Congenital Heart Defects

Learning Folder

By

Nichola Pope
ACHD Transition Nurse
(Adult Congenital Heart Disease)

(Some diagrams used by kind permission of Dr S Thorne and Dr P Clift)

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Contents: Page Number

Introduction to Congenital heart disease 3

Atrial septal defect (ASD) 4
Patent Foramen Ovale (PFO) 5
Ventricular septal defect (VSD) 6
Coarctation of the Aorta 7
Bicuspid aortic valve (BAV) 8
Aortic stenosis (AS) 9
Pulmonary stenosis (PS) 10
Tetralogy of Fallot (TOF) 11
Pulmonary atresia and VSD 12
Truncus arteriosus 13
Transposition of the Great Arteries (TGA) 14 - 15
Congenitally corrected TGA (ccTGA) 16
Ebsteins anomaly 17
Single ventricle defects and Fontan circulation 18-26
Caring for patients with a Fontan circulation 27-29
Caring for sick cyanotic ACHD patient 30
Eisenmengers 31
Contraception and pregnancy 32
Endocarditis 33
Glossary 34-35

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Congenital heart defects.

Congenital heart disease (CHD) is where there is a cardiovascular malformation that is

present from birth. Approximately 3 in a 100 live born infants will have some form of CHD
from simple defects to complex ones. Due to the success of Paediatric cardiology and
surgery over the past 25 years, there is a marked increase in the number of these patients,
especially those with complex defects that are surviving into adulthood. More than 95% of
significant congenital heart conditions can be corrected or alleviated by surgical intervention.

CHD can affect the interior walls of the heart, the valves or the blood vessels going to or from
the heart and can change the normal flow of blood through the heart.

The complexity of some of these defects means that the patients have surgically altered
physiology and anatomy and when complications occur, they can decompensate very quickly.
This poses a real challenge and requires expert cardiology care.

There are already more adults than children with congenital heart disease in the UK. It is
predicted that there will be a 50% increase in the numbers of adult patients with complex
congenital heart disease in the next 10 years.

Complex congenital heart defects account for 10% of the adult congenital patients but
they account for 80% of acute admissions.

There will therefore be an increase in the numbers of adult congenital heart disease patients
presenting as emergencies in the future.

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Atrial Septal Defect

Types of ASD;

Secundum ASD – most common and may be device closable, if large will need surgical
closure.
Primary ASD – ( part of AVSD) needs a surgical repair, usually closed in childhood.
Sinus venosus ASD – needs a surgical repair.

An Atrial septal defect (ASD) is where there is hole in the membrane between the left and
right atria of the heart and these can be multiple. Blood usually shunts between the atria
going from left to right, so oxygenated blood mixes with deoxygenated blood, thus the patient
will be pink in the absence of other lesions.

Depending upon the size of the hole these can either be closed with a device during cardiac
catheterisation or if larger may need to be closed surgically via a sternotomy. If
haemodynamically insignificant they are left alone.

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Patent Foramen Ovale (PFO) is where the foramen ovale remains open after birth, thereby
allowing a communication between the atria. The foramen ovale is present during foetal
circulation and should normally close a few hours after birth. About 20% of the population
have a PFO without any problems. Sometimes micro-emboli can pass through the PFO
resulting in a TIA / stroke, if this occurs they can be closed with a device, much like the small
ASD’s.
These can be discovered later in life and depending on size may be left or closed as
described above.

Diagram by Dr S Thorne - PFO

Fetal Circulation
In utero the baby’s circulating volume does not all need to go to the lungs for oxygenation as
this is obtained from the mother’s blood. Enough needs to go to the lungs to provide oxygen
and nutrients for growth and development only. Babies therefore have a foreman ovale to
allow blood to cross over from right to left as the pressure in the right side is higher than the
left in the foetus. Babies also have a ductus arteriosus, which is a duct between the
pulmonary artery (PA), as the PA is muscular and non-compliant this means that the
pulmonary vascular resistance in utero is high which is needed to divert the oxygenated blood
from the umbilical vein to bypass the lungs and cross over into the aorta through the ductus
arteriosus.
At birth the newborn baby breaths for the first time, inflating the lungs and blood is drawn into
the pulmonary circulation fully supplying the lungs for the first time. This increase in
pulmonary venous return increases the left atrial pressure and the foramen ovale closes. As
the flow through the ductus arteriosus reverses it stimulates the contraction and closure of the
duct, this occurs over a few days and pulmonary and systemic circulations are then
established.

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Ventricular Septal Defect (VSD)

A ventricular septal defect (VSD) is where there is a hole in the muscle wall that separates the
right and left ventricles of the heart. It is a common form of congenital heart defect,
accounting for 21% of all cases. They can be single or multiple and occur in different parts of
the ventricular septum. As the pressure is greater in the left ventricle and the resistance in
the lungs is significantly lower than in the body tissues, oxygenated blood from the left
ventricle is shunted across the hole into the right ventricle.

Small holes usually close spontaneously in the first couple of years of life. Large holes nearly
always require surgical closure in the first year of life. VSDs may be present with other heart
defects. VSDs can either be closed by suturing or patching, depending on the size of the hole.

