Ann Thorac Surg CASE REPORT ACAR ET AL 731

2006;82:7313 3-D ASSESSMENT OF EBSTEINS MALFORMATION

Ebsteins Anomaly Assessed by

Real-Time 3-D Echocardiography
Philippe Acar, MD, PhD, Sylvia Abadir, MD,
Daniel Roux, MD, Assaad Taktak, MD,
Yves Dulac, MD, Yves Glock, MD, PhD, and
Gerard Fournial, MD
Medical and Surgical Unit of Pediatric Cardiology, Toulouse,
France

The outcome of patients with Ebsteins malformation

depends mainly on the severity of the tricuspid valve
malformation. Accurate description of the tricuspid anat-
omy by two-dimensional echocardiography remains dif-
ficult. We applied real-time three-dimensional echocardi-
ography to 3 patients with Ebsteins anomaly.
Preoperative and postoperative descriptions of the tricus-
pid valve were obtained from views taken inside the
right ventricle. Surface of the leaflets as well as the

commissures were obtained by three-dimensional echo-
cardiography. Real time three-dimensional echocardiog-
raphy is a promising tool, providing new views that will
help to evaluate the ability and efficiency of surgical
valve repair in patient with Ebsteins malformation.
(Ann Thorac Surg 2006;82:7313)
2006 by The Society of Thoracic Surgeons
FEATURE ARTICLES
Fig 1. Three-dimensional echocardiography (patient 1). The tricus-
pid valve was viewed from the right ventricle. The three leaflets
with the commissures were visualized from below. Only the septal
(S) leaflet had abnormal attachment to the ventricular septum. The
anterior (A) and posterior (P) leaflets had normal coaptation.

E bsteins malformation is a rare congenital heart dis-

ease [1]. Patients with Ebsteins anomaly have a
wide spectrum of anatomic abnormalities. The predictors
of outcome depend on the severity of the tricuspid valve
malformation [2 4]. Precise description of the tricuspid
anatomy by conventional two-dimensional echocardiog-
raphy remains difficult [5].
Three-dimensional (3-D) echocardiography offers a di-
rect view to evaluate the leaflet surface [6]. Very few data
were reported on 3-D reconstruction of the tricuspid
valve from transoesophageal acquisition [7]. The recent
introduction of the transthoracic 3-D matrix array probe
allows real-time 3-D acquisition and visualization [8].
Our aim was to obtain preoperative and postoperative
description of the tricuspid valve using 3-D
echocardiography.
Real-time, 3-D echocardiographic ultrasound was per-
formed with the Sonos 7500 (Philips) using the cardiac
matrix probe (2 to 4 MHz). The system scanned a 60
60 3-D pyramid of data. Two orthogonal reference plans
were used to localize cardiac structures in the volume.
Navigation by cropping inside the volume was per-
formed to obtain 3-D views of the tricuspid valve. Live
3-D images were visualized directly on the ultrasound
system.
Fig 2. Three-dimensional (3-D) echocardiography (patient 2). The
tricuspid valve was viewed from below. Because of a restrictive mo-
Accepted for publication Sept 6, 2005. tion and reduced functional surface, the posterior leaflet did not co-
Address correspondence to Dr Acar, Unit de Cardiologie Pdiatrique, apt with the septal (S) leaflet. The posterior (P) commissure dis-
Hpital des Enfants, 330 Avenue de Grande-Bretagne, B-P 3119, Toulouse played a huge hole compared with the continent anterior (A) and
Cedex 3, 31026 France; e-mail: acar.p@chu-toulouse.fr. septal commissures. The surgeons confirmed the 3-D findings.

