CONGENITAL HEART DISEASE CONGENITAL HEART DISEASE usually manifests in childhood but may pass unrecognised and usually manifests in childhood but may pass unrecognised and

not present until adult life.not present until adult life. Defects which are well tolerated, e.g. atrial septal defect, Defects which are well tolerated, e.g. atrial septal defect,

may cause no symptoms until adult life or may be detected may cause no symptoms until adult life or may be detected

incidentally on routine examination or chest X-ray. incidentally on routine examination or chest X-ray.

Congenital defects that were previously fatal in childhood can Congenital defects that were previously fatal in childhood can

now be corrected, or at least partially corrected, so that survival now be corrected, or at least partially corrected, so that survival

to adult life is the norm. Such patients may remain well for manyto adult life is the norm. Such patients may remain well for many

years and subsequently re-present in later life with related years and subsequently re-present in later life with related

problems such as arrhythmia or ventricular dysfunctionproblems such as arrhythmia or ventricular dysfunction

The fetal circulation The fetal circulation Understanding the fetal circulation helps to Understanding the fetal circulation helps to

understand how some forms of congenital heart understand how some forms of congenital heart disease occur. disease occur.

The fetus has only a small flow of blood through the The fetus has only a small flow of blood through the lungs, as it obviously does not breathe in utero.lungs, as it obviously does not breathe in utero.

The fetal circulation therefore allows oxygenated The fetal circulation therefore allows oxygenated blood from the placenta to pass directly to the left blood from the placenta to pass directly to the left side of the heart through the foramen ovale without side of the heart through the foramen ovale without having to flow through the lungs. Congenital defects having to flow through the lungs. Congenital defects may arise if the changes from fetal circulation to the may arise if the changes from fetal circulation to the extrauterine circulation are not properly completed. extrauterine circulation are not properly completed.

PRESENTATION OF CONGENITAL HEART DISEASE PRESENTATION OF CONGENITAL HEART DISEASE THROUGHOUT LIFETHROUGHOUT LIFE

Birth and neonatal periodBirth and neonatal period

Cyanosis Cyanosis

Heart failure Heart failure

Infancy and childhoodInfancy and childhood

Cyanosis Cyanosis

Heart failure Heart failure

Arrhythmia Arrhythmia

Murmur Murmur

Failure to thrive Failure to thrive

Adolescence and adulthoodAdolescence and adulthood Heart failure Heart failure

Murmur Murmur

Arrhythmia Arrhythmia

Cyanosis due to shunt reversal (Eisenmenger's Cyanosis due to shunt reversal (Eisenmenger's syndrome) syndrome)

Hypertension (coarctation) Hypertension (coarctation)

Late consequences of previous cardiac surgery, e.g. Late consequences of previous cardiac surgery, e.g. arrhythmia, heart failure arrhythmia, heart failure

INCIDENCE AND RELATIVE FREQUENCY OF CONGENITAL INCIDENCE AND RELATIVE FREQUENCY OF CONGENITAL CARDIAC MALFORMATIONS Lesion% of all CHDCARDIAC MALFORMATIONS Lesion% of all CHD

%% Ventricular septal defect 30Ventricular septal defect 30

Atrial septal defect 10Atrial septal defect 10 Patent ductus arteriosus 10 Patent ductus arteriosus 10

Pulmonary stenosis 7 Pulmonary stenosis 7

Coarctation of aorta 7 Coarctation of aorta 7

Aortic stenosis 6 Aortic stenosis 6

Tetralogy of Fallot 6 Tetralogy of Fallot 6

Complete transposition of 4Complete transposition of 4 great arteries great arteries Others 20 Others 20

Aetiology and incidence Aetiology and incidence The incidence of haemodynamically significant The incidence of haemodynamically significant

congenital cardiac abnormalities is about 0.8% of congenital cardiac abnormalities is about 0.8% of live births.live births.

Maternal infection or exposure to drugs or toxins Maternal infection or