In adult patients with a large VSD the left to right shunting of blood through the VSD results in
increased pulmonary blood flow resulting in increased pulmonary pressure leading to
increased RV pressure. If left untreated, this can cause irreversible changes in the pulmonary
circulation and there is a shunt reversal with a right to left shunt through the VSD so that
deoxygenated blood mixes with oxygenated blood and is circulated round the body and the
patient becomes blue. This is known as Eisenmenger Syndrome

Nowadays VSD repair is normally a low risk procedure. However patients who had some of
the earlier surgical treatments and procedures carried out may have complications later in life.
These can include developing ventricular dysfunction, aortic regurgitation, arrhythmias and
heart block. It is therefore important that they are followed up periodically.

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Coarctation of the Aorta

Coarctation of the Aorta is a narrowing in the aorta, usually on the descending aorta where
the subclavian artery exits the aorta on its way to the upper body, usually at the site of the
Ductus Arteriosus.

Up to 80% of cases the aortic valve, through which blood enters the aorta from the left
ventricle is abnormal, with only two leaflets rather than the usual three (Bicuspid Aortic Valve).

The obstruction to the blood flow resulting from the coarctation results in high blood pressure
in the left ventricle. While the blood pressure in the upper body becomes high, the blood
pressure in the lower body is low due to reduced blood flow through the aorta.

The majority of significant Coarctations are picked up in childhood, however some are found
in adults with difficult in managing hypertension.

Surgery may be required to treat this in infancy and an end to end anastomosis may be
needed. In adults, other ways of treating it are with the insertion of stents, during cardiac
catheterisation, to widen the affected part of the aorta. If the aortic valve is deformed it may
be repaired.

Blood pressure should always be taken on the right arm as any intervention or surgery would
involve the vessels on the left so you get a more accurate reading on the right. These
patients can be more at risk of developing hypertension and should have a cardiac MRI every
2 years to check for any re narrowing or aneurysm formation at the site of repair. They
should also be advised not to lift heavy weights as the increase in thoracic pressure that this
causes, can result in tearing of blood vessels. They can also be prone to premature
atherosclerosis. Long term complications in repaired coarctation include re coarctation and
aneurysm formation

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Bicuspid Aortic Valve (BAV)

Bicuspid Aortic Valve

(BAV) is where instead of
the normal 3 leaflets, there
are only 2. This can
happen in isolation with an
otherwise structurally
normal heart, or it can
occur along with other
cardiac defects.

Isolated BAV is the most

common congenital heart
defect and can cause no
effects on heart function. If
the valve does not open
normally it can result in
aortic valve stenosis.

Difficulties can occur later

in life, the valve opening
can become calcified
causing narrowing or it
may not open or close
completely resulting in the
valve being leaky.
Occasionally the
ascending aorta can
become dilated which can
result in an aneurysm
forming and possible
dissection.
If the aortic valve becomes
severely narrowed or leaky it may be necessary to replace the valve.

There is a long term risk of aortic stenosis due to calcification with or without regurgitation

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Aortic Stenosis

Aortic Valve Stenosis is where the outflow tract from the heart into the aorta is obstructed, the
most common type being valvular stenosis where the aortic valve itself is narrowed; this may
be due to a BAV. Occasionally the obstruction will be caused by a narrowing either above
(supravalvular) or below (subvalvular) the valve.

This obstruction of the aorta means that the left ventricle has to work harder to move blood
resulting in its walls getting thicker (hypertrophy). Aortic stenosis is normally progressive with
the obstruction getting worse and so leading to left ventricular failure. Those patients with
moderate to severe obstruction are at risk of sudden death with exercise.

Where there is aortic regurgitation a valve replacement will be required. If this has been done
when the patient was a child they may have had the Ross repair, when the aortic valve is
replaced with the patient’s own pulmonary valve and using a human donated valve to replace
the pulmonary valve. If the aortic valve is replaced later in life a mechanical valve, which can
last for 20 plus years, or a human donated valve or animal valve is used which can last for 10-
15 years and will need to be replaced. Patients with mechanical valves will need to be on
warfarin for life.

With supravalvular stenosis the narrowing above the aortic valve is removed and remaining
parts of the vessel are stitched back together. In subvalvular stenosis the obstructing tissue
is removed by going through the aortic valve, this excessive muscle tissue can grow back
again requiring future treatment.

Aortic stenosis (AS) can be progressive so all cases should be followed up regularly.
Symptoms of severe AS include angina, laboured breathing in response to exertion or
fainting.

Patients who have left ventricular hypertrophy or arrhythmias should only do moderate levels
of isotonic exercise. Patients with severe AS should not do athletics or intensive exercise due
to the risk of fainting (Stokes Adams attack) or sudden death.

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Pulmonary Stenosis (PS)

This is one of the commonest congenital heart defects. It is where the pulmonary valve and
/or the outflow tract from the RV into the pulmonary artery (PA) is narrowed.
Valvular pulmonary stenosis is where the leaflets of the valve are abnormal, often being
thickened and not opening fully. This causes a narrowing and sometimes a leak, meaning
that blood flows back into the RV.
Supravalvular stenosis is where the narrowing is above the pulmonary valve (PV).
Subvalvular / infundibular stenosis is where the narrowing is at the part of the RV that
leads into the pulmonary valve.