2006 by The Society of Thoracic Surgeons 0003-4975/06/$32.00

Published by Elsevier Inc doi:10.1016/j.athoracsur.2005.09.012
 732 CASE REPORT ACAR ET AL
3-D ASSESSMENT OF EBSTEINS MALFORMATION
Fig 3. Postoperative three-dimensional (3-D) echocardiography (patient 3). The tricuspid (T) and mitral (M) valves were viewed from below.
(A) Early diastole (mitral and tricuspid valves are open). (B) End diastole (mitral valve is closed and tricuspid valve is open). (C) Systole (mi-
tral and tricuspid valves are closed). The surgically enlarged anterior leaflet was the only mobile leaflet. The tricuspid valve repair created a
functional monocusp with trivial regurgitation without stenosis.
Case Reports
FEATURE ARTICLES
Patient 1
A 6-year-old girl underwent two-dimensional echocardi-
ography because of a systolic murmur. Tricuspid regur-
gitation was mild. Valve anatomy is described in Figure 1.
Because the patient was asymptomatic and the regurgi-
tation was mild, surgery was not indicated and an annual
echographic follow-up was scheduled.
Patient 2
A 15-year-old boy was determined to be in functional
class II of the New York Heart Association. Cardiac
auscultation found a 3/6 holosystolic murmur. Chest
roentgenogram showed increased cardiothoracic index to
0.65. Two-dimensional echocardiography found severe
tricuspid regurgitation with septal attachment. Valve
anatomy is described in Figure 2. The surgeon confirmed
that the posterior leaflet was deficient and did not coapte
with the septal and anterior leaflets of the tricuspid valve.
The tricuspid valve was replaced with a Carpentier-
Edwards pericardial bioprosthesis.
Patient 3
A 10-year-old boy underwent surgical repair of Ebsteins
malformation. Severe tricuspid regurgitation was cor-
rected by De Vega annuloplasty and enlargement of the
anterior leaflet with pericardial patch. Two years after the
surgery, the patient was asymptomatic with trivial tricus-
pid regurgitation. Anatomy and function of the repaired
tricuspid valve are described in Figure 3.
Comment
We report the first description of Ebsteins anomaly
using real-time 3-D echocardiography. Ebsteins anom-
aly of the tricuspid valve consists of various degrees of
inferior displacement of the proximal attachments of the
septal leaflet [1 4]. Because the apical four-chamber
plane provides good visualization of the septal leaflet,
two-dimensional echocardiography allows the initial di-
Ann Thorac Surg
2006;82:7313
agnosis of Ebsteins anomaly [5]; however downward
displacement could involve the anterior and posterior
leaflets of the tricuspid valve. Precise description of the
tricuspid anatomy could be difficult from only the two-
dimensional planes. The surface of the tricuspid leaflets
as well as the commissures could be displayed by 3-D
echocardiography. The previous 3-D system was cum-
bersome due to the transoesophageal approach and the
time needed for reconstruction [7]. Introduction of the
transthoracic 3-D matrix array probe allowed the use of
real-time 3-D echocardiography in routine [8]. Three-
dimensional echocardiography allows the cardiologist
and the surgeon to evaluate the ability and efficiency of
surgical valve repair.
In conclusion, real-time 3-D echocardiography is a
feasible method in addition to conventional two-
dimensional echocardiography to evaluate tricuspid
valve anatomy and function in patients with Ebsteins
malformation.
References
1. Chen JM, Mosca RS, Altmann K, et al. Early and medium-
term results for repair of Ebsteins anomaly. J Thorac Cardio-
vasc Surg 2004;127:990 8.
2. Celermajer DS, Bull C, Till JA, et al. Ebsteins anomaly:
presentation and outcome from fetus to adult. J Am Coll
Cardiol 1994;23:170 6.
3. Chauvaud S, Berrebi A, dAttellis N, Mousseaux E, Hernigou
A, Carpentier A. Ebsteins anomaly: repair based on func-
tional analysis. Eur J Cardiothorac Surg 2003;23:52531.
4. Marianeschi SM, McElhinney DB, Reddy M, Silverman H,
Hanley FL. Alternative approach to the repair of Ebsteins
malformation: intracardiac repair with ventricular unloading.
Ann Thorac Surg 1998;66:1546 50.
5. Gussenhoven EJ, Stewart PA, Becker AE, et al. Offsetting of
the septal tricuspid leaflet in normal hearts and in hearts with
Ebsteins anomaly. Anatomic and echographic correlation.
Am J Cardiol 1984;54:172 6.
6. Acar P, Laskari C, Rhodes J, Pandian NG, Warner K, Marx G.
Determinants of mitral regurgitation after atrioventricular
septal defect surgery: a three-dimensional echocardiographic
study. Am J Cardiol 1999;83:7459.
Ann Thorac Surg
2006;82:7335
7. Ahmed S, Nanda NC, Nekkanti R, Pacifico AD. Transesoph-
ageal three-dimensional echocardiographic demonstration of
Ebsteins anomaly. Echocardiography 2003;20:3057.
