disease.
Keywords Diastolic function Echocardiography Systolic function Vascular
property
M. Toyono (*)
Department of Pediatrics, Akita University, 1-1-1 Hondo, Akita 010-8543, Japan
e-mail: manatomo@doc.med.akita-u.ac.jp
Springer Japan 2015
DOI 10.1007/978-4-431-54355-8_7
143
144
7.1
M. Toyono
Ventricular Function
7.1.1
Left Ventricle
7.1.1.1
Systolic Ventricular Function
7.1.1.1.1
Shortening Fraction
Shortening fraction (SF) represents the change in the left ventricular

(LV) dimension that occurs during contraction:
SF % LV-EDD LV-ESD = LV-EDD 100
where LV-EDD represents LV end-diastolic dimension and LV-ESD represents LV
end-systolic dimension (Fig. 7.1). Normal values for SF range from 28 to 38 %
[1]. Values <28 % suggest reduced systolic function while values >38 % suggest
hyperdynamic function. The short-axis view at the papillary muscle level is most
frequently used to measure SF. SF assesses radial ventricular function in the LV
basal part.
SF has limitations that should be taken into account when used in the clinical
settings. SF assumes that there are no regional wall motion abnormalities.
Hypokinesis or dyskinesis of the interventricular septum (IVS) occurs in the
presence of right ventricular (RV) volume overload that is seen in signicant atrial
Fig. 7.1 M-mode LV-SF measurement. M-mode measurement is obtained from the parasternal
short-axis view at the level of LV papillary muscles. ED and ES measurements are obtained and
FS and EF are calculated. PW posterior wall
7 Assessment of Ventricular-Vascular Function by Echocardiography
145
septal defect. This causes paradoxical septal motion that the basal IVS moves away
from the inferolateral wall during systole. Septal hypokinesis and dyskinesis also
occur after open heart surgery. SF is also inuenced by preload and afterload. For
instance, it increases in mitral and aortic regurgitation while it decreases in immediate postoperative signicant patent ductus arteriosus. Therefore, SF does not
directly reect intrinsic myocardial function. In addition, hypertrophic myocardium
causes overestimation of systolic function in using FS.
7.1.1.1.2
Ejection Fraction
The most popular expression of global LV function is ejection fraction (EF). EF is a

measure of the ratio of stroke volume to end-diastolic volume with each contraction. Although EF is inuenced by loading conditions as well as SF, it is conrmed
as a predictor of outcome in various cardiac diseases and is used to select therapeutic strategy [2, 3]. EF may be determined by semiquantitative 2-dimensional
echocardiographic (2DE) LV images. Such visual assessment is fairly reliable when
performed by experienced interpreters and sonographers; however, it can cause
considerable interobserver variation [4]. Therefore, we should quantify EF by
volumetric measurements. EF is computed from M-mode, 2DE and
3-dimensional echocardiograms (3DE). M-mode recording of 2DE measurements
of LV dimensions from mid-ventricular papillary muscle level is used to calculate
the EF as follows (Fig. 7.1):

EF % LV-EDD3 LV-ESD3 = LV-EDD3 100
EF is preferably calculated from 2DE or 3DE volume measurements. The disk
summation or biplane Simpson method is often used. The LV endocardial border is
traced using one apical or two orthogonal apical views to create multiple cylinders
whose volume is summated to provide LV volume. The trabeculations and papillary
muscles are included as a part of LV cavity. Another crucial technical point for
reliably measuring LV volume is avoiding foreshortening of apical views. The
long-axis dimensions from two apical views should be similar. The biplane
Simpson method is preferable for measuring LV volume with regional wall motion
abnormalities. Normal EF values range between 54 and 75 % [1]. The smaller the
LV, the larger is the effect of the measurement error. This is particularly important
in the borderline LV in patients with aortic stenosis where calculations of LV
volume have a possibility of determining a biventricular vs. univentricular treatment. Real-time 3DE is more reliable and accurate for measuring LV volume and
this is in course of the standard mode of measuring LV volume and EF.
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7.1.1.1.3
M. Toyono
Velocity of Circumferential Fiber Shortening

and the Stress-Velocity Index
The ratio of LV ber shortening is noninvasively assessed by M-mode echocardiography. This measurement is termed mean velocity of circumferential ber shortening (Vcf). It is normalized for LV-EDD and is obtained from the following
equation:
Vcf circumferences=s LV-EDD LVE-SD = LV-EDD LV-ET
where ET represents ejection time. Reported normal values for mean Vcf are
1.5 0.04 circumferences (circ)/s for neonates and 1.3 0.03 circ/s for children
between 2 and 10 years of age [5, 6]. Vcf assesses not only the SF degree but the
rate at which this shortening occurs. To normalize Vcf for variation in heart rate,
LV-ET is divided by the square root of the RR interval to derive a rate-corrected
mean Vcf (Vcfc, circ/s):
Vcfc circ=s Vcf RR0:5
Normal Vcfc is reported to be 1.28 0.22 and 1.08 0.14 circ/s in neonates and
children, respectively [7]. Because Vcfc values are corrected for heart rate, a
signicant decrease in Vcfc between neonates and children is attributed to
increased systemic afterload with advancing age. Vcf is sensitive to the changes
in contractility and afterload. In contrast, Vcfc is relatively insensitive to the
changes in preload. Similar to SF, Vcf relies on the elliptical LV shape and is
invalid with altered LV geometry. For this reason, it is not suitable for some form of
congenital heart disease.
FS, EF, and Vcf are dependent on the LV loading state. When Vcfc is corrected
for afterload, it becomes a good parameter of contractility. An assumption has been
made to calculate wall stress based on the Laplace formula where wall stress in a
passive tube is related to pressure and size and is inversely related to wall thickness
[8]. Namely, higher LV pressure and larger LV size increase wall stress while
thicker wall decreases it. Wall stress is derived from M-mode echocardiographic
measurements, blood pressure measurements, and carotid pulse tracing.
End-systolic wall stress (ESWS) is the most important parameter determining
systolic shortening [9]. The formula to calculate ESWS is:

ESWS g=cm2 1:35 Pes LV-ESD = 4 hes 1 hes= LV-ESD
where 1.35 is the conversion factor from mmHg to g/cm2, Pes is the end-systolic
pressure derived from linear interpolation of the dicrotic notch on the pulse tracing,
and hes is the LV end-systolic wall thickness. ESWS differentiates states of
increased LV afterload from decreased LV contractility. A simplied formula
includes mean or peak systolic pressure instead of end-systolic pressure derived
from pulse tracing [7].
147
Abnormal LV contractility is dened as values for Vcfc-ESWS relation falling

below the normal expected range. In younger children, the linearity of the relationship has been questioned. A study on the growth-related changes in the stressvelocity index from preterm infants to pupils demonstrated that the slopes and
y-intercepts of the regression lines of Vcfc-ESWS relation are signicantly steeper
and greater in preterm infants and neonates compared with those in older children
[10]. The clinical application of stress-velocity index is somewhat limited by its
difcult acquisition and its time-consuming off-line analysis.
7.1.1.1.4
The Rate of Systolic Ventricular Pressure Increase (dP/dt)
Because SF, EF, and Vcfc are based on calculations of geometrical dimensions,
their use in congenital heart disease is partially limited. As an alternative to
geometrical measurements, Doppler echocardiography is used in the quantitative
evaluation of LV systolic function. If signicant mitral regurgitation is present, the
peak and mean rate of the change in LV systolic pressure (dP/dt) can be derived
from the continuous wave regurgitant Doppler signal. This rate of the change of LV
pressure is determined during the isovolumic phase of the cardiac cycle before
aortic valve opens. By the simplied Bernoulli equation, two velocity points along
the regurgitant Doppler envelope are selected from that and corresponding LV
pressure change is derived [11]. This change in LV pressure is then divided by the
change in the time between the two Doppler velocities to derive LV dP/dt (Fig. 7.2).
Practically, dt is calculated between 1 and 3 m/s: dP between those two points is
32 mmHg. dP/dt is subsequently calculated by the following formula:
dP=dtmmHg=s 32 = time interval in seconds
Fig. 7.2 Measurement of dP/dt. This image demonstrates Doppler velocity curve of mitral
regurgitation jet in a child with single LV and severe LV dysfunction
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M. Toyono
Normal value for mean dP/dt is reported to be >1,200 mmHg/s. Peak dP/dt
correlates accurately with invasive measurements [12]. To ascertain peak LV dP/dt
noninvasively, mitral regurgitant signal is digitized to obtain the rst derivative of
the pressure gradient curve from that of peak positive and peak negative dP/dt. The
same calculation is applied to the RV (sect. dP/dt) and the univentricular heart.
While dP/dt is reective of myocardial contractility, it is substantially affected by
changes in preload and partially afterload because it is measured before aortic valve
opening.
7.1.1.1.5
Myocardial Performance Index
Myocardial performance index (MPI, Tei index) is a Doppler-derived quantitative

measure of global ventricular function that comprises both systolic and diastolic
time intervals [13]. MPI is dened as the sum of isovolumic contraction time (ICT)
and isovolumic relaxation time (IRT) divided by ejection time (ET) (Fig. 7.3):
MPI ICT IRT=ET
The components of MPI are routinely measured from pulsed-wave Doppler

signals at the atrioventricular valve and ventricular outow tract of not only the
LV but the RV. To derive the sum of ICT and IRT, ET is subscribed from the
Doppler interval between cessation and beginning of the mitral valve inow signal.
Fig. 7.3 MPI. The mitral closure to opening time is measured on the mitral inow pattern as
shown in the left part of the picture. LV-ET is measured on the aortic outow as shown in the right
part of the picture
149
Increasing MPI values correlate with increasing degrees of global ventricular

dysfunction. Systolic dysfunction results in a prolongation of IRT and a shortening
of ET. Both systolic and diastolic dysfunctions result in abnormality in myocardial
relaxation which prolongs IRT.
Both adult and pediatric studies established normal values for MPI. In adults,
normal LV-MPI value is 0.39 0.05 [13]. In pediatric population, similar value for
the LV is reported to be 0.35 0.03 [14]. MPI is shown to be a sensitive predictor of
outcome of adult and pediatric population with heart disease as well as fetuses
[1518]. Because MPI comprises measures of both systolic and diastolic components, it is more sensitively an early measure of ventricular dysfunction in the absence
of other obvious changes in isolated systolic or diastolic indices by echocardiography.
In addition, because MPI is derived from Doppler, it is easily applied to the assessment of ventricular function even in complex ventricular geometries of congenital
heart disease [14, 19, 20]. MPI determined by tissue Doppler imaging (TDI) is also a
useful mean assessing global ventricular function. The modied index has the
advantage of simultaneous recording of systolic and diastolic velocity patterns
[21]. MPI has limitations like other indices. It is signicantly affected by changes
in loading conditions and has a paradoxical change with high lling pressure or
severe aortic valve dysfunction [22]. Finally, the combined nature of MPI fails to
promptly distinguish between systolic and diastolic ventricular dysfunction.
7.1.1.1.6
Tissue Doppler Imaging
TDI is presently added to the methods used in the clinical cardiology. TDI is less
load dependent than corresponding Doppler velocities of blood ow and has both
systolic and diastolic components (Fig. 7.4) [23]. Measurement of myocardial wall
Fig. 7.4 Typical longitudinal TDI tracing obtained in the basal IVS from the apical 4-chamber
view. Peak systolic velocity (S0 ), peak early diastolic velocity (E0 ), and peak late diastolic velocity
(A0 ) are measured. IVA is measured from the baseline to the peak and is indicated as the yellow
solid line
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M. Toyono
velocities by TDI is useful for the assessment of longitudinal systolic performance.