The narrowing can cause the RV to work harder in order to pump the blood into the
pulmonary artery, so increasing the blood pressure in the RV. This can lead to hypertrophy
(thickening) of the right ventricular walls.

Mild PS may require no treatment just regular check ups. More severe cases may need
intervention such as a balloon or surgical valvuloplasty. If the pulmonary valve is too small for
either of these procedures, it can be widened by inserting a homograft patch to widen the PA
from the valve upwards. If the RVOT needs to be widened, a transannular patch may be
needed; this covers part of the right ventricular wall as well as the PA and PV.

In adulthood the degree of obstruction may remain stable or increase and calcification of the
valve can set in and arrhythmias develop. It may be necessary to replace the PV in these
cases.
Significant stenosis causes hypertrophy of the RV, resulting in narrowing of the RVOT leading
to right heart failure and reduced tolerance to exercise. If untreated there is a risk of sudden
death for those over 40.

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Tetralogy of Fallot (TOF)

Tetralogy of Fallot

PA
Ao

LA
RA

*
RV LV

• All (nearly) post radical repair: pink

• Nearly all well
• Most have severe PR

Tetralogy of Fallot is the most common cyanotic heart defect and accounts for 10% of cases
of congenital heart disease. It involves 4 anomalies of the structure of the heart.

 A narrowing (stenosis) of the pulmonary artery.

 A large VSD.
 The aorta ‘overrides’ the VSD.
 The right ventricular muscle wall becomes thickened resulting in right ventricular
hypertrophy.

Patients nowadays have a primary surgical repair in childhood, with a patch to the VSD, most
also need a transannular patch to open up the small pulmonary valve. The pulmonary outflow
tract usually becomes dilated, therefore becoming leaky over time, so they will require a
pulmonary valve replacement later in life, to reduce right ventricular volume overload resulting
from pulmonary regurgitation that is a consequence of the original Tetralogy repair.

Prior to the radical repair, patients may have undergone a systemic-pulmonary shunt called a
BT shunt, older patients may have been treated with other central shunts such as a Pott’s or
Waterson’s shunt to increase the pulmonary blood flow,

These caused excessive blood flow and the complications that arise from that. Therefore
these older patients may have developed pulmonary vascular obstructive disease (PVOD) or
have distorted pulmonary arteries and left ventricular dysfunction.

The prognosis is excellent with the primary repair but there is a small risk of sudden death,
which is usually caused by ventricular arrhythmias

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Pulmonary Atresia (PA) with VSD

Pulmonary atresia is where there is no pulmonary valve or opening through which blood can
enter the pulmonary artery to go to the lungs; this prevents blood flow to the lungs to become
oxygenated. (Atresia means absence of a normal opening)
There are 2 main types of Pulmonary Atresia, those with a VSD present and those with an
intact ventricular septum.
If a VSD is present it may have allowed blood flow into the right ventricle through the hole,
during foetal development, resulting in some growth of that chamber. If the RV is normal or
near normal size an outflow tract is constructed from the RV to the pulmonary artery, so the
closed section is replaced with an artificial, normally functioning pulmonary artery base and
pulmonary valve. This group of patients may need PVR or RVOT (right ventricular outflow
tract) replacements in the future.

This group of patients can also develop MAPCAs (multiple aorto-pulmonary collateral
arterioles), which are large systemic arterial collateral vessels that arise from the aorta or its
major branches to allow blood to flow past the obstruction. They can sometimes be recruited
during an operation to repair the primary defect.

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Truncus Arteriosus

Truncus Arteriosus is where the aorta and pulmonary artery are combined in one large
vessel, known as the Truncus Arteriosus. This then carries blood to the lungs and the body.
There is also a VSD and the truncal artery overrides (straddles) the VSD, so mixed
oxygenated and deoxygenated blood is pumped from both the right and left ventricles to the
lungs and body. This reduces the efficency of the circulatory system. The truncal valve is
often abnormal sometimes having 4 leaflets instead of the normal 3; it can also be thickened
and narrowed, resulting in regurgitation back into the pumping chamber as there is an
obstruction to the blood flow that leaves the heart.
As the lung arteries are connected to the high pressure pumping chambers there is high
blood pressure in the lungs which if untreated will lead to irreversible pulmonary hypertension
and would prove fatal in childhood.
Surgical treatment involves the separation of the pulmonary arteries from the truncus
arteriosus and connected to the right ventricle through a valved tube or conduit. The VSD is
closed and the truncus vessel now has the functional role of the aorta. The valve is repaired
or replaced.
Patients may need replacement of conduits or truncal valve replacements in the future. The
effects of surgery and irregularities in the ventricular outflow tract may cause ventricular
dysfunction and arrhythmias.
The long term outlook for this defect is now improving with better interventional techniques.

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Transposition of the Great Arteries (TGA)

Schematic representation of complete TGA

(ventriculoarterial discordance)

,
Ao aorta, LA left atrium, LV left ventricle,
PA pulmonary artery, MV mitral valve, RA right atrium,
RV right ventricle, TV tricuspid valve
* patent foramen ovale
** patent arterial duct

Transposition of the Great Arteries is where the two main arteries (aorta and pulmonary
artery) are swapped round and are connected to the wrong chambers of the heart. Therefore
the aorta leaves the right ventricle (RV) rather than the left and takes unoxygenated blood to
the body, while the pulmonary artery (PA) leaves the left instead of the right and takes
oxygenated blood to the lungs. It accounts for 5% of all cases of congenital heart disease and
requires surgery in the first week of life.