8. Acar P, Dulac Y, Taktak A, Abadir S. Real time three-
dimensional fetal echocardiography using cardiac matrix
probe. Prenatal Diagn 2005;25:370 5.
Valvular Reoperations by Left
Thoracotomy in Patients With
Pectus Excavatum
Scott A. LeMaire, MD, Roderick G. MacArthur, MD,
and Joseph S. Coselli, MD
Cardiovascular Surgery Service, Texas Heart Institute at St.
Lukes Episcopal Hospital and the Michael E. DeBakey
Department of Surgery, Division of Cardiothoracic Surgery,
Baylor College of Medicine, Houston, Texas
Several alternative approaches to the aortic and mitral
valves have been reported recently. We describe a left
anterior thoracotomy approach for valvular reoperations
in 3 patients with Marfan syndrome and severe pectus
excavatum.
(Ann Thorac Surg 2006;82:7335)
2006 by The Society of Thoracic Surgeons
I n the attempt to develop less invasive approaches for
operations on the heart and great vessels, several
alternative exposures have been reported recently, in-
cluding limited upper median sternotomy and right
parasternal minithoracotomy. Left thoracotomy is infre-
quently used for cardiac operations; this approach is
usually used for myocardial revascularization and is only
rarely used in valve replacement procedures [1 8]. The
purpose of this report is to describe left anterior thora-
cotomy as a valuable approach to valvular reoperations
in a unique patient population. In 3 patients with Marfan
syndrome (MFS) and severe pectus excavatum, left tho-
racotomy avoided high-risk repeat sternotomy while
providing excellent exposure for valve replacement.
Case Reports
A review of our patient database and records for this
report, with a waiver of consent, was classified as exempt
from formal review by the Institutional Review Board for
Baylor College of Medicine and Affiliated Hospitals.
Patient 1
An 18-year-old man with MFS and severe pectus exca-
vatum presented with severe aortic valvular insufficiency
5 years after homograft aortic root replacement. The
patient had minimal symptoms attributable to his aortic
valve disease, but surgery was recommended because he
had progressive left ventricular enlargement (left ventric-
ular end-diastolic dimension, 77 mm) and dysfunction
Accepted for publication Oct 17, 2005.
Address correspondence to Dr LeMaire, One Baylor Plaza, BCM 390,
Houston, TX 77030; e-mail: slemaire@bcm.tmc.edu.
2006 by The Society of Thoracic Surgeons
Published by Elsevier Inc
CASE REPORT LEMAIRE ET AL 733
VALVE REOPERATIONS BY LEFT THORACOTOMY
Fig 1. A computed tomographic scan shows severe pectus excavatum
and adherence of the right atrium to the posterior table of the
sternum.
FEATURE ARTICLES
(ejection fraction, 45%). A preoperative computed tomo-
graphic scan of the chest revealed dense adherence of the
right atrium and ventricle to the posterior table of the
sternum, displacement of mediastinal structures into the
left chest, and calcification of the previously placed
homograft (Fig 1).
Given this anatomy, a left thoracotomy approach was
selected to avoid high-risk repeat median sternotomy.
General anesthesia with a single-lumen endotracheal
tube was established, and the patient was placed supine
with the left chest elevated approximately 30 degrees.
The thorax was entered through the fifth intercostal
space, and the left internal thoracic artery was divided.
Pericardial retraction sutures held the left lung outside of
the operative field. Exposure of the ascending aorta and
aortic root was excellent (Fig 2), and cardiopulmonary
bypass was established after left common femoral arte-
rial and venous cannulation. A left atrial vent was placed
through the left inferior pulmonary vein. The distal
ascending aorta was clamped, and the heart was arrested
with antegrade cardioplegia. The aortic root was replaced
with a 23-mm St. Jude composite valve graft (St. Jude
Medical, Inc, St. Paul, MN). The left main and right
coronary buttons were directly reimplanted. The thora-
cotomy incision was closed in standard fashion.
Patient 2
A 17-year-old Marfan patient was referred to our insti-
tution with symptomatic, severe mitral insufficiency 6
years after having his aortic root replaced with a 21-mm
St. Jude composite valve graft. This patient had severe
pectus excavatum with displacement of the heart into the
left hemithorax. Therefore, a left anterior thoracotomy
was used. The left common femoral vein and artery were
cannulated for cardiopulmonary bypass. A cannula was
placed in the superior vena cava, and a left ventricular
sump was placed through the right superior pulmonary
vein. The left atrium was very large and facilitated a
standard left atriotomy approach to the mitral valve. A
0003-4975/06/$32.00
doi:10.1016/j.athoracsur.2005.10.012