Relationship between pulsed-wave tissue velocities in healthy children is reported
by age group [24]. The systolic velocities of the mitral annulus correlate well with
LV-EF [25]. It is shown that the systolic velocity is a good predictor of clinical
outcome [26]. Signicant decrease in mitral annular systolic TDI velocities is
demonstrated in adult patients with LV dysfunction and elevated LV lling pressure [27]. Tissue velocities are rather geometry independent. This has important
implications in applying TDI velocities to congenital heart disease in which there is
a large variety of ventricular geometry. In dilated cardiomyopathy, systolic tissue
velocities are reduced in various segments [28, 29]. This is consistent with reduced
and heterogeneous systolic ventricular dysfunction in this disorder. In aortic valve
stenosis, systolic basal velocities are reduced in the septum and lateral wall
[30]. Systolic dyssynchrony is assessed by measuring the precise timing of peak
systolic velocity in the ejection phase with reference to the beginning of the QRS
complex [31].
During the ICT, another short-lived peak can be recorded. Myocardial acceleration during ICT (IVA) is an index of ventricular contractility. IVA is calculated as
average rate of myocardial acceleration during the ICT (Fig. 7.4). As the ICT is
short, high temporal resolution images are better for the IVA calculation. IVA is
validated as an index of LV and RV contractility that is unaffected by preload and
afterload within physiologic changes [32]. It is noted that IVA may be dependent on
preload when LV regional dysfunction exists [33]. IVA is reported to have heart
rate dependency [32].
TDI cannot differentiate between active contraction and passive motion, which
is a limitation in assessment of regional myocardial function [34]. Similar to other
Doppler modalities, TDI velocities are angle dependent. In addition, tissue velocities also measure cardiac translation; therefore, motion and velocity of a myocardial segment are inuenced not only by its own contraction but by adjacent
myocardial tethering. Therefore, regional myocardial dysfunction by TDI is difcult to identify when localized myocardial disease exists [35].
7.1.1.1.7
Strain Rate Imaging
Regional strain rate (SR) corresponds to the rate of regional myocardial deformation and is calculated from the spatial gradient in myocardial velocity between two
points within the myocardium. Regional strain represents the amount of deformation or the fractional change in length and is calculated by integrating SR curve
during the cardiac cycle (Fig. 7.5).
Strain measures the total amount of deformation in the radial, longitudinal, and
circumferential directions while SR calculates the velocity of shortening and is
expressed as second1 [36]. During systole, deformation is thickening in the radial
151
Fig. 7.5 Longitudinal LV strain in a normal subject. The picture represents the strain curves
obtained from the apical 4-chamber view
direction and shortening in the longitudinal direction. During diastole, deformation

is thinning in the radial direction and lengthening in the longitudinal direction.
Shortening is characterized by negative strain and SR while lengthening is characterized by positive strain and SR.
Strain % L1 L0 100 = L0
where L0 is the original length and L1 is the nal length.
SR =s V a V b = d
where VaVb is the instantaneous velocity difference at points a and b and d is the
distance between the two points.
These two measurements reect different aspects of myocardial function and
provide important information. In contrast to TDI velocities, strain and SR are not
inuenced by cardiac translation, rotation, or tethering of adjacent segments and,
therefore, are regarded as better indices of regional myocardial function. In the
normal heart, longitudinal strain rate values are similar from the base to the apex
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M. Toyono
unlike tissue velocity which is higher at the base than at the apex [37]. A reference
database of strain and SR has been obtained from healthy children [38].
Strain and SR are shown to be reduced in patients with dilated and ischemic
cardiomyopathy [39]. In patients with regional myocardial ischemia or bundle
branch block, heterogeneous contraction pattern is present [34, 40]. Therefore, it
is important to compare local measurements to global measurement for analyzing
regional wall motion. In this situation, myocardial thickening or shortening, i.e.,
postsystolic shortening, occurs after aortic valve closure [41]. Timing of aortic and
mitral valve closure is important to recognize presence of postsystolic shortening.
In patients with hypertrophic cardiomyopathy, there is severely reduced strain and a
substantial postsystolic shortening in the basal septum [42]. In less hypertrophied
regions, strain is higher and there is very little postsystolic shortening [43]. It is
demonstrated that values of strain and SR are inuenced by heart rate [44]. It is
shown that reference values for normal 2DE strain have a difference among
different vendors [45].
7.1.1.1.8
Three-Dimensional Echocardiography
The limited accuracy of M-mode and 2DE is attributed to the need for geometric
assumption which the LV is ellipsoid. The missing information on dimensions is
considered the main source of the wide inter-measurement variability of the
echocardiographic estimates of LV size and function. This is particularly tted to
congenital heart disease of which ventricles have distorted morphology and do not
follow geometric assumption. A special advantage of 3DE over 2DE is providing
full-volume datasets that overcome the need for geometric assumption of ventricles
(Fig. 7.6). Several studies comparing 3DE with magnetic resonance imaging as a
gold standard have shown the quantication of LV volumes and function is feasible,
accurate, and reproducible in both children with morphologically normal ventricles
and those with abnormal geometry [46, 47].
Visualization of the endocardial surface is challenging especially in the apical
and lateral myocardial segments. This is commonly compensated for by tilting a
transducer in 2DE. This maneuver generally improves endocardial visualization at
the expense of rendering foreshortened LV views. Finally, it results in an additional
source of error in calculating LV volumes by 2DE. In this regard, 3DE has an
additional advantage of image plane positioning that results in more accurate
chamber quantication.
As a large number of patients have coronary artery disease in adults, the
assessment of regional wall motion is frequently evaluated. Volumetric 3DE
imaging makes it possible to obtain complete dynamic information on all myocardial segments from a single dataset. 3DE during stress is feasible and useful for
detection of stress-induced wall motion abnormalities [48].
153
Fig. 7.6 3DE assessment of LV volumes. 3DE and semiautomated analysis of 3D volumes are
used to measure LV volumetric changes throughout the cardiac cycle. The images represent an
apical 4-chamber (left upper) and an apical 3-chamber (right upper) cut through the LV volume.
The planes through the volumes are illustrated in a left middle panel. On right middle and lower
panels, the result of volumetric analysis and the volume time curve are shown, respectively. MV
mitral valve
7.1.1.2
7.1.1.2.1
Diastolic Ventricular Function

Mitral Inow Velocity Wave
Mitral inow obtained by pulsed-wave Doppler echocardiography represents the

diastolic pressure gradient between the left atrium (LA) and LV. The early diastolic
lling (E) wave represents the peak LA-to-LV pressure gradient at the onset of
diastole. The deceleration time of the mitral E wave reects the time period needed
for equalization of LA and LV pressure. The late diastolic lling (A) wave represents the peak pressure gradient between the LA and the LV in late diastole at the
onset of atrial contraction. Normal mitral inow is characterized by a dominant E
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M. Toyono
Fig. 7.7 Normal mitral inow Doppler
wave, a smaller A wave, and a ratio of E-to-A waves between 1 and 3 (Fig. 7.7). The
normal E/A velocity ratio in children >2 years of age is 2.3 0.6 and A wave
duration is 140 21 ms. Mitral inow Doppler velocities are affected not only by
changes in LV diastolic function but by additional hemodynamic factors, including
age, altered loading conditions, heart rate, and changes in LA and LV compliance.
The diastolic lling pattern is characterized by measuring the time interval from
the peak of E velocity to its extrapolation to baseline that is called deceleration time
(DT). Normal duration of mitral DT varies with age. Its reference value is reported
in both pediatric and adult populations [49, 50]. In relaxation abnormality, DT is
prolonged because it takes longer for LA and LV pressures to balance with a slower
decrease in LV pressure until mid-to-late diastole. DT is shortened if there is rapid
lling due to active LV relaxation and elastic recoil as seen in normal young
subjects. DT is also shortened if there is a decrease in LV compliance or marked
increase in LA pressure. Nomograms for deceleration time vs. heart rate are already
reported [51]. IRT generally parallels DT. IRT dividing by the square root of the
cardiac cycle length indenties a corrected IRT of 63 7 in children [52].
The duration of mitral inow A wave is useful for estimating LV end-diastolic
pressure because it is shortened with a higher lling pressure [53]. In patients with
impaired relaxation and mild-to-moderate increase in lling pressures, the mitral
inow pattern resembles a normal lling pattern because of the opposing effects of
myocardial relaxation and increased lling pressures. Therefore, normalized lling
pattern due to moderate diastolic dysfunction is termed pseudonormalization.
In neonates and fetuses, reversed E and A waves as well as TDI early and late
diastolic waves are produced due to myocardial immaturity. Prolonged IRT is also
noted. The maturation from fetus to childhood pattern generally occurs by 3 months
of age [54].
7.1.1.2.2
155
Pulmonary Venous Flow Velocity Wave
Pulmonary venous (PV) Doppler provides an assessment of LA and LV lling

pressure. PV ow consists of four Doppler waves: 1 or 2 systolic waves (S waves), a
diastolic wave (D wave), and a reversal wave with atrial contraction (Ar wave). In
normal adolescents and adults, the characteristic pattern of PV ow consists of a
dominant S wave, a smaller D wave, and an Ar wave of low velocity and short
duration (Fig. 7.8). In neonates and younger children, a dominant D wave is often
present with a similar low-velocity and short duration Ar velocity or absent
[55]. The A wave velocity is 21 5 cm/s with duration of 130 20 ms [56]. The
normal S/D wave ratio in children from 3 to 17 years of age is 0.8 0.2.
First S wave is related to atrial relaxation, which decreases LA pressure and
promotes PV ow into the LA. Second S wave is produced by the increase in PV
pressure. At normal LA pressure, the late systolic increase in PV pressure is larger
and is more rapid than LA pressure. However, at elevated lling pressures, an
increase in the late systolic pressure is equal to or more rapid than that in the PV,
resulting in earlier peak of second S wave velocity [57]. S waves are closely
connected and a distinct rst component is not identied in 70 % of patients.
With worsening LV diastolic function, LA pressure increases and it leads to a
decreased S wave with a relatively increased D wave. In this situation, both velocity
and duration of Ar wave are importantly increased [51, 58]. An Ar wave duration
>30 ms as well as longer than the corresponding mitral A wave duration or a ratio
of Ar wave-to-mitral A wave duration >1.2 is predictive of elevated LV lling
pressure [59]. D wave velocity correlates with mitral E velocity because the LA
functions mainly as a conduit for ow during early diastole.
Fig. 7.8 Normal PV ow Doppler
156
7.1.1.2.3
M. Toyono
Mitral Inow Velocity Wave Combined With PV Flow

Velocity Wave
In determining diastolic dysfunction, abnormal relaxation and restrictive lling are

recognized as typical patterns. In the Doppler manifestations of diastolic dysfunction, patterns of ventricular lling gradually change: normal diastolic function,
abnormal relaxation, and restrictive lling.
Abnormal relaxation is considered as grade I diastolic dysfunction. This is the
initial abnormality seen in most forms of heart disease and ventricular compliance
usually remains normal. Abnormal relaxation is common in myocardial hypertrophy. Mitral E wave velocity is reduced and mitral A wave becomes dominant
(the E/A ratio <1). Impaired relaxation results in prolongation of IRT and mitral
DT. PV-D wave velocity decreases in parallel with mitral E wave. A mild increase
in PV-S wave is also observed. PV-Ar wave usually remains within normal limits at
this stage (Fig. 7.9).
As diastolic dysfunction advances, mitral E wave velocity increases. This is
primarily due to increasing LA pressure that is caused by reduced ventricular
compliance. Elevated LA pressure increases the transmitral ow gradient in early
diastole and subsequently restores a mitral inow pattern with a normal E/A ratio,
leading to pseudonormalization. This is grade II diastolic dysfunction and is
distinguished from truly normal lling in 1 of 2 ways. First, grade II dysfunction
demonstrates an abnormally large and long Ar wave (Fig. 7.10). Ar duration is
usually 30 ms longer than A wave duration. This is commonly associated with
moderate diastolic impairment, mildly to moderately elevated LA pressure, and
rising LV diastolic pressure.
Valsalva maneuver is useful in a situation of poor or confusing ow signals.
In the setting of grade II dysfunction, E wave velocity decreases, A wave velocity
increases, and DT lengthens during the maneuver. This maneuver decreases
preload and subsequently unmasks the underlying relaxation abnormality. In contrast, normal relaxation displays symmetrical reduction in E and A wave velocities
and DT does not change after the maneuver. In the pediatric population, the
difference between Ar wave duration and A wave duration also identies those
with elevated LV lling pressure only when the LV end-diastolic pressure
>18 mmHg [51].
The most advanced stage of diastolic pattern is restrictive lling. In markedly
reduced LV compliance, ow signals display a high velocity as well as short
duration E wave, rapid DT, and little ventricular lling with atrial contraction.
Mitral inow typically features the E/A ratio >2 and DT <150 ms [60]. S wave
velocity is decreased due to increased atrial pressure. E and D wave velocities
are increased due to elevated venous and atrial pressure. At this stage, Ar wave is
quite prominent as far as sinus rhythm is maintained and the LA contracts normal
(Fig. 7.11). The Ar duration is >30 ms longer than the A wave duration [59]. This is
grade III-to-IV diastolic dysfunction. Only this stage means an irreversible change
in ventricular function.
157
Fig. 7.9 Grade I diastolic dysfunction. Pulsed-wave mitral inow Doppler demonstrating abnormal relaxation pattern with the E/A ratio <1 (upper). Pulsed-wave PV ow Doppler (lower)
7.1.1.2.4
TDI
TDI velocities are shown to be clinically helpful in the distinction between normal
and pseudonormal transmitral Doppler lling patterns. In addition to changes
obtained by loading conditions, alterations in LA pressure and LV end-diastolic
pressure also affect mitral E wave velocity. However, the corresponding TDI
velocity is characteristically decreased in pseudonormal lling and allows differentiation of abnormal lling pattern from normal transmitral Doppler inow.
158
M. Toyono
Fig. 7.10 Grade II diastolic dysfunction. Mitral inow Doppler demonstrating pseudonormalized
pattern (upper). Pulsed-wave PV ow Doppler (lower)
A ratio of E wave velocity to lateral mitral annular early diastolic velocity (mitral
E/E0 ) is reported as a noninvasive measure of LV lling pressure. Nagueh
et al. demonstrated a signicant correlation of mitral E/E0 with invasively measured
mean pulmonary capillary wedge pressure [23]. Subsequent studies validated the
ratio and reported its applicability in a variety of hemodynamic settings [6163].
The ratio of E0 velocity to ow propagation velocity correlates closely with invasive
LV end-diastolic pressure. Septal E0 velocity correlates with the time constant of
relaxation (tau) [64].
159
Fig. 7.11 Grade III-to-IV diastolic dysfunction. Pulsed-wave mitral inow Doppler demonstrating restrictive lling pattern with increased E wave velocity, decreased A wave velocity, and the
E/A ratio >2 (upper). Pulsed-wave PV ow Doppler demonstrating decreased S wave velocity and
prolonged Ar wave duration
Early diastolic velocity of the mitral annulus measured with TDI is a good indicator
of LV myocardial relaxation [65]. This is one of the most important components
of myocardial diastolic function as well as LV compliance and lling pressure.
TDI records the velocity of the longitudinal motion. In the normal myocardial relaxation, E0 velocity of the mitral annulus increases with an increasing transmitral gradient, increasing preload, exercise, and dobutamine infusion [66, 67].
160
M. Toyono
Fig. 7.12 Grading of diastolic function based on mitral annulus velocity. The images represent a
normal diastolic function (left upper), grade I (right upper), II (left lower), and III-to-IV diastolic
dysfunction (right lower). Decreased E0 wave velocity of the mitral annulus is shown in all stages
of diastolic function
However, in case of impaired myocardial relaxation, E0 velocity is affected