The operation of choice nowadays is the Arterial Switch, which is where they reconnect the
aorta to the left ventricle and the pulmonary artery to the right artery, ensuring the coronary
arteries are also reconnected in a good position. This was routinely done in Birmingham from
the late 1980’s

Prior to this, the procedure carried out was either a Senning or Mustard procedure, where a
baffle was created within the heart to direct blood flow from one atrium to the other. This
corrected the direction of circulation but the right ventricle remained the systemic one for
which it was not designed

Ao - Aorta
PA - Pulmonary artery
RV - Right ventricle
LV - Left ventricle
SVA - Systemic venous atrium
PVA - Pulmonary venous atrium

Schematic representation of interatrial

repair(Senning or Mustard operation)

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The long term prognosis for patients with Mustard or Senning procedure is not as good as for
those who now have the Arterial Switch. Arrhythmias often develop over time and there is
also the risk of right ventricular failure and pulmonary vein stenosis from scarring of the atrial
baffle. These symptoms are usually progressive.

Patients who have a VSD and pulmonary stenosis as well as TGA may have the Rastelli
operation ( RV – PA conduit)

Ao - Aorta
PA - Pulmonary artery
RV - Right ventricle
LV - Left ventricle
VC – Valved conduit

Schematic representation of Rastelli operation

Rastelli patients can also develop arrhythmias, the conduit may become narrowed and need
replacing.

The long term prognosis for the arterial switch is very good, although due to this being a
comparatively new procedure long term follow up is necessary. They may develop dilated
aortic roots and aortic regurgitation and need further surgery.

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Congenitally Corrected Transposition of the Great Arteries (ccTGA)

Schematic representation of ccTGA

(atrioventricular and ventriculoarterial discordance)

Ao aorta, LA left atrium, LV left ventricle,

PA pulmonary artery, MV mitral valve, RA right atrium,
RV right ventricle, TV tricuspid valve

Also known as AV – VA discordance, ( atrioventricular and ventriculoarterial) this is very rare

accounting for 1% of all congenital heart disease. 95% of patients with ccTGA have
associated anomalies such as
- VSD / PS
- Ebstein Anomaly
- AS
- AVSD
- Coarctation of the Aorta
- Abnormalities of situs

In ccTGA deoxygenated systemic venous blood is pumped to the PA by the morphological

LV, which has been transposed. Oxygenated pulmonary venous blood is pumped to the
Aorta by the morphological RV, the atrio- ventricular valves have also been transposed.

If ccTGA on its own it may remain undiagnosed until middle age, but most become
symptomatic by their 40’s due to failure of systemic RV, tricuspid regurgitation (TR) and
onset of complete heart block and atrial arrhythmias.

If early diagnosis a double switch or Senning-Rastelli can be performed to restore the LV to

the systemic circulation but this is rarely successful if performed in adulthood with a late
diagnosis.

Tricuspid regurgitation (systemic AV valve) is common and may require TVR ( tricuspid valve
replacement) in patients with TR if RVEF ( RV ejection fraction) is greater than 40%.

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Ebstein Anomaly

Ebstein Anomaly is where there is an abnormality of the tricuspid valve when it becomes
displaced into the RV this results in part of the RV becoming functionally part of the RA, this
is called atrialisation of the right ventricle. One or more of the valve leaflets are usually
abnormal causing the valve to be leaky and the right atrium to be larger than usual, which can
cause arrhythmias. There is sometimes an ASD present with this defect and because of the
increased pressure in the right atrium, caused by the leaky tricuspid valve, there may be a
right to left shunt and cyanosis This means that deoxygenated blood crosses into the left side
of the heart and the patient is cyanosed.
In mild cases no treatment is needed, symptoms can be variable and this anomaly is
sometimes not discovered till adulthood. Those that are diagnosed later in life may have
developed an enlarged heart, recurrent arrhythmias due to the enlarged right atrium,
cyanosis and reduced exercise tolerance. If there are significant symptoms the tricuspid
valve is repaired or replaced and the ASD closed. Prognosis post operatively is good with a
decrease in symptoms and increase in exercise tolerance. Those that had a valve
replacement may need it replacing in the future but prosthetic valves can last decades.

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Single ventricle circulation
Single ventricle circulation is used to describe a number of complex congenital heart
conditions in patients have only one functional ventricle rather than the normal two. This
therefore affects the way blood is delivered to the lungs and the rest of the body. Depending
upon the type of heart condition the patient may have a morphological right or left ventricle as
the only pumping chamber. These patients are treated with palliative, rather than curative
surgical procedures.

Conditions which may result in the patient having a Fontan circulation:

 Double outlet right ventricle (DORV)

 Hypoplastic left heart syndrome (HLHS)
 Pulmonary atresia with intact septum (PA/IVS)
 Double inlet left ventricle (DILV)
 Tricuspid atresia.
 Other defects e.g. some forms of Atrioventricular Canal Defects and Pulmonary
Atresia may create single ventricle conditions in the heart.

Adult patients with these defects, whose early treatment consisted of a Glenn procedure and
/ or a modified or classic Blalock-Taussig shunt may be suitable for the Fontan in later life,
depending on assessment of risks and benefits. This group of patients is getting smaller as
patients are now having the Fontan done in childhood.

The prognosis after the Fontan operation for patients with single ventricles is better than other
treatments as improvements in surgical techniques continue to be made.

Blalock- Taussig Shunt – Palliative shunt to increase pulmonary blood flow in PA /PS.
- Classic BT shunt – direct anastomosis between subclavian artery and ipsilateral PA.
- Modified BT shunt – Goretex tube graft between subclavian or innominate and
pulmonary artery

Glenn - Palliative shunt to increase pulmonary blood flow.

- Classic Glenn: SVC – end to end anastomosis with divided RPA
- Bidirectional Glenn: end to side anastomosis of divided SVC to the individual PA so
there is blood flow to both lungs. Also called a CP shunt.

Fontan – Palliative procedure for functionally univentricular hearts, usually proceeded by a

CP shunt.

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Tricuspid Atresia (TA)

Unoperated tricuspid atresia

Ao PA

LA
RA

RV

LV

Tricuspid atresia is where the tricuspid valve has failed to develop. This results in a small
rudimentary RV. The tricuspid valve, which connects the right atrium and right ventricle in the
normal heart, is absent, the right ventricle is small and the pulmonary valve can be narrowed.
There is also an ASD, which allows the right atrium to off load through into the left atrium.
There is also usually a VSD which allows blood to flow into the right ventricle and up the
pulmonary artery.

Adults with this condition will usually have had a Fontan operation in infancy. For those older
adults who had earlier treatments, which consisted of a Glenn procedure and/or the insertion
of a modified or classic Blalock Taussig Shunt, they may be able to have a Fontan in later life.

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Double Outlet Right Ventricle (DORV)

Double Outlet Right Ventricle (DORV) is where the aorta (Ao) and the pulmonary artery (PA)
both exit from the right ventricle (RV), instead of the normal. There is also a large VSD
present (a hole in the septum between the two ventricles) and there can also be some degree
of pulmonary valve stenosis.

Depending upon the size of the right and left ventricle, the surgical treatment can be a Rastelli
procedure, so the VSD is closed and separate the heart to lungs and heart to body
circulations. This means that the patient still has 2 working ventricles. But when either the
RV or LV is hypoplastic (very small), or when the VSD cannot be committed to the aorta, then
the patient will have had the palliative surgeries that result in a Fontan circulation.

The prognosis and long-term treatment depends on which procedure was done. Patients with
the Rastelli repair will need further surgery to replace the pulmonary artery conduit as they
grow or develop conduit or valve stenosis.

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Double inlet left ventricle (DILV)

In the majority of DILV, the positions of the pulmonary artery and the aorta and the left and
right ventricles are reversed. The right ventricle is usually small and the mitral and tricuspid
valves open into the enlarged left ventricle, which is on the right side. There are also present
an ASD and VSD.

It is one of the single ventricle heart defects as it has only one effective pumping chamber.
The majority of patients will now have the palliative treatment which results in a Fontan
circulation in the 3 stages.

In those adult patients where the early treatment consisted of a Glenn procedure and/or
insertion of a shunt between the systemic circulation and the pulmonary artery ( Blalock-
Taussig shunt).

Most patients who have not had the Fontan operation will start to be symptomatic with:
 Cyanosis
 Fatigue
 Arrhythmias
 Exercise intolerance

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Pulmonary atresia with Intact ventricular septum PA /IVS

In pulmonary atresia the pulmonary valve has failed to develop resulting in a small main
pulmonary artery and an intact ventricular septum is present, blood can not flow to the lungs
and because of this lack of blood flowing through the right side of the heart, the structures on
this side do not develop normally and are hypoplastic (small). In cases where the RV is small
and therefore cannot function effectively as a pumping chamber the course of treatment ends
in a Fontan circulation.

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Hypoplastic Left Heart Syndrome (HLHS)

HLHS is the most complex form of univentricular circulation. It is characterised by varying

degrees of hypoplasia of the LV and aorta, and affects the formation of other left sided
structures being associated with aortic and mitral atresia or stenosis. It relies on the
morphological RV to support the systemic circulation.

If left untreated it is incompatible with life . There are no untreated adults with HLHS who have
survived into adulthood.

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These patients will have had their final definitive palliation to a Norwood Fontan circulation at
around 5-6 years of age. Survivors have a Fontan circulation with a systemic RV and
coronary circulation arising from an often diminutive aorta. The procedure was only developed
in the1980’s so this population is much younger than other Fontan survivors and the long-
term complications are unknown.

These complications are likely to be those of any Fontan but may also include:
 Recoarctation at the original repair site.
 LPA- ? stenosis
 ASD- ? restrictive
 Coronaries arising from diminutive aorta- ? ischaemia
 Systemic RV- ? dysfunction
 Systemic tricuspid valve- ? regurgitation.
 Arrhythmias.