less or even unchanged by transmitral gradient or preload [65, 66]. Therefore, a
decrease in E0 velocity is one of the earliest markers for diastolic dysfunction.
A decrease in E0 velocity is present in all stages of diastolic dysfunction [65].
Usually, E0 velocity from the lateral annulus is higher than that from the septal
annulus (Fig. 7.12).
Late diastolic velocity of the mitral annulus at the time of atrial contraction
increases during early diastolic dysfunction, as is the case for the mitral inow
A wave. In contrast, it decreases as atrial function deteriorates. Late diastolic
velocity of the mitral annulus is correlated with LA function [68, 69].
In adult patients with reduced LV function, mitral inow velocity alone is
usually sufcient to estimate lling pressures. A mitral inow E/A ratio >1.5 and
deceleration time <140 ms indicate increased lling pressures. In adult patients
with LV-EF > 40 %, the E/E0 ratio is the best parameter to estimate lling pressure.
The E/E0 ratio increases as pulmonary capillary wedge pressure increases. Pulmonary capillary wedge pressure is demonstrated to be >20 mmHg when the E/E0 ratio
is >10 using the lateral annulus velocity or >15 using the septal annulus velocity
[23, 70]. The E/E0 ratio works well even in patients with fused mitral inow signals
and those with atrial brillation [62, 71]. The only exception is patients with
constrictive pericarditis because E0 velocity is increased and the E/E0 ratio is
161
reduced with high lling pressures [72]. In contrast, restrictive cardiomyopathy has
decreased early diastolic and systolic TDI velocities [73].
In efcient myocardial relaxation which sucks blood from the LA to the LV
during early diastole, the time of onset of mitral inow E concurs with that of mitral
annulus E0 wave. However, in delayed myocardial relaxation and increased lling
pressure, onset of E wave depends more on the increased LA pressure and occurs
earlier than the onset of mitral annulus E0 wave. Therefore, the time interval
between the onset of mitral E wave and that of mitral annulus E0 wave increases,
and this increased interval is proposed as a variable to assess LV lling pressure
[74, 75]. A limitation of measuring cardiac time intervals by pulsed-wave Doppler
echocardiography is non-simultaneity because different cardiac cycles are needed
to measure various intervals. One solution is to have the capability of obtaining
multiple pulsed-wave recordings simultaneously. Another means to measure cardiac intervals from a single cardiac cycle is to use color M-mode from the anterior
mitral leaet [69].
A number of studies are performed in pediatric population to establish normal
reference values of TDI velocities [24, 51, 76, 77]. Similar to previous reports for
adults, pediatric TDI velocities vary with age, heart rate, ventricular wall and
location, and LV dimension as well as mass [65]. The E/E0 ratio is highest in
neonates and decreases with advancing age, primary due to an increased E0 velocity
over this period [24]. In infants and children, TDI velocities did not signicantly
correlate with LV-SF, LV and RV MPIs, and transmitral inow Doppler [78]. This
lack of correlation is likely that pulsed-wave TDI assesses longitudinal ventricular
function while other methods assess radial and global measures of ventricular
function. In patients with ventricular septal defect, the E/E0 ratio correlates with
invasively measured LV end-diastolic pressure [79].
7.1.2
RV
7.1.2.1
Systolic Ventricular Function
7.1.2.1.1
Fractional Area Change
One of the echocardiographic surrogates for RV-EF is measurement of percent

fractional area change (FAC). RV end-diastolic (EDA) and end-systolic areas
(ESA) are measured and FAC is calculated as (Fig. 7.13):
RV-FAC % 100 RV-EDA RV-ESA = RV-EDA
FAC has a reasonable correlation with MRI-derived EF [80]. However, in case
that delineation of the RV lateral wall is difcult, it may show inter- and intraobserver variability [81]. FAC is not reliable in the presence of RV outow
dysfunction because this part is not included in the measurement.
162
M. Toyono
Fig. 7.13 RV-FAC. From the apical 4-chamber view, the RV endocardial borders are traced.
RV-FAC (5535) 100/55 37 %
Fig. 7.14 TAPSE. An M-mode cursor is placed through the tricuspid valve annulus at the RV free
wall and longitudinal displacement of the annulus is measured along the line of the gure
7.1.2.1.2
Tricuspid Annular Plane Systolic Excursion
As the RV bers show mainly longitudinal orientation, longitudinal deformation of

the RV is more important compared with radial and circumferential deformation. An
easy method for assessing longitudinal function is by measuring tricuspid annular
plane systolic excursion (TAPSE) by M-mode echocardiography (Fig. 7.14).
163
In adults, normal excursion is >15 mm [1]. In children, it is dependent on ventricular

size and normal values are already published [82]. TAPSE is correlated well with
measurements of EF if there is no signicant regional RV dysfunction or tricuspid
regurgitation [83].
7.1.2.1.3
MPI
MPI is applied to any ventricular geometry [84]. The ability of MPI to quantitatively assess RV function is validated in adults and patients with congenital heart
disease [85, 86]. In addition, MPI has demonstrated prognostic power in differentiating outcome in RV failure [8790]. Care should be taken in using MPI for
congenital heart disease with altered loading conditions. Although, RV-MPI is
shown to be relatively independent of changes in chronic loading conditions, the
impact of acute changes in loading conditions are substantial.
MPI is a powerful variable for differentiating patients with idiopathic pulmonary
hypertension from normal subjects [18]. In adults, normal RV-MPI value is
0.28 0.04 [84]. In pediatric population, similar value for the RV is reported to
be between 0.28 0.07 and 0.37 0.04 [24, 76]. MPI is signicantly affected by
important pulmonary regurgitation after repair of tetralogy of Fallot. MPI determined by TDI is a useful mean assessing global ventricular function in these
patients [91].
7.1.2.1.4
dP/dt
dP/dt is used as a measure of RV systolic function when tricuspid regurgitant ow

velocity is recorded. RV-dP/dt is shown to have correlation with invasive measures
of RV hemodynamics [92]. Normal value of RV-dP/dt is reported to be
255 18 mmHg/s [93]. RV-dP/dt is also shown to be helpful in the assessment
of congenital heart disease [94]. Similar to the LV, RV-dP/dt is affected by changes
in loading condition.
7.1.2.1.5
TDI
Tricuspid annular motion is shown to correlate with RV function. Systolic annular

velocity well correlates with RV-EF [95]. TDI is shown to be a reproducible
noninvasive method of assessing systolic and diastolic annular motion and RV
function. While tricuspid annular velocities are affected by both preload and
afterload, they are demonstrated to be less inuenced by altered preload than
tricuspid inow Doppler.
Quantitative assessment of RV function after repair of tetralogy of Fallot
deserves careful attention. TDI velocities are decreased in these patients with
some regional RV wall motion abnormalities. At the apex, direction of myocardial
164
M. Toyono
velocities is opposite compared with the base. Interestingly, this correlates with
duration of QRS, which indicates that this is a parameter for degree of
dyssynchrony within the RV [96]. In addition, IVA is reduced in patients with
repaired tetralogy of Fallot, and it is related to a degree of pulmonary
regurgitation [97].
Paulikis et al. evaluated RV systolic velocities in children before and after
percutaneous device closure of atrial septal defects. Before closure, they had
increased tricuspid and mitral annular velocities compared with controls whereas
IVA was similar between the two groups. After closure, transient and immediate
decrease in TDI velocities in all myocardial segments was demonstrated while IVA
did not change evidently. Although TDI velocities normalized until 24 h after
closure, IVA remained unchanged [98]. These ndings demonstrate load dependence of TDI velocities and relative load independence of IVA in this setting.
7.1.2.1.6
SR Imaging
Quantitative assessment of RV function by strain and SR imaging has been

published concerning various congenital heart diseases. In patients with tetralogy
of Fallot, values of peak systolic strain and SR are reduced in the basal, mid, and
apical segments of the RV free wall as well as the IVS. The degree of reduction in
peak systolic SR of the basal segment of the RV free wall signicantly correlates
with QRS duration on electrocardiograms [99]. There is an inverse relationship
between peak systolic strain and SR at the RV base and the degree of pulmonary
regurgitation [100].
Postoperative patients with tetralogy of Fallot demonstrated that there is a
reduction in LV deformation parameters [99]. These patients are shown to have
LV asynchrony by measuring the time interval between the onset of the QRS
complex and peak strain in different segments. It is also shown that there is a
relationship between the degree of asynchrony and different parameters for global
and regional myocardial function. These patients have an increased paradoxical
septal motion that correlated with global LV function [101]. Based on these
ndings, effect of RV abnormalities on LV function through ventricular interaction
is important and strain and SR are helpful for this assessment in right-sided heart
disease.
Strain and SR imaging are applied to patients with systemic RV such as post
Senning or Mustard repair. Values of regional peak systolic strain and SR are
reduced in the basal, mid, and apical segments of the RV free wall. The values of
peak systolic strain are well correlated with EF obtained by cardiac MRI
[102]. Strain and SR imaging have a possibility of usefulness for serial assessment
of these patients. Reduced longitudinal RV strain and SR imaging are also demonstrated in patients with congenitally corrected transposition of the great
arteries [103].
165
Fig. 7.15 3DE calculation of RV volumes. The left upper panel represents the transverse plane,
the right upper conal plane, and the left lower the sagittal plane. The right lower panel is the
reconstructed RV volume with the tricuspid valve (TV) on the right and the RV outow tract
(OT) on the left
7.1.2.1.7
3DE
An accurate evaluation of RV volume and function is of great importance in

congenital heart disease. Conventional methods for estimation of LV volume
assume a prolate ellipsoid shape of the ventricle. However, these geometric
assumptions are not applicable to the RV due to a complex, asymmetrical, and
crescent shape. This makes estimation of RV volumes based on geometric assumption from 2DE image extremely challenging. By MRI, entire RV is visualized, and
RV volumes and function can be measured. Real-time 3DE emerges as an alternative to MRI for assessment of RV shape and function (Fig. 7.15). 3DE is validated
with MRI as a gold standard for calculation of RV volumes and EF in congenital
heart disease [104].
7.1.2.2
Diastolic Ventricular Function
Tricuspid inow velocity characterizes RV diastolic lling pattern with the use of
similar criteria of mitral inow velocity. The main difference between mitral and
tricuspid velocities in normal subjects is a respiratory variation of tricuspid ow
velocities.
166
M. Toyono
Hepatic vein ow velocities provide important hemodynamic insights. Hepatic

vein ow velocities reect changes in the pressure, volume, and compliance of the
right atrium. In normal subjects, hepatic vein ow velocities essentially consist of
four components: systolic forward ow, diastolic forward ow, systolic ow
reversal, and diastolic ow reversal. Under normal hemodynamics, systolic forward
ow velocity is higher than diastolic velocity, and there are no prominent reversal
velocities. Timing and respiratory changes in forward and reversal velocities of
hepatic vein ow are important in the assessment of various conditions, including
tricuspid regurgitation, constriction, tamponade, restriction, and pulmonary hypertension. As RV lling pressure increases, hepatic systolic ow velocities decrease
and diastolic ow velocity increases that is similar to the PV pattern. With marked
increase in RV lling pressure, a prominent ow reversal occurs during systole
and diastole. Severe tricuspid regurgitation produces a systolic ow reversal;
however, a systolic ow reversal does not always indicate severe tricuspid
regurgitation [105].
When RV compliance is substantially reduced and pulmonary artery pressure is
low, it is possible that atrial contraction opens the pulmonary valve and causes
forward ow into the pulmonary artery. This phenomenon is often observed in
chronic RV outow obstruction [106].
7.2
Arterial Function
7.2.1
Systemic Artery
7.2.1.1
Brachial Artery Reactivity Testing
Brachial artery reactivity (BAR) testing has been developed as a noninvasive