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Types of Fontan Circulation:

A Fontan procedure is where the venous return to the heart has been diverted directly into the
pulmonary circulation and is therefore not dependent on the RV pump. The Fontan operation
can be performed in several ways and is done in staged operations during childhood and not
all at once. The 3 main ways that there have been to ensure as much blood as possible
flows to the lungs are:

Atrio pulmonary connection (AP Connection) (rarely used now)

The right atrium is connected to the pulmonary artery, if the tricuspid valve is present it needs
to be closed off with a patch ( to prevent blood flowing into the ventricle). The atrial septal
defect is closed and blood now flows from the superior and inferior vena cava through the
right atrium and into the pulmonary arteries. Blood returns via the pulmonary veins into the
left atrium then into the single ventricle up the aorta and round the body.

AP Connection Fontan

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Extracardiac Fontan:
The superior vena cava is connected to the right pulmonary artery, the inferior vena cava is
re-routed through a Gore-Tex tube or conduit which runs outside the heart and is connected
to the pulmonary arteries. The atrial septum is removed. Blood returns via the pulmonary
veins into the left atrium and passes into the right atrium freely. Therefore blood flow is as
follows: From the body via the vena cava straight to the lungs > from the lungs to the heart via
the pulmonary veins > left atrium > right atrium > single ventricle > aorta > body.

Extracardiac Fontan in
patient with HLHS –
(Norwood)

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Total Cavopulmonary Connection (TCPC):
Venous return is redirected to the pulmonary artery and into the lung by joining the superior
vena cava and the inferior vena cava by a large patch that creates a tunnel through the right
atrium. The vena cavas are then connected directly to the pulmonary arteries above the
heart. The atrial septum is removed and blood returns via the pulmonary veins into the now
independent left atrium down into the single ventricle and then up the aorta and round the
body.

Lateral Tunnel Fontan

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Caring for patients with Fontan circulation.

General management points;

 All patients should be anticoagulated with warfarin.

The flow in the Fontan circuit is not pulsatile but passive, so spontaneous thrombus
formation is possible.

Micro thrombi in distal pulmonary arterioles will result in increased pulmonary vascular
resistance which is detrimental to the Fontan circuit.

 Avoid dehydration.

Dehydration will reduce the filling pressure in the Fontan circuit and reduce pre load in the
single ventricle, thereby compromising cardiac output and systemic blood pressure.

If the patient is NBM they need to have IV fluids 1L normal saline over 12 hours.

Because of the risk of micro thrombi if IV fluids are given a filter should always be used.

 General Anaesthetic.

Avoid GA unless absolutely necessary, if required this should be by an experienced

cardiac anaesthetist. However GA cardioversion is urgent in atrial arrhythmias.

NBM so needs IV fluids 1L normal saline over 12 hours.

Should be first on a list in case of any problems.

All anaesthetic agents cause systemic vasodilatation which can cause circulatory
collapse.

Positive pressure ventilation reduces venous return and therefore cardiac output.

 Management of atrial flutter tachycardia;

These are common and life threatening events. Mechanism is scar related intra atrial re-
entrant tachycardia (IART) or atypical atrial flutter.

Rhythm is often regular and not rapid, so can be mistaken for sinus rhythm.

Chemical cardioversion should not be performed as may cause rapid conduction and
circulatory collapse.

Needs prompt DC cardioversion.

Amiodarone is often needed for long term prevention, which can cause thyrotoxicosis.

EP studies and ablations may be successful but access to the heart is often difficult or
limited.

Failing Fontan

The Fontan procedure is a palliative one and results in the patient being in a low cardiac
output state with long term sequelae including: effort intolerance, arrhythmias and
premature death.

28 Nichola Pope ACHD CNS

 Effort tolerance is limited even in ‘well’ patients.

Signs of failure:

 Ventricular dysfunction.

Increased afterload leads to hypertrophy of the ventricle, which leads to systolic

dysfunction. Limited pre-load recruitment results in diastolic dysfunction.

Management: 1. Treating with beta blockers and spironolactone may help symptoms.
2. Oxygen therapy may help.
3. Diuretics used with caution due to risk of hypovalaemia.
4. Exclude any underlying cause e.g. outflow tract obstruction, Fontan
circuit obstruction.

 Reduced effort tolerance.

This is due to the impaired ventricular function.

Management: 1. Make sure there is no lung involvement.

2. Any restrictive lung defect may benefit from Oxygen therapy
and / or non-invasive ventilation.
3. Exercise may improve symptoms of breathlessness.

 Arrhythmias.

Arrhythmias can develop in patients with Fontan circulation soon after the procedure is done
or much later. Generally the older the patient is when operated on, the more likely they may
have conduction problems.

Patients who had some of the early Fontan procedures had enlargement or hypertrophy of
the right atria due to high systemic blood pressures. This slowed conduction and promotes
the development of arrhythmias.
Recent advances in the Fontan circulation have reduced the cutting of atrial tissue and there
is less exposure of the RA to high systemic blood pressure, which will hopefully reduce the
risk of post operative arrhythmias.

Atrial arrhythmias can occur due to multiple scarring related to suture lines and bypass
cannulations. These can provide circuits for macro re-entrant tachycardias.

These can increase in frequency with age and are associated with a decline in ventricular
function.