assessment of endothelial function [107]. Endothelial dysfunction precedes the
development of atherosclerotic plaque and is characterized by reduced bioavailability of nitric oxide that is a potent endothelium-derived vasodilator. BAR testing
consists of measuring (1) the reactive hyperemic ow after a brief period of arterial
occlusion and (2) the ow-mediated dilation (FMD) of the brachial artery in
response to hyperemia. Both measures are considered to be endothelium-dependent
responses: FMD reects conduit artery endothelial function and reactive hyperemia
reects microvascular endothelial function. BAR test is performed with standard
echo-Doppler equipment that has a 7.0 MHz or higher ultrasound scanning transducer [108]. Although upper arm occlusion produces a greater degree of reactive
hyperemia compared with forearm occlusion, there is a possibility of ischemia of
the wall of the brachial artery per se [109]. The measurement of reactive hyperemia
and FMD is followed by an assessment of vasodilation to nitroglycerin that is
167
considered an endothelium-independent response. Standardization of measurement

technique is needed for BAR testing.
7.2.1.2
Carotid Intima-Media Thickness
Intimal thickening of the carotid artery is considered a marker of systemic atherosclerosis. Because of the difculty in measuring intimal thickness alone by ultrasonography, the combined intima-media thickness (IMT) is measured. As the
carotid artery is an elastic artery and its media is not so thick, carotid IMT
represents mainly intimal thickening. The extracranial arteries are preferred for
testing because of their size and supercial location.
7.2.1.3
Arterial Stiffness
The aorta (Ao) and its major branches act as an elastic reservoir that provides a
hemodynamic cushion for cardiac pulsations. By that means, they help to convert
intermittent cardiac output to steady arterial ow. Stiffening of the central and
conduit arteries occurs with increasing age and accelerates in the presence of
cardiovascular risk factors. Eventually, it alters arterial pressure and ow dynamics
because cardiac performance and coronary perfusion are affected. Increased arterial
stiffness causes increased systolic (SBP) and decreased diastolic blood pressures
(DBP) which result in increment of pulse pressure and also increases cardiac
workload and vulnerability to ischemia. Increased arterial stiffness is associated
with increased risk of myocardial infarction, stroke, congestive heart failure, and
cardiovascular as well as overall mortality [110]. Therefore, it is quite likely that a
noninvasive assessment of arterial stiffness serves as a useful method of cardiovascular risk stratication and risk management.
7.2.1.3.1
Ao-Pulse Wave Velocity
Pulse wave velocity (PWV) is a velocity of a pressure wave that is generated with
each pulsation of the heart and is transmitted centrifugally along the arterial tree.
PWV is related to the biomechanical properties of the arterial wall. PWV is
generally measured using indirect arterial pressure and waveform with a
micromanometer-tipped piezoelectric transducer applied over an artery. The common carotid and femoral arteries are preferred for PWV measurement because they
are supercial and the distance between them covers most of the Ao length. Doppler
echocardiography-derived PWV is also measured in clinical settings [111]. In
healthy adults, Ao-PWV is generally 610 m/s [112]. Among hypertensive subjects, an Ao-PWV >13 m/s has a possibility of identifying those at especially high
cardiovascular risk [113].
168
7.2.1.3.2
M. Toyono
Ao-Augmentation Index
The forward moving arterial pressure wave is partially reected at the points of
impedance mismatch along the arterial tree and backs toward the central Ao. These
reecting points are usually bifurcations, branches, arterioles, and sites of discontinuity in arterial elastic properties. Augmentation index (AI) is a measure of the
contribution of the reected pressure wave to the central arterial pressure waveform. An ascending Ao-pressure wave form is derived from noninvasively obtained
waveform of radial artery or a ngertip using a generalized transfer function [114,
115]. AI is related inversely to heart rate and height and increases with age until
around fth decades and then plateaus or decreases [116]. This is in contrast to
PWV that increases with age without a plateau.
7.2.1.3.3
Other Values of Aortic Stiffness
Transthoracic 2DE along with noninvasive measurement of blood pressure provides simple and repeatable evaluation of the elastic properties of the ascending
Ao. This noninvasive method is validated in patients with coronary artery disease
and healthy subjects through comparison of data obtained from invasive methods
such as changes in Ao diameter and pressure [117]. Ao stiffness index (SI, stiffness
parameter ), Ao distensibility (D), and Ao strain (S) showing a percent change in
aortic diameter are calculated as follows [118120]:
Ao-SI ln SBP=DBP = Ao-ESD Ao-EDD = Ao-ESD
Ao-D cm=dyne 104 2 Ao-ESD Ao-EDD = Ao-EDD SBP - DBP
Ao-S % 100 Ao-ESD Ao-EDD = Ao-EDD
Using these methods, it is demonstrated that central Ao elastic properties of

patients after Kawasaki disease is altered and is independently correlated with
coronary artery aneurysms and LV hypertrophy [121].
7.2.2
Pulmonary Artery
Pulmonary vascular resistance (PVR) is an important hemodynamic variable in the

management of patients with congenital heart disease. PVR is conventionally
obtained by cardiac catheterization with the use of the following formula:
PVR Wood unit mean pulmonary artery pressure PCWP = cardiac output
Estimation of PVR with Doppler echocardiography is to divide tricuspid regurgitation velocity (TRV) by time integral velocity of the RV outow tract (RV-OT
TVI) [122]:
169
PVR Wood unit 10 TRV = RV-OT TVI 0:16

A cutoff value of 0.2 for TRV/RV-OT TVI separates a group with PVR > 2 Wood
units. Estimation of PVR with color M-mode-derived propagation velocity of the
pulmonary artery ow is also attempted [123]. The slope of the aliasing line on the
color M-mode of the main pulmonary artery ow decreases as PVR increases.
7.2.3
Coronary Artery
Assessment of coronary artery pathology and physiology is important for pediatric

and congenital heart disease. Doppler ow velocities of the coronary artery are
found to correlate well with invasive measurements by Doppler guide wire in adults
and pediatric studies. Normal values for Doppler ow velocities in the left coronary
artery are reported in a number of children and are also studied in the branch
arteries. Peak ow velocities during diastole range up to 60 cm/s in young children
and decrease with age and increase with heart rate [124128].
Coronary ow reserve (CFR) reects the increase in coronary ow in response to
hyperemia by adenosine triphosphate, dipyridamole, and exercise. It is calculated
as the ratio:
CFR peak velocity after hyperemia = baseline peak velocity
Peak velocity is measured during diastole. Mean velocity can be used instead of
peak velocity. CFR reects the resistance of the coronary bed, its autoregulatory
ability, and the ability to augment blood ow in response to stress. CFR is affected
by stenosis of the proximal coronary arteries in Kawasaki disease [125] or by
abnormalities in the coronary microcirculation in dilated cardiomyopathy [129].
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Chapter 8
Assessment of Hemodynamics by Magnetic

Resonance Imaging
Masaya Sugimoto
Abstract In recent years, remarkable technological revolutions in cMRI methods
have made it possible to better ascertain patients conditions. For example, cine MRI
makes it possible to take moving images while the patient is holding their breath, has
excellent time resolution, and easily and accurately evaluates cardiac function.
Compared to echocardiography, it provides a superior visual overview of blood
vessels and allows the user to freely obtain section images without the interference
of bones, the lungs, and other structures. The use of phase contrast technique makes
it possible to perform quantitative evaluation of shunt volume and all types of
valvular regurgitation. An imaging technique that allows the imaging of myocardial
strain has also been developed, which has further improved the ability of MRI to
evaluate cardiac function and cardiac wall motion. When MRI contrast medium is
used, MRI is superior to myocardial scintigraphy for the diagnosis of myocardial
ischemia, myocardial infarction, and cardiomyopathy. Currently, cMRI is an essential tool for the evaluation of cardiac function in the eld of pediatric cardiology.
Keywords Cine MRI Delayed contrast-enhanced MRI Tagging cine MRI
Velocity-encoded cine MRI
8.1
8.1.1
An Overview of Functional Evaluation Using MRI

Introduction
Methods for evaluating cardiac function include cardiac catheterization, echocardiography, nuclear cardiology, multidetector CT (MDCT), and cardiac magnetic
resonance imaging (cMRI). Patients with congenital heart disease (CHD) require
long-term observation over the course of their treatment and inevitably require
frequent cardiac catheterization. Cardiac catheterization is an extremely important
test that facilitates the measurement of blood pressure, blood ow volume, and
ventricular capacity, but because it is invasive, it is difcult to perform repeatedly.
M. Sugimoto, MD, PhD (*)
Department of Pediatrics, Asahikawa Medical University, 2-1-1-1 Midorigaoka, Higashi,
Asahikawa 078-8510, Japan
e-mail: masaya5p@asahikawa-med.ac.jp
Springer Japan 2015
DOI 10.1007/978-4-431-54355-8_8
177
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M. Sugimoto
Since echocardiography is noninvasive and can be easily performed at a patients

bedside, it is considered the gold standard method for cardiac assessment. However,
limitations of this method include the fact that obesity and the lungs can block the
echo beam, sometimes making it impossible to obtain clear images, in addition to
poor repeatability between different testers or even among different tests performed
by the same tester. MDCT is useful for evaluating cardiac morphology, and nuclear
cardiology is useful for evaluating cardiac function; however, both these tests
expose the patient to radiation, and, therefore, they are not recommended for
repeated use in pediatric patients. However, cMRI can be used not only for
evaluating the morphology of the major great vessels but also for simultaneously
evaluating cardiac function, and because it does not expose the patient to radiation
and is noninvasive, it can be repeated. This technique represents a groundbreaking
innovation in the eld of pediatric cardiology and cardiac surgery and may become
the future gold standard of tests.
In recent years, remarkable technological revolutions in cMRI methods have
made it possible to better ascertain patients conditions. For example, cine MRI
makes it possible to take moving images while the patient is holding their breath,
has excellent time resolution, and easily and accurately evaluates cardiac function.
Compared to echocardiography, it provides a superior visual overview of blood
vessels and allows the user to freely obtain section images without the interference
of bones, the lungs, and other structures. The use of phase contrast (PC) makes it
possible to perform quantitative evaluation of shunt volume and all types of
valvular regurgitation. An imaging technique that allows the imaging of myocardial
strain has also been developed, which has further improved the ability of MRI to
evaluate cardiac function and cardiac wall motion. When MRI contrast medium is
used, MRI is superior to myocardial scintigraphy for the diagnosis of myocardial
ischemia, myocardial infarction, and cardiomyopathy. Currently, cMRI is an essential tool for the evaluation of cardiac function in the eld of pediatric cardiology.
8.1.2
cMRI Features
8.1.2.1
Electrocardiogram (ECG) Gating
A heartbeat is a complex movement that combines longitudinal contractions, shortaxis contractions, and rotational motion. The use of ECG gating improves image
quality because it collects signals of specic cardiac phases from several heartbeats
and minimizes the effect of the heartbeat motion.
8.1.2.1.1
Prospective ECG Gating
The ECG R wave acts as a trigger to initiate imaging. Although between 80 and
90 % of the signal of the RR interval is captured, end-diastole is lost because the
8 Assessment of Hemodynamics by Magnetic Resonance Imaging
179
signal for the last 1020 % of the imaging time is not captured. Therefore, this
method is suitable only for still images and systolic phase cine imaging.
8.1.2.1.2
Retrospective ECG Gating
This method takes images by using a specic repetition time (TR) unrelated to ECG
before matching these images to MR signals based on ECG signals that were
simultaneously recorded. Normally, an RR interval oversampling of 125 % is
made and is later compared to R waves to determine from which phases the signals
originated. This method includes nearly all cycles, but depending upon the
sequence, one cardiac cycle is equivalent to systole and diastole. Since pediatric
patients have a fast heart rate, the systole interval is wide, causing the image quality
to deteriorate. Thus, a method for reducing one cardiac cycle is required. GRE and
other cine imaging methods are the most effective ways to produce phase contrast
images.
8.1.2.2
Respiratory Gating
Respiration-related body movements can be compensated for by the patient holding

their breath or by respiratory gating. An adult human can hold their breath for 15
25 s, but as this time is reduced in patients with cardiopulmonary diseases, there are
limitations to this method. It is almost impossible for children under school age to
hold their breath. Respiratory gating tracks the movements of the diaphragm either
indirectly by using a bellows around the chest and abdomen or directly by using a
navigator echo pulse. Both methods track both the direction and the depth of
respiration-related body movements and stop collecting data when these exceed a
pre-set range. However, since Korperich et al. found that there was no difference in
measured values between blood ow measurement values gated to respiration and
those not gated to respiration in CHD patients [1], respiratory gating is probably of
lower priority than is ECG gating in the eld of pediatric cMRI [2].
8.1.2.3
Typical Sequences
The names and details of the sequences differ between manufacturers, and there are
differences in the images themselves as well.
8.1.2.3.1
Gradient Echo (GRE) (SPGR, FLASH, Field Echo)
This sequence is mainly used during cine MRI. GRE uses free induction decay
(FID) that occurs immediately after RF pulse irradiation to create images. When
used in cine MRI, blood owing into the slice section is not affected by signal
180
M. Sugimoto
saturation, causing a relatively high signal intensity to be displayed, as compared

with the signal intensity of the myocardium. However, if there is turbulence, a
low-intensity signal is obtained. This sequence is useful in evaluating ventricular
volume, ejection fraction, wall movement, and anatomical morphology.
8.1.2.3.2
Steady-State Free Precession (SSFP) (True FISP, FIESTA,

Balanced TFE)
SSFP is used in cine MRI, coronary artery MRA, myocardial perfusion MRI, and
other types of imaging and is the main cMRI sequence. SSFP irradiates RF pulses
repeatedly at short intervals and receives all types of ultrasound and FID signals
that occur when equilibrium is reached. SSFP provides high signal intensity for
blood, pericardial uid, and pleural uid, regardless of ow.
8.1.2.3.3
Phase Contrast (PC)
PC is a sequence used when performing velocity-encoded cine MRI (VEC-MRI).