Management: 1. Amiodarone often needed to suppress arrhythmia.

2. Pacemakers.
3. Radiofrequency ablations.
4. May be triggered by excessive alcohol.
5. ICD
Due to the previous surgery it is very difficult to get access to the heart.

 PLE (Plasma losing enteropathy)

This is a unique problem for patients with Fontan circulation due to the high mesentric
venous pressure resulting in protein loss into the gut.

Management: 1. Bloods to look for low serum albumin, immunoglobulins.

29 Nichola Pope ACHD CNS

 2. Fresh stool specimen to look for raised faecal alpha-1 antitrypsin levels.
This needs to be liaised with Biochemistry as the sample has to be fresh and delivered
immediately.

Low albumin, total protein and immunoglobulin levels leads to effusions, ascites and
dependent oedema, malnutrition, recurrent cellulites and septicaemia. No effective treatment
and there is a 50% 5 year mortality.

If perfuse diarrhoea present may benefit from Imodium

Long term outcome for patients with Fontan circulation.

The long term outcome is unknown as the current adult patients are the pioneers of this type
of operation. But we have a good 25 years of data in this group of patients. We are also now
getting young adults coming into the service that have had their Fontan operations at an
earlier age, while still in the paediatric sector. The type of Fontan has evolved so there is less
scarring of atrial tissue at the time of surgery thereby hopefully reducing some complications
for this group of patients.

The above complications will inevitably result in significant morbidity and mortality.
Fontan patients account for a small proportion of the total number of patients with congenital
heart disease but account for 50% of emergency admissions.
Prompt management of their medical emergencies prevents premature deaths.

30 Nichola Pope ACHD CNS

Caring for sick/ cyanotic CHD patients:

Cyanosis is present when there is a right to left shunt, this shunting can be anywhere:
between the aorta and pulmonary artery, intracardiac or intrapulmonary. It is usually clinically
seen when oxygen saturations are below 85% and can be associated with low, normal or high
pulmonary blood flow or pulmonary vascular resistance.

 These patients need to avoid dehydration and receive IV fluids when NBM.

 IV fluids must be infused through a filter or an infusion pump with a bubble detector to
reduce the risk of paradoxical embolism causing a stroke.

 These patients are at risk of renal failure so need to avoid or use with care;

1. Aminoglycosides
2. NSAIA
3. Contrast agents

 Maintain adequate Hb post operatively for optimum oxygen carrying capacity.

 Avoid vasodilators as they increase cyanosis.

 Tachyarrhythmias: Atrial arrhythmias –usually not tolerated well so prompt

cardioversion is required.

 Haemoptysis – can be life threatening so admission to hospital is needed. IV

fluids, X match, FFP and platelets and CT scan needed to assess site of
bleeding. May need bleeding collaterals embolised and systemic
hypertension treated. Major bleeding may need to consider intubation and a
catastrophic bleed may be fatal so high dose opiates would be appropriate.

 Cerebral abscess- CT scan and blood culture to rule out if there is

unexplained fever, leucoctyosis, headache or neurological signs.

 Contact Cardiologist on call or ACHD SpR as soon as possible.

 If a non- cardiac operation is needed a cardiac anaesthetist should be used

31 Nichola Pope ACHD CNS

Eisenmenger Syndrome
This is usually seen with large untreated VSDs but can also happen with large
communications between systemic and pulmonary circulations such as ASDs and Patent
Ductus Arteriosus (PDAs). To begin with there is a left to right shunt as the higher blood
pressure in the LV will cause blood to flow into the RV and so increase the blood flow to the
lungs via the PA. This leads to damages of the lung arterioles and gradually increases the
resistance to pulmonary blood flow resulting in pulmonary hypertension when pulmonary
artery pressures equals systolic pressure, and shunt reversal occurs resulting in cyanosis. It
is irreversible
This causes cyanosis as deoxygenated blood is then shunted into the LV and pumped back
round the body.
The damage caused from the high pulmonary blood pressure may not be seen till later in life
when the patient deteriorates over time and symptoms worsen and there is collateral damage
to the heart where the function of the RV and tricuspid valve is affected.
Features include:
 Fainting
 Chest pain
 Arrhythmias
 Reduced exercise tolerance
 Haemoptysis
Sudden death can also occur.
Once pulmonary hypertension has developed it cannot be reversed. Early surgical closure of
large VSD is the best way to avoid the development of Eisenmenger Complex.
Patients can lead long, active lives but must avoid situations that cause a decrease in the
resistance to blood flow from the heart to the body, as this can result in dangerously low
levels of oxygen ( hypoxaemia)
Patients should avoid dehydration, high altitudes, respiratory infections, sudden immersion in
cold water, recreational drugs use, pregnancy, prolonged exposure to external heat or fever,
aspirin and other AI drugs that can cause bleeding.
Arrhythmia and a tendency to bleed can develop over time.
There is also a secondary erthrocytosis (polycythaemia), high haemoglobin, present which is
a physiological adaptation to hypoxia, by maximising oxygen carrying capacity. It can cause
symptomatic hyperviscosity but does not increase the risk of stroke and routine venesection is
not advised.

32 Nichola Pope ACHD CNS

Contraception and Pregnancy
The numbers of women surviving with complex congenital heart disease is growing. The
maternal risk of this group varies from no difference to that of the general population of child
bearing age to a high risk of long term morbidity and >40% risk of death.

Therefore this group of patients need expert advice which should include:
 Contraception options.
 Pre pregnancy counselling including maternal and foetal risks both ante and post-
nasally.
 Ante natal and post natal care.
 Possible delivery at a specialist obstetric centre.

Contraception is important to discuss and they should have a reliable, safe and effective form
of contraception.
Patients with the following defects should be on progesterone only forms of contraception due
to the risks of blood clot formation:
- Single ventricle defects / Fontan circulation
- Severe AS/AR
- Patients on Warfarin
- Pulmonary hypertension
- Cyanotic patients
- Atrial arrhythmias
- Dilated atria
- Dilated poorly functioning LV
Cardiovascular changes involved in pregnancy are especially high risk for women with single
ventricular defects, the degree of risk depends on their ventricular function and other factors.
Arrhythmias may cause complications and nearly a third of pregnancies in patients with
Tricuspid Atresia and HLHS will result in miscarriage.

Warfarin is oxygenic to the developing foetus and will usually need to be changed to cleaner
as soon as possible.

Patients with complex congenital heart defects may need hospitalization especially towards
the end of the pregnancy and as long as there are no obstetric contraindications a normal
vaginal delivery is usually preferred.

33 Nichola Pope ACHD CNS

Endocarditis

Patients with congenital heart disease have a risk of bacterial endocarditis and should
therefore be aware of the symptoms and the need for good oral hygiene and regular dental
checkups.

Current NICE guidelines 2008, recommend that patients do not need antibiotic cover prior to
dental treatment but this should always be discussed with their cardiologist, especially if they
have had a previous episode of endocarditis.

Patients requiring valve or catheter intervention should always have a recent dental check up
before the procedure.
There is also a small risk of endocarditis following piercings and tattoos.

34 Nichola Pope ACHD CNS

Glossary

Atresia - Congenital or acquired condition where a valve or artery fails to develop causing
complete occlusion.

Baffle – Surgically created wall within the atrial chambers.

Blalock- Taussig Shunt – Palliative shunt to increase pulmonary blood flow in PA /PS.
- Classic BT shunt – direct anastomosis between subclavian artery and ipsilateral PA.
- Modified BT shunt – Goretex tube graft between subclavian or innominate and
pulmonary artery

Conduit - Surgically created tube connection, can be valved, between 2 vascular channels
such as extra cardiac conduit Fontan or RV-PA.

Damus Kaye Stansel operation – direct anastomosis of the main PA, which is separated
from the branch PA’s, onto the aorta to create a single arterial outlet to the heart. The branch
PA’s must be supplied by a shunt or conduit, usually performed for univentricular hearts.

Double switch operation – Corrective procedure for ccTGA. Arterial and atrial switch,
restoring the morphological LV to the systemic circulation.

Fontan – Palliative procedure for functionally univentricular hearts, usually proceeded by a

CP shunt.

Fontan circulation – IVC and SVC are connected directly to the pulmonary artery. Blood flow
through the lungs relies on passive flow down a venous pressure gradient. Pulmonary and
systemic circulations are separated but some have a fenestration allowing a small R-L shunt.
- Classic Fontan (Atrio pulmonary (AP) Connection) - direct anastomosis between RA
and PA.
- Total cavo- pulmonary connection (TCPC) – both the IVC and SVC are connected
individually onto the PA’s using a bidirectional Glenn for the SVC and a lateral tunnel
created within the RA between the IVC and RPA.
- Extra cardiac – SVC connected as above and the IVC to the PA by using a goretex
conduit between the IVC and PA outside of the heart.

Glenn - Palliative shunt to increase pulmonary blood flow.

- Classic Glenn: SVC – end to end anastomosis with divided RPA
- Bidirectional Glenn: end to side anastomosis of divided SVC to the individual PA so
there is blood flow to both lungs. Also called a CP shunt.

Mustard / Senning procedure - Palliative procedure for simple TGA where systemic and
pulmonary venous return are redirected by means of an intra –atrial baffle to relieve
cyanosis.
Norwood procedure – Stage 1 palliative operation done in the neonatal period for HLHS.

Potts anastomosis – palliative shunt to increase pulmonary blood flow used in PS and PA.
Descending aorta to LPA by direct anastomosis.

Protein losing enteropathy (PLE) – condition where there is persistent loss of protein
through the GI tract. Patients have low serum albumin and chronic pleural effusions, ascites
and dependent oedema and high faecal Alpha 1 antitrypsin levels.

Rastelli - Conduit is placed between the Right ventricle and the pulmonary artery. The VSD

35 Nichola Pope ACHD CNS

is closed so that the LV empties into the aorta and therefore supports the systemic
circulation. Corrective operation for TGA/VSD/PS.

Ross procedure – Aortic valve replacement using the patient's pulmonary valve as an
autograft to the aorta and then putting in a homograft PVR. Only tends to be successful if
done in childhood.

Waterston shunt – palliative shunt for PS /PA to increase pulmonary blood flow. Ascending
aorta to RPA by direct anastomosis.

36 Nichola Pope ACHD CNS