PC combines bipolar magnetic eld gradients with cine MRI for imaging phase
shift changes. The use of PC makes it possible to measure ow velocity in all target
anatomical regions. The average ow velocity through the heart chamber and blood
vessel sections during each cardiac phase can also be measured. Cardiac output,
pulmonary-systemic ow ratio (Qp/Qs), and the atrioventricular, aortic, and pulmonary valve regurgitation volume and regurgitation fraction can be quantitatively
measured.
8.1.3
Precautions When Using cMRI in Pediatric Cardiology
When performing imaging in pediatric CHD patients, it is essential to receive

specialist advice regarding the anatomical position of structures. Infants have
small bodies and hearts, and their heart rate is 100 beats/min, which means that
rotational and other positional relations vary widely among individuals. This makes
it necessary to coordinate a number of parameters to nd the appropriate settings
(Fig. 8.1). The level of operator skill is an important element in producing better
images. In addition, because testing takes 30 min and the device is noisy, pediatric
patients must be kept sufciently sedated in order to obtain measurements. However, since oversedation lowers both the systemic blood pressure and the respiratory
rate, care must be taken because the subjects hemodynamics may not necessarily
be the same as when not sedated [3]. Furthermore, since testing of patients with
pacemakers is contraindicated, areas where stents or coils are present are difcult to
image, and care must be taken to ensure that image quality does not deteriorate
when arrhythmia occurs.
181
Fig. 8.1 Cardiovascular MR setup for pediatric general anesthetic cases. View of the MR scanner
room showing the monitoring equipment
The three imaging techniques listed below represent the three groups in which
the subjects in this study were divided for the purpose of performing hemodynamic
evaluation using cardiac function analysis/MRI in CHD patients. A detailed discussion of each technique is also included.
1. Cine MRI
2. VEC-MRI
3. Other: MRI with contrast and tagging cine MRI
8.2
8.2.1
Evaluation of Cardiac Function by Using MRI:

Detailed Discussion of the Methods Used
Cine MRI
The cine MRI technique has the advantage of allowing the operator to take video
images of the heart in segments in any direction without interference from bones or
air [4]. It is the most accurate method to evaluate cardiac function and wall
movement and has high reproducibility. Iodinated contrast media must be administered to the patient when MDCT is used for cardiac function evaluation, and rapid
intravenous injection of contrast medium may affect cardiac function. However,
since cine MRI does not involve exposure to radiation, contrast medium is
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M. Sugimoto
unnecessary, making it a highly noninvasive procedure. Further, in MDCT, the

heart rate of patients is effectively lowered by beta-blocker administration, causing
reduction of artifacts and radiation exposure; however, the operator needs to be
careful while performing MDCT after the beta-blocker administration to prevent a
signicant increase in left-ventricular end-diastolic and end-systolic volumes,
which can cause a drop in cardiac output [5]. In contrast, since cine MRI does not
require beta-blocker administration, accurate evaluation of cardiac function in the
physiological state is possible.
8.2.1.1
Characteristics and Imaging Technique of Cine MRI
The cine MRI technique uses ECG gating, imaging 1640 video frames of heart
movement per beat. The SSFP imaging technique obtains high-intensity blood
signals without the use of contrast media and clearly renders both the inner and
outer sides of the pericardium at the left ventricle [68]. This sequence refocuses all
gradients of three axes in a single TR in order to obtain the signals, making it
possible to obtain powerful signals in a steady state. Imaging in TR and echo time
(TE) is possible, which reduces the imaging time to less than half of that required by
the GRE technique. Since contrast is determined by T2/T1, the myocardium is
shown with a low-intensity signal and blood is shown with a high-intensity signal.
Further, since this technique does not rely upon inow effects, its advantages are
that even slow blood ow can be shown with a high-intensity signal and that lumen
is shown with a uniform high-intensity signal. Fat and pericardial effusions are also
shown with a high-intensity signal, and the border of the outer margin of the
myocardium is also clearly shown; however, care must be taken because it is
difcult to distinguish between fat and pericardial effusions.
The cine MRI procedure is as follows: after performing cine MRI of the vertical
and horizontal longitudinal sections, serial cine-MRI sections that cover the entire
left ventricle from the mitral valve to the apex are obtained, and, when necessary,
four-chamber and three-chamber long-axis cine MR images that show the leftventricular outow tract are also generally obtained. Since each cine image slice is
a series of images of different cardiac phases, one slice includes images of several
phases (Fig. 8.2). Thus, not only is the time that a patient must hold their breath
Fig. 8.2 Cine MRI using steady-state free precession. The panels are typical long-axis views; the
left panel shows systole and the right panel shows diastole. MR images of a 1-year-old patient with
mitral valve regurgitation
183
reduced, but image quality is also improved. In the past, a single slice required the
patient to hold their breath for approximately 20 s, but when steady state is used,
this time is reduced to approximately 7 s. Further, when parallel imaging is
concurrently used, this time can be further reduced to approximately 4 s, without
any loss of image quality.
ECG gating of cine MRI can be either prospective or retrospective. In general,
prospective ECG gating requires a shorter imaging time, but the signal for the last
1020 % of the cardiac cycle, i.e., the end-diastole, cannot be obtained, which
means that the cardiac cycle data are incomplete. This is a particularly important
issue for physiological and functional images. Retrospective ECG gating is used
when data for the entire cardiac cycle are required. Since cine imaging using ECG
gating requires the patient to hold their breath, image quality deteriorates if
arrhythmia occurs or if the patient does not hold their breath completely.
8.2.1.2
Analysis of Cardiac Function
Volume analysis using echocardiography and left ventriculography calculates

approximate spheroid and other shaped geometric models of the left ventricle.
Thus, errors increase when there are morphological abnormalities in the left
ventricle. Cine MRI has the advantage of allowing the operator to accurately set
the direction of the image slices freely so that they are taken at regular intervals and
in parallel. When cine MRI and the Simpson technique are used, deformation of the
left ventricle leads to almost no deterioration in accuracy, making it possible to
obtain ideal cardiac function measurements (Fig. 8.3) [9, 10]. Specically, leftventricular short-axis cine images from the base to the apex are taken in succession,
and each slice traces the outer pericardial edge of the myocardium during both the
diastole and systole. Thus, left-ventricular end-diastolic volume, left-ventricular
end-systolic volume, and myocardial mass can be calculated (Fig. 8.4). Even in
cases of left-ventricular deformation, localized thickening of the wall, and lump
formation, accurate analysis of volume and myocardial mass is possible. Thus,
currently, cine MRI is the gold standard for the evaluation of ventricular volume
[11, 12]. When measuring the rate of systolic wall thickening from the localized
areas of myocardial wall thickness by using cine MRI during the diastole and
systole, a more accurate diagnosis of localized myocardial contractility can be
Fig. 8.3 Cine MRI used to assess cardiac index. The MR images are from the same patient as that
in Fig. 8.1, i.e., a 1-year-old patient with mitral valve regurgitation. By measuring end-diastolic
and end-systolic endocardial borders on all slices, stroke volume can be calculated
184
M. Sugimoto
LV cavity volume (ml)
130
110
90
70
50
0
200
400
600
800
Time (msec)
Fig. 8.4 Timevolume curve of the left ventricle during the cardiac cycle in a 13-year-old patient
with mitral valve regurgitation. Short-axis cine imaging using contiguous 8-mm-thick slices and
2-mm slice gaps
Fig. 8.5 Cine MRI used to assess right-ventricular volume and ejection fraction. The MR images
are from the same patient as that in Fig. 8.1, i.e., a 1-year-old patient with mitral valve regurgitation. By measuring the end-diastolic and end-systolic endocardial borders on all slices, stroke
volume can be calculated
made than is possible with visual evaluation. Further, since all frames trace the
short axis, diastolic function can be evaluated on the basis of timevolume curves.
In the eld of pediatric cardiology, testing of ventricular volume and diastolic
function determines the timing of surgery, and it is very important to do this after
diagnosing and treating cardiac insufciency. In many cases of CHD, it is important
to evaluate not only left-ventricular function but also right-ventricular function.
Since the right ventricle has a complicated three-dimensional shape, unlike the left
ventricle, the accuracy of measurements of the right-ventricular capacity taken by
echocardiography and cardiac catheterization is severely limited. However, axial
transverse section slices taken using cine MRI allow accurate evaluation of rightventricular volume and diastolic function (Fig. 8.5) [13]. After surgery for tetralogy
of Fallot (TOF), complications such as pulmonary stenosis and pulmonary regurgitation are often observed, and these conditions can cause right-ventricular
185
hypertrophy, enlargement, and right-ventricular insufciency over the long term.

Geva et al. evaluated cardiac function after surgery for TOF in patients by using
MRI and reported the risk factors [14]. Schmitt evaluated cardiac function during
exertion after the Fontan procedure in patients by using MRI [15]. As shown in the
abovementioned and many other studies, cine MRI is used more often in the
evaluation of cardiac function than is any other technique in cases of CHD [16, 17].
8.2.1.3
Disadvantages of Cine MRI
One of the disadvantages of cine MRI is that images become blurred when
arrhythmia is present. Arrhythmia causes errors to occur when this technique,
which involves imaging using ECG gating, is used. In clinical settings, the time
required for posttreatment is also a major problem. Most of the types of measurement software currently in use recognize the lumen and have functions that allow
them to automatically set the borders. However, these settings require that an
operator check and correct the borders. Especially when assessing CHD in pediatric
patients, specialist advice regarding the anatomical position of structures is
required. When patients cannot hold their breath reliably or sufciently long
enoughpediatric patients are completely unable to hold their breathor when
imaging is performed while the patient is breathing normally, the position of the
heart changes, owing to the movements of breathing. The resulting image may be
blurred, the tracing function of the measurement software may be impossible to use,
and measurement errors may occur. In the future, it will be necessary to speed up
the imaging time and develop posttreatment methods.
8.3
Velocity-Encoded Cine MRI (VEC-MRI)
In general, cardiac catheterization and echocardiography are used for quantitative

measurements of cardiac output and volume of blood ow. Cardiac catheterization
can be used to perform quantitative measurements using the Fick principle and
thermodilution; however, this method has disadvantages in that it is invasive.
Echocardiography has the advantage of being a noninvasive bedside technique
that can be performed at any time. Thus, it is the rst diagnostic method of choice
for blood ow volume measurement. However, this method also has disadvantages
in that it is often difcult to obtain images with satisfactory quality or a good
incidence angle of the ultrasonic beam and reproducibility is poor between different
testers and even when performed multiple times by the same tester. Among the cine
MRI techniques, VEC-MRI using the PC method has been attracting attention as a
less invasive and safer measurement technique than the invasive cardiac catheterization method, because it can accurately measure blood ow volume as the ow
velocity of each cardiac phase of a freely selected blood vessel section even if the
intravascular blood ow distribution is complex.
186
8.3.1
M. Sugimoto
Principle and Technique of VEC-MRI

for Blood Flow Measurements
VEC-MRI allows noninvasive measurements of blood ow velocity in the heart and

blood vessels, including those locations where an ultrasonic beam cannot reach.
VEC-MRI switches the polarity of the gradient magnetic eld for blood ow
measurements and collects two types of MRI data. On comparing the phases of
the images obtained, the same phases are observed for stationary objects, but for
moving objects, phase differences are observed that are proportional to the speed of
the moving objects, which makes it possible to measure the velocity of movement.
In VEC-MRI, the mean value [the intensity within a region of interest (ROI), which
represents the blood ow velocity] of every image pixel can be calculated in images
from diastole to systole. The methods include the use of body array coil and spine
array coil. To ascertain the blood ow direction in the blood vessels whose blood
ow volume is desired, PC is used to make the sections vertical, and then, cine MR
images are obtained (Fig. 8.6a). After several minutes of imaging time for each
slice have elapsed, magnitude images (Fig. 8.6b) and PC images (Fig. 8.6c) are
obtained. The ROI of the target blood vessel is set, and blood ow velocity is
measured using the analytical software included in the imaging equipment
(Fig. 8.7). A timevelocity curve is then constructed using the average velocity of
the blood ow passing through the ROI during one cardiac cycle (Fig. 8.8a).
A timeow curve is then produced by multiplying the velocity curve with the
area of the ROI (Fig. 8.8b). The blood ow volume (timenet ow curve) passing
through the ROI can be obtained by integrating the owvolume curve (Fig. 8.8c).
8.3.2
Investigation of Usefulness in Pediatric Patients
We investigated the usefulness of VEC-MRI for measuring the following

data in pediatric patients with CHD (Table 8.1): pulmonary blood ow (Qp),
Fig. 8.6 (a) Localization of the left pulmonary artery. Phase-mapping sequence applied perpendicular to the left pulmonary artery axis. The magnitude (b) and phase contrast (c) images allowed
measurement of the vessel cross-sectional area, ROI
187
Fig. 8.7 The vascular cross

section is divided in many
pixels. Momentary velocity
of each pixel is measured
using VEC-MRI. Average
velocity is obtained by
dividing the sum of these
velocities by the number
of pixels
100
80
Flow (ml/sec)
Velocity (cm/sec)
60
40
20
0
0
Flow (ml/sec)
200
400
600
Time (msec)
-20
Time (msec)
1600
1400
1200
1000
800
600
400
200
0
0
200
400
600
Time (msec)
Fig. 8.8 (a) Timevelocity curve: the cross axis indicates one cardiac cycle and the vertical axis
indicates velocity. (b) Timeow curve: velocity is multiplied by the area of the ROI; the cross
axis indicates one cardiac cycle and the vertical axis indicates ow volume. (c) Timenet ow
curve: ow volume is added during one cardiac cycle; the cross axis indicates one cardiac cycle
and the vertical axis indicates true blood ow
188
M. Sugimoto
Table 8.1 Characteristics of patients

Without shunt (including ICR)
Ventricular septal defect (VSD)
Atrial septal defect (ASD)
Patent ductus arteriosus (PDA)
Tetralogy of Fallot (ToF)
Double-outlet right ventricle (DORV)
Atrioventricular septal defect (AVSD)
Transposition of the great arteries (TGA)
Single ventricle (SV)
Coarctation of the aorta (CoA)
Aortic valve stenosis (AS)
Total
With shunt
5
5
4
3
1
1
1
2
3
4
5
15
19
ICR intracardiac surgical repair
(b) VSD, PH
100
(a) normal
Velocity (cm/sec)
80
(c) ASD
60
(d) VSD,Eisemmenger
40
20
0
0
-20
1
Cardiac cycle
Fig. 8.9 The velocity curve for normal pulmonary arteries is represented by an isosceles triangle
(a). In the case of VSD (b), where the systolic pulmonary arterial pressure was high, the period
from the start of ejection to the peak was much shorter than normal. In contrast, the ejection period
of the velocity curve pattern was longer, reecting an increase in pulmonary blood ow. However,
in cases of ASD (c), where there was no pulmonary hypertension, the velocity curve pattern had a
wider base along the time axis, which is similar to VSD, but the period from the start of ejection to
the peak was the same as normal. Moreover, in cases of VSD with Eisenmenger syndrome (d), the
period from the start of ejection to the peak was extremely short, and the ejection time was shorter
than normal
cardiac output (CO), and pulmonary blood ow/systemic blood ow ratio (Qp/Qs).
The velocity curve for normal pulmonary arteries is represented by an isosceles
triangle (Fig. 8.9a). In the case of ventricular septal defect (VSD) (Fig. 8.9b), where
the systolic pulmonary arterial pressure was high, the period from the start of
MRI-Qp [ rt+lt] (/min)
Fig. 8.10 Comparison

between the blood ow
volume of the main
pulmonary arteries
(MRI-Qp) and the sum of
that of the right and left
pulmonary arteries
(MRI-Qp [Rt + Lt]),
measured using VEC-MRI
189
5
4
3
2
y = 0.96x 0.09
R= 0.97
(p< 0.001) , n= 29
1
0
0
MRI-Qp (/min)
ejection to the peak was much shorter than normal. In contrast, the ejection period
of the velocity curve pattern was longer, reecting an increase in pulmonary blood
ow. However, in cases of atrial septal defect (ASD) (Fig. 8.9c), where there was no
pulmonary hypertension, the velocity curve pattern had a wider base along the time
axis, which was similar to VSD, but the period from the start of ejection to the peak
was the same as normal. Moreover, in cases of VSD with Eisenmenger syndrome
(Fig. 8.9d), the period from the start of ejection to the peak was extremely short, and
the ejection time was shorter than normal.
(a) Comparison of the sum of the blood ow volumes of the main pulmonary
artery (MRI-Qp) and the right and left pulmonary arteries (MRI-Qp [Rt + Lt])
(Fig. 8.10)
We measured the blood ow volumes of the main pulmonary artery and the
right and left pulmonary arteriesthree vessels that differ in direction and
diameterto determine if the sum of the blood ow volumes of the two
vessels matches that of the third vessel, which should normally be the same.
Our results showed an extremely good correlation. This conrms the reproducibility and accuracy of VEC-MRI to measure blood ow in pediatric
patients, including infants.
(b) Comparison of cardiac output (Fig. 8.11)
The use of thermodilution with a SwanGanz catheter is the gold standard for
measuring cardiac output. However, it is difcult to use this method in
pediatric patients. Caputo et al. performed phantom experiments that showed
that cardiac output measured using VEC-MRI was slightly lower than the
values obtained using echocardiography [18]. Kuehne et al. used swine in their
comparison between VEC-MRI and thermodilution, but to our knowledge,
almost no studies have been conducted on pediatric patients [19]. Our study on
190
M. Sugimoto
6
y = 0.78 x + 0.35
R = 0.68
(p< 0.005) , n= 18
MRI - Qs (/min)

between the systemic blood
ow measured using
VEC-MRI (MRI-Qs)
and that measured using
cardiac catheterization
with thermodilution
(Thermo-Qs)
4
3
2
1
0
Thermo -Qs (/min)

a
b
3
y = 0.494x + 0.242
R = 0.705
(p< 0.005) , n= 13
MRI - R/L
R/L = 681/414 = 1.64
0
0
Scinti - R/L
Fig. 8.12 (a) Pulmonary perfusion scintigraphy image of a 3-year-old patient with left pulmonary
arterial stenosis after intracardiac repair of TOF. (b) Comparison between the ratio of right and left
pulmonary blood ow measured using VEC-MRI (MRI-R/L) and that measured using scintigraphy (Scinti-R/L)
pediatric patients showed a good correlation between the use of

thermodilution and VEC-MRI to measure cardiac output.
(c) R/L comparison of the blood ow volume of the right and left pulmonary
arteries and R/L comparison of pulmonary perfusion scintigraphy (Fig. 8.12)
191
Pulmonary perfusion scintigraphy is a physiological function test that allows a

comparison between the right and left pulmonary blood ow. Osada
et al. compared the use of VEC-MRI and pulmonary perfusion scintigraphy
for assessing pulmonary blood ow in adults not having heart disease and
obtained satisfactory outcomes [20]. Ordovas et al. evaluated the use of
VEC-MRI and pulmonary perfusion scintigraphy for assessing right and left
pulmonary blood ow in postoperative congenital heart disease patients
[21]. Our study on pediatric patients also showed a good correlation between
the R/L comparison of scintigraphy and that of MRI. Fratz et al. compared the
use of VEC-MRI and pulmonary perfusion scintigraphy for assessing the right
and left pulmonary blood ow in patients who had undergone the Fontan
procedure and found that VEC-MRI was more accurate [22]. This is because
VEC-MRI not only provides a comparison of blood ow but also shows blood
ow volume. Further, R/L comparison of pulmonary perfusion scintigraphy
after the Glenn procedure and the Fontan procedure in patients showed that
radioisotope (RI) had to be intravenously injected at several sites, but when
VEC-MRI was used, this was not necessary, suggesting that the target values
could be obtained with a single test.
(d) Comparison of pulmonary to systemic blood ow ratio (Qp/Qs) in CHD
patients (Fig. 8.13)
The use of VEC-MRI to measure the blood ow volume of the ascending aorta
and pulmonary arteries in CHD patients allows the noninvasive measurement
of the left and right shunt blood ow volume and Qp/Qs. Qp/Qs is an
important indicator that helps determine whether surgery is appropriate for
CHD patients with ASD, VSD, or other conditions. Brenner et al. compared
5
MRI - Qp/Qs
y = 0.933x
0.028
R = 0.897
(p< 0.001) , n= 19
1
between Qp/Qs measured
using VEC-MRI (MRI-Qp/
Qs) and that measured
using the Fick method
(Fick-Qp/Qs)
0
0
Fick - Qp/Qs
192
M. Sugimoto
Qp/Qs values that were obtained using VEC-MRI and echocardiography in

adults with a long history of ASD [23]. Peterson et al., in their study on VSD
and patent ductus arteriosus (PDA) in addition to ASD, showed that there was
a good correlation between Qp/Qs values obtained using VEC-MRI and those
obtained using oximetry in cardiac catheterization tests performed on 17 adult
patients [24]. Beerbaum et al. found a good correlation between Qp/Qs values
obtained using VEC-MRI and those obtained using cardiac catheterization in
pediatric ASD patients [25].
We performed the same comparison in 19 pediatric CHD patients. These
patients included those who had diseases other than ASD and VSD, for
example, TOF and other diseases characterized by low pulmonary blood
ow. Our results showed that there was an extremely good correlation between
Qp/Qs values obtained using VEC-MRI and those obtained using oximetry
with cardiac catheterization (Fig. 8.13) [26].
8.3.3
Evaluation of Regurgitation Diseases by

Using VEC-MRI
We attempted to quantify regurgitation at the atrioventricular, aortic, and pulmonary valves. Sechtem et al. showed that there was a correlation between the
regurgitant fraction obtained via VEC-MRI and that obtained via echocardiography, and a number of studies have followed this research [2729]. In cases of aortic
valve regurgitation (AR), the use of VEC-MRI to image the origin of the ascending
aorta and directly measure the anterograde ow and regurgitant ow of aortic blood
ow made it possible to calculate the regurgitant blood ow volume and regurgitant
fraction of AR (Fig. 8.14). In cases of mitral valve regurgitation (MR), it was
possible to calculate the regurgitant blood ow volume of MR from the difference
between the left-ventricular outow volume obtained by imaging the origin of the
ascending aorta by using VEC-MRI and the left-ventricular inow volume obtained
by imaging the mitral annular ring level by using VEC-MRI (Fig. 8.15). Our
investigation on pulmonary regurgitation after surgery for TOF in ten patients
showed that evaluation of regurgitant fraction by using VEC-MRI was nearly the
same as that by using echocardiography (Table 8.2). MRI allows not only the
quantitative measurement of regurgitant fraction but also the simultaneous measurement of right heart function. Thus, it is the most appropriate method for followup after CHD surgery [3032].
8.3.4
Evaluation of Valvular Stenosis by Using VEC-MRI
VEC-MRI can be used to evaluate stenosis of the aortic and pulmonary valves. Yap
et al. studied 20 adult patients with bicuspid aortic valves and found a good
correlation between ow velocity values obtained using VEC-MRI and those
193
Flow (ml/sec)
Velocity (cm/sec)
Stroke volume = 25.1ml
Time (msec)
Time (msec)
Regurgitant volume = 8.2 ml
Fig. 8.14 (a)(c) MR images of a 7-year-old patient with severe aortic regurgitation. (d) Time
velocity curve at the aortic valve in the patient. (e) The graph of aortic ow volume over time
illustrates the stroke volume in systole (red) and the regurgitant volume in diastole (blue), which is
calculated as the area bounded by the curve under the baseline in diastole. The regurgitant fraction,
which is dened as regurgitant volume divided by stroke volume, is 33 % (8.2 mL/25.1 mL), a
nding that indicates severe aortic regurgitation
a
c
400
Flow (ml/sec)
300
200
100
0
0
100
200
300
400
500
600
-100
-200
-300
-400
Time (msec)
Fig. 8.15 (a), (b) MR images of a 16-year-old patient with mitral regurgitation. Sagittal long-axis
cine gradient-echo MR images and four-chamber view. An abnormal ow jet due to mitral
regurgitation in systole (arrow). In this case, the severity of mitral regurgitation was evaluated
by measuring the ventricular volumes. The regurgitant fraction was calculated as 0.27
194
M. Sugimoto
Table 8.2 The regurgitation grade between echocardiography and VEC-MRI in the patients after
repair of TOF
Case 1
Case 2
Case 3
Case 4
Case 5
Case 6
Case 7
Case 8
Case 9
Case 10
Echo
VEC-MRI
PR
SV (mL)
PR-volume (mL)
Regurgitant fraction
volume/SV (%)
Slight
Slight
Moderate
Moderate
Moderate
Moderate
Moderate
Moderate
Severe
Severe
34.8
26.4
29.6
33.3
70.1
25.1
42.8
22.9
49.6
45.8
1.4
1.2
7.3
8.3
18.6
8.2
14.4
9.1
31.0
20.6
4
5
25
25
27
33
34
40
63
45
PR pulmonary valve regurgitation, SV stroke volume
obtained using transesophageal echocardiography [33]. When VEC-MRI is used in

pediatric patients with aortic valve stenosis, it is possible to obtain the same high
velocities in the narrowed segment as those obtained using echocardiography [28].
8.3.5
Quantication of Collateral Vascular Flow by

Using VEC-MRI
VEC-MRI is useful for quantifying collateral vascular ow and determining the

severity of stenosis in cases of coarctation of the aorta (CoA). In healthy patients,
blood ow volume at the distal descending aorta measured using VEC-MRI is
slightly lower than that measured at the proximal descending aorta. However, in
cases of CoA, blood ow volume at the distal descending aorta increases in
accordance with the severity of the stenosis, and collateral vascular ow volume
through the intercostal artery and other vessels can be quantitatively measured
[34, 35]. Furthermore, this method can be used to quantify collateral vascular ow
volume through arterio-pulmonary and arteriovenous shunts [15]. These results
provide important pre- and postoperative information about CHD patients.
8.3.6
Estimation of Pulmonary Arterial Pressure by

Using VEC-MRI
The gold standard for measuring pulmonary arterial pressure is cardiac catheterization, which is an invasive and quantitative method. However, because this
195
method is invasive, it is difcult to perform frequently. Laffon et al. studied

31 adult patients and showed that it is possible to estimate pulmonary arterial
pressure from the ow velocity of the main pulmonary artery by using VEC-MRI
[36]. Mousseaux et al. showed a good correlation of acceleration volume from the
owvolume curve obtained using VEC-MRI to pulmonary vascular resistance
[37]. We performed cardiac catheterization and VEC-MRI in 34 pediatric patients
with CHD (Table 8.1). They were divided into two groups according to the ratio
of pulmonary pressure (Pp) to systemic pressure (Ps), as measured by cardiac
catheterization, to give a low PAP group (Pp/Ps < 0.25, n 17) and a high PAP
group (Pp/Ps > 0.25, n 17) (Table 8.3). Using the timevelocity curve, we
calculated the acceleration time (AcT), dened as the time from the onset of
ow to the peak velocity (PV) in the main pulmonary artery, and the ejection
time (ET) (Fig. 8.16). Acceleration volume (AcV), dened as the volume ejected
during the AcT, and the maximal change in ow rate during ejection (MCFR),
dened as the maximal value of the ascending slope of the ow rate, were
measured using the timeow curve. A series of comparisons between the low
and high PAP groups in terms of the standardized parameters AcT, ET, AcT/ET,
PV, AcV, and MCFR are shown in Fig. 8.17. No signicant difference between
these groups was observed in terms of the AcT, ET, AcT/ET, PV, and AcV
parameters; however, a signicant difference was observed in the MCFR. Our
study on pediatric patients showed a good correlation between MCFR assessed
using VEC-MRI and pulmonary arterial systolic pressure assessed using cardiac
catheterization (Fig. 8.18) [26].
Table 8.3 Demographic and hemodynamic data of Table 8.1 patients
Age (year)
BSA (m2)
Gender (male)
sPAP (mmHg)
mPAP (mmHg)
dPAP (mmHg)
ABP (mmHg)
Qp/Qs
Pp/Ps
Rp (Um2)
Cardiac index
(l min1 m2)
Low PAP Pp/Ps < 0.25

(N 17)
High PAP Pp/Ps 0.25

(N 17)
P
value
6.5 4.1
0.9 0.4
10
20.5 3.4
13.0 3.1
9.8 3.8
106.1 13.4
1.35 0.54
0.20 0.04
1.01 0.46
5.16 1.13
2.7 3.8
0.5 0.2
7
39.3 14.2
25.2 8.6
20.4 16.5
87.5 12.0
1.58 1.05
0.47 0.02
2.45 1.35
4.88 1.28
ns
<0.05
ns
<0.01
<0.01
<0.05
ns
<0.05
<0.01
<0.01
ns
BSA body surface area, sPAP systolic PAP, mPAP mean PAP, dPAP diastolic PAP, Qp/Qs the
ratio of pulmonary to systemic blood ow, Pp/Ps the ratio of pulmonary to systemic blood
pressure, Rp pulmonary arterial vascular resistance
196
M. Sugimoto
100
ET
AcT
80
Velocity (cm/sec)
Fig. 8.16 (a) Denitions of

the acceleration time (AcT),
ejection time (ET), and peak
velocity (PV) in the velocity
curve. (b) Denitions of the
acceleration volume (AcV)
and the maximal change in
ow rate during ejection
(MCFR) in the ow curve
60
PV
40
20
0
0
200
-20
400
600
Time (msec)
250
200
Flow (ml/sec)
MCFR =
150
100
a
b
AcV
50
b
0
0
200
-50
8.4
8.4.1
400
600
Time (msec)
Other MRI Cardiac Function Tests

Delayed Contrast-Enhanced MRI
Delayed contrast-enhanced MRI requires imaging to commence at least 10 min

after the contrast medium is administered, and during this time, the contrast
medium is evenly distributed in the extracellular uid. The contrast effect for
myocardial infarction and brotic lesions in cardiomyopathy is thought to be due
to the distribution of gadolinium contrast medium in the expanded intercellular
stroma, which provides a relatively higher-intensity signal from a damaged myocardium than from a normal myocardium. Areas that have high-intensity signals in
delayed contrast-enhanced MRI often correspond to pathological infarct regions
and indicate myocardial necrosis in cases of acute myocardial infarction and
brosis in cases of old myocardial infarction. Since MRI has high spatial resolution,
it can be used to clearly render subendocardial infarction and right-ventricular
197
Fig. 8.17 Comparison of hemodynamic parameters between the low PAP and high PAP groups,
including standardized AcT, ET, AcT/ET, PV, AcV, and MCFR. Open rectangles represent the
low PAP group (Pp/Ps < 0.25, n 17) and shaded rectangles represent the high PAP group
(Pp/Ps 25, n 17). The bars represent the median and the 5th, 25th, 75th, and 95th percentiles
infarction. In the eld of pediatric cardiology, it has been reported as useful in the
evaluation of coronary lesions associated with Kawasaki disease (Fig. 8.19) [38].
Delayed contrast-enhanced MRI is extremely useful in the evaluation of myocardial brosis in cases of cardiomyopathy [39]. In most cases of acute myocardial
infarction due to CHD, delayed contrast is observed in the subendocardium, but in
cases of dilated cardiomyopathy, delayed contrast accompanying brosis is
observed in the mid-layer myocardium [40]. Hypertrophic cardiomyopathy
(HCM) patients are the same, with contrast medium observed in the mid layers of
the hypertrophic myocardium, in which case, the delayed contrast indicates myocardial brosis (Fig. 8.20) [41]. Studies indicate that compared to HCM patients
without brosis, HCM patients in whom brosis is detected by delayed contrastenhanced MRI develop ventricular tachycardia, which means that MRI can be used
to classify risk [42].
198
M. Sugimoto

between the MCFR
determined by VEC-MRI
and Pp/Ps determined by
cardiac catheterization
1.0
y = 13.336x - 0.121
R = 0.895, n = 34
(p< 0.001)
Pp/Ps
0.8
0.6
0.4
0.2
0.0
0
0.02
0.04
0.06
0.08
MCFR
Fig. 8.19 (a) Coronary magnetic resonance (CMR) angiography with RCA aneurysm (arrow). (b)
Contrast-enhanced CMR angiography, indicative of subendocardial inferior infarction in the same
patient (arrow). Images from Journal of Cardiovascular Magnetic Resonance (Mavrogeni
et al. [38])
Hartke et al. demonstrated that exposure to contrast should be delayed when

using MRI in patients with right-ventricular hypertrophy caused by high rightventricular pressure after CHD surgery [43]. This nding indicates the development
of myocardial brosis long after CHD surgery and suggests the possibility that
delayed contrast-enhanced MRI may be a useful source of information for the longterm follow-up of adult patients with CHD.
199
Fig. 8.20 MR images of a 13-year-old patient with hypertrophic cardiomyopathy. A bolus of

gadolinium is injected and followed through the heart in the fourth chamber and short axis. The
myocardial blush should be uniform. However, defects in myocardial perfusion will show up as
dark spots in the myocardial blush (arrows), which is typical of HCM images [38]
8.4.2
Tagging Cine MRI
MR tagging is a technique in which the noninvasive evaluation of myocardial wall

movements is possible by adding tags to the myocardium by using spatially selected
RF pulses. Analysis of the obtained images allows the detailed evaluation of
localized cardiac function by myocardial strain [44, 45]. Strain is indicated by the
deformation caused when force is applied to an object. In other words, myocardial
strain is indicated by contractile force (Fig. 8.21) [46]. One parallel lines or lattice
patterns in the images obtained using MR tagging is superimposed. These lines or
lattice patterns that accompany a systole deform or move, but immobile or only
slightly mobile areas of the myocardium remain as straight lines.
If heart failure advances, cardiac dilatation will advance, resulting in the loss of
myocardial contraction coordination and, in many cases, left bundle branch block.
Thus, a method for evaluating coordination between the ventricles is necessary.
Since MR tagging provides very good temporal and spatial analysis, a noninvasive
analysis of a three-dimensional localized strain in the ventricles can be performed
using tagging. In the recent years, tests are being conducted on combining delayed
contrast and strain evaluation to estimate sites of cardiomyopathy caused by
ischemia and infarction. In cases of CHD, MR tagging is being used to image
myocardial contractions in patients with a single-ventricle circulation and after
repair of transposition of the great arteries [47].
200
M. Sugimoto
Fig. 8.21 Myocardial tissue tagging. These SPAMM images are created by multiple
radiofrequency pulses, dividing the wall into cubes of magnetization. Tracking this movement
and distortion allows for assessment of wall motion and deformation (strain). MR images of a
1-year-old patient with mitral regurgitation. The left short-axis image is at end-diastole and the
right image is at end-systole
Conclusion
Pediatric cMRI testing could provide the same ndings as or superior ndings
to conventional contrast radiography and cardiac function tests. As this
method is noninvasive and does not expose patients to radiation, it is a
revolutionary development in the treatment of CHD patients who require
frequent observation of the progress of their disease, and it may become the
gold standard test in the future.
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Index
A
Abdominal compression, 117
Abnormal relaxation, 156
Absent pulmonary valve, 83
Accommodation of images, 67
Acute myocardial infarction, 196
Airway, 83
Ambiguous connection, 54
Ambiguus, 50
Amplatzer device, 5666
Amplatzer septal occluder (ASO), 18
Annulus, 3437
Anomalous origin of the left coronary
artery from the pulmonary artery
(ALCAPA), 80
Anterior leaet, 22
Ao distensibility (D), 168
Aortic valve regurgitation (AR), 192
Aortic valve stenosis, 150
Ao stiffness index, 168
Ao strain, 168
Arrhythmia, 185
Arterial characteristics, 128
Arterial function, 129135
Arterial stiffness, 167
ASD. See Atrial septal defect (ASD)
ASO. See Amplatzer septal occluder (ASO)
Asplenia syndrome, 13
Atrial septal defects (ASD), 118119,
144145, 164
Atrioventricular level, 50
Atrioventricular valve, 2140
development of, 2122
disease, 12
regurgitation, 13
Atrioventricular valvuloplasty, 6, 1214

Augmentation index (AI), 135, 168
B
Balloon angioplasty, 120
BAR. See Brachial artery reactivity (BAR)
Bicuspid aortic valves, 192
Billowing, 35
Black-blood protocol, 4748
Brachial artery reactivity (BAR), 166
Bright-blood protocol, 46
C
Cardiac output, 120, 189190
Carotid intima-media thickness, 167
Carperitide, 115
CARTO, 90
Catecholamine, 115
Catheter ablation, 90
Catheter closure of atrial septal defect, 4
Catheter intervention, 1820
Central venous pressure (CVP), 136
CFR. See Coronary ow reserve (CFR)
Characteristic impedance (Zc, Rc),
130131, 135
CHD. See Congenital heart
disease (CHD)
Chordae tendineae, 24
Cine MRI, 48, 181185
Coarctation of aorta, 72, 113
Collateral vascular ow, 194
Common atrioventricular valve, 3134
regurgitation, 13
Springer Japan 2015

DOI 10.1007/978-4-431-54355-8
205
206
Common-inlet atrioventricular connection,
3234
Compliance, 109
Compression, 86
Concordant connection, 54
Conductance catheters, 104
Congenital heart disease (CHD), 44, 98
Conus, 51
Coronary artery, 17
Coronary ow reserve (CFR), 169
Corrected transposition of great arteries, 164
Crista supraventricularis, 57
Cropping, 911
CVP. See Central venous pressure (CVP)
D
Deceleration time (DT), 154
Delayed contrast-enhanced MRI, 196199
Device closure, 119
Dextrocardia, 50
Diastolic wave, 155
Dilated cardiomyopathy, 169
Discordant connection, 54
d-loop, 53
Doppler, 110
Double orice mitral valve, 2628
Double-outlet right ventricle, 6
dP/dt, 147, 163
D-transposition, 54
3D volume data, 7
3D volume isotropic T2-weighted
acquisition: VISTA, 47
3D volumetric black-blood angiography, 47
E
Early diastolic lling wave, 153
Ebsteins anomaly, 29
ECG gating, 178
ECG-synchronized multiple slices, 9
EDPVR. See End-diastolic pressurevolume
relationship (EDPVR)
Ees. See End-systolic elastance (Ees)
Effective regurgitant orice area (EROA), 39
Efciency, 107
Ejection fraction (EF), 100, 145
Elastic property, 132133
End-diastolic pressurevolume relationship
(EDPVR), 102
End-diastolic relationships, 109
End-systolic elastance (Ees), 106
End-systolic wall stress (ESWS), 146
Index
Eparterial bronchus, 50
EROA. See Effective regurgitant orice
area (EROA)
Erosion, 19
ESWS. See End-systolic wall stress (ESWS)
F
FAC. See Fractional area change (FAC)
Fibrosis, 196
Filling, 99
Flow-mediated dilation (FMD), 166
FMD. See Flow-mediated dilation (FMD)
Fontan, 89, 120
Fractional area change (FAC), 161
G
Glutaraldehyde-treated autopericardium, 13
Grade I diastolic dysfunction, 156
Grade II diastolic dysfunction, 156
Gradient echo (GRE), 179180
Guide, 4
H
HCM. See Hypertrophic cardiomyopathy
(HCM)
Heart failure, 128, 199
Hepatic vein ow velocities, 166
Heterotaxy syndrome, 32, 54, 77
Hyparterial bronchus, 50
Hypertrophic cardiomyopathy (HCM),
152, 197
Hypoplastic left heart syndrome, 3031
I
Inodilator, 112
Input impedance, 129130
Intracardiac route, 4
Intracardiac structure, 44
Intraoperative ndings, 13
Inversus, 50
Isolated mitral valve cleft, 26
Isovolumic contraction, 99
Isovolumic relaxation, 108
IVA, 164
IVC occlusion, 103
J
Juxtaductal pulmonary artery coarctation, 74
Index
K
Kawasaki disease, 168, 169, 197
Korteweg-Moens, 133
L
Late diastolic lling wave, 153
Left atrial volume, 110
Left superior caval vein (LSVC), 83
Left-ventricular end-diastolic volume, 183
Left-ventricular end-systolic volume, 183
Left ventricular outow tract stenosis, 1112
Levocardia, 50
l-loop, 53
L-malposition, 54
LSVC. See Left superior caval vein (LSVC)
LV lling pressure, 158
M
Magnetic resonance imaging (MRI), 44, 105
Magnitude images, 186
Maximal change in ow rate during ejection
(MCFR), 195
Maximum-intensity projection (MIP), 80
MCFR. See Maximal change in ow
rate during ejection (MCFR)
MD-CT. See Multidetector computed
tomography (MD-CT)
Mean velocity of circumferential ber
shortening, 146
Medial papillary, 25
Membranous ventricular septum (MVS), 60
Mesocardia, 50
Metabolic energy, 107
Milrinone, 112
MIP. See Maximum-intensity projection (MIP)
Mitral inow velocity wave, 153154
Mitral valve (MV), 2224
Mitral valve regurgitation (MR), 192
Mitral valvuloplasty, 6
Mixing chamber, 87
Monitoring, 4
MPI. See Myocardial performance index (MPI)
MRI. See Magnetic resonance imaging (MRI)
MSW, 106
Multidetector computed tomography
(MD-CT), 71, 177
Multiplanar reformatted (MPR), 80
Mustard repair, 164
MVS. See Membranous ventricular
septum (MVS)
Myocardial acceleration during ICT, 150
207
Myocardial mass, 183
Myocardial performance index (MPI), 148, 163
N
Natriuretic peptide, 115
Noncompaction, 59
Nonconuent pulmonary arteries, 75
Noninvasive estimation, 100
O
Off-line image analysis, 4
P
Paradoxical septal motion, 145
PC images, 186
Percutaneous closure of atrial septal
defect, 46, 18, 20
Pericardium, 6, 7
Peripheral arterial resistance, 131
Peripheral pulmonary stenosis, 119
Phase contrast (PC), 180
Position of great vessels, 50
Posterior leaet, 22
Preoperative surgical planning, 67
Pressure-area relationships, 105
Pressure-ow, 130
Pressure guidewire, 104
Pressurevolume area (PVA), 106
Pressurevolume relationships, 97123
Prolapse, 3839
Prospective ECG gating, 178179
Pseudonormalization, 154
Pulmonary arterial pressure, 194196
Pulmonary artery sling, 83
Pulmonary capillary wedge pressure, 158
Pulmonary hypertension, 163, 166
Pulmonary perfusion scintigraphy, 191
Pulmonary regurgitation, 192
Pulmonary to systemic blood ow ratio
(Qp/Qs), 191192
Pulmonary vascular resistance
(PVR), 168, 195
Pulmonary veins, 14
Pulmonary venous ow velocity wave, 155
Pulmonic RV, 51
Pulse wave velocity (PWV), 133135, 167
PVA. See Pressurevolume area (PVA)
PVR. See Pulmonary vascular resistance
(PVR)
PWV. See Pulse wave velocity (PWV)
208
R
RAI. See Right atrial isomerism (RAI)
Rastelli classication, 31
Rate-corrected mean, 146
Ratio of E wave velocity to lateral mitral
annular early diastolic velocity, 158
Real-time three-dimensional
(3D) echocardiography, 320
Reection index, 132
Region of interest (ROI), 185196
Replicas, 92
Respiratory gating, 179
Restrictive lling, 156
Retrospective ECG gating, 179
Reversal wave with atrial contraction, 155
Rheumatic mitral valve disease, 28
Right atrial isomerism (RAI), 32
ROI. See Region of interest (ROI)
S
Saddle shape, 35
Segmental approach, 49
Senning, 164
Septal leaet, 25
Shaher classication, 17
Shortening fraction (SF), 144
Solitus, 50
Spatial resolution, 72
Steady-state free precession (SSFP), 180
Stent, 122
Stereolithography, 67, 92
Stiffness, 100, 109
Strain, 150, 164, 199
Strain rate (SR), 150, 164
Strut chordae, 24
Surgeons view, 12
Surgery, 67
Systemic RV, 51
Systolic waves, 155
T
TAC. See Total arterial compliance (TAC)
Tagging cine MRI, 199200
TAPSE. See Tricuspid annular plane
systolic excursion (TAPSE)
TAPVC. See Total anomalous pulmonary
venous connection (TAPVC)
TDI. See Tissue Doppler imaging (TDI)
Tethering, 3839
Tetralogy of Fallot (TOF), 134, 163, 164
Thermodilution, 190
Index
3D display, 518
3-dimensional echocardiograms (3DE),
152, 165
Three-dimensional reconstruction, 5666
Timeow curve, 186
Timenet ow curve, 186
Time-varying elastance model, 101
Timevelocity curve, 186
Tissue Doppler imaging (TDI), 110, 149,
157, 163
TOF. See Tetralogy of Fallot (TOF)
Total anomalous pulmonary venous
connection (TAPVC), 77
Total arterial compliance (TAC), 132133
Trabecula septomarginalis, 25, 57
Transpericardial 3D echocardiography, 6
Transposition of great arteries, 6, 1718
Tricuspid annular plane systolic
excursion (TAPSE), 162
Tricuspid valve, 2426
Truncus arteriosus, 16
TV dysplasia, 29
V
Valsalva, 89
Valvular stenosis, 192194
Vascular rings, 72
Veins, 128
Velocity-encoded cine MRI (VEC-MRI),
185196
Vena contracta width, 39
Venous function, 136137
Venous return curves, 136
Ventricular-arterial coupling, 112, 113
Ventricular-arterial stiffening, 123
Ventricular interaction, 109
Ventricular loop, 50
Ventricular septal defect (VSD), 60, 89, 161
Ventricular stiffening, 116
Ventricularventricular interaction, 119
Ventricular volume, 184
Ventriculoarterial level, 50
Virtual cardiotomy, 67
Visceroatrial situs, 50, 87
Volume data collection, 79
Volume-rendered (VR), 80
VSD. See Ventricular septal defect (VSD)
W
Wave intensity analysis, 137138
Whole-heart protocol, 4446

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Hideaki Senzaki Satoshi Yasukochi

Congenital Heart Disease

Anatomical or morphological abnormalities in congenital heart disease (CHD) are

friend, Dr. Satoshi Yasukochi, and to invite world-leading Japanese pediatric

Real-Time Three-Dimensional (3D) Echocardiography

Assessment of Intracardiac Anatomy by Magnetic

Assessment of Extracardiac and Intracardiac Anatomy

Morphological Assessment of Congenital Heart Disease

Functional Assessment of Congenital Heart Disease

Assessment of Ventricular Function Using the Pressure-Volume

Assessment of Vascular Function by Using Cardiac

Assessment of Ventricular-Vascular Function

Assessment of Hemodynamics by Magnetic

Morphological Assessment of Congenital

Abstract How to use real-time three-dimensional (3D) echocardiography for

K. Takigiku, MD, PhD (*)

1 Real-Time Three-Dimensional (3D) Echocardiography for Diagnosis . . .

3D Display of the Intracardiac Structure

Roll as a tool for detailed diagnosis and a guide for surgery.

Although transthoracic 3D echocardiography has a certain level of diagnostic

1 Real-Time Three-Dimensional (3D) Echocardiography for Diagnosis . . .

Volume Data Collection

After adjusting the 2D images, start to work on the 3D image collection. It is

Fig. 1.3 Type of the ventricular septal defects

defect exists. It might be better to understand the anatomical knowledge and

1 Real-Time Three-Dimensional (3D) Echocardiography for Diagnosis . . .

1 Real-Time Three-Dimensional (3D) Echocardiography for Diagnosis . . .

Postoperative Left Ventricular Outow Tract Stenosis

In children with atrioventricular valve disease, it is also very important for

Fig. 1.9 Transpericardial 2D echocardiography in congenital mitral regurgitation. Ao aorta, LA

1 Real-Time Three-Dimensional (3D) Echocardiography for Diagnosis . . .

Fig. 1.10 Transpericardial 3D echocardiography in congenital mitral regurgitation

Pulmonary Venous Obstruction

Figure 1.13 shows the transpericardial 2D echographic image of a case that

1 Real-Time Three-Dimensional (3D) Echocardiography for Diagnosis . . .

This is the transpericardial 3D echocardiographic image of truncus arteriosus.

1 Real-Time Three-Dimensional (3D) Echocardiography for Diagnosis . . .

Fig. 1.17 Transpericardial 3D echocardiography of coronary arteries in transposition of the great

Coronary Artery in Transposition of the Great Arteries

To diagnose for the morphology of coronary artery in transposition of the great

Transposition of the Great Arteries After Conotruncal Switch

Decision of Indication of Catheter Intervention

1 Real-Time Three-Dimensional (3D) Echocardiography for Diagnosis . . .

in the image quality. In a common ASO, transesophageal 2D echocardiography is

Assessment of Atrioventricular Valve

Anatomical Aspects of the Atrioventricular Valve

atrioventricular canal starting with the formation of endocardial cushions by an

Normal Anatomy of the Mitral Valve

2 Assessment of Atrioventricular Valve Anatomy and Function in Congenital. . .

Normal Anatomy of the Tricuspid Valve

2 Assessment of Atrioventricular Valve Anatomy and Function in Congenital. . .

Anomalies of the Atrioventricular Valve

Mitral Valve Disease

2 Assessment of Atrioventricular Valve Anatomy and Function in Congenital. . .

Table 2.1 Congenital mitral valve anomalies

2. Anomalies of the commissure

4. Anomalies of the papillary muscle

5. The supramitral ring

Cleft mitral valve

an isolated form and is usually associated with an atrioventricular septal defect,

2 Assessment of Atrioventricular Valve Anatomy and Function in Congenital. . .

Tricuspid Valve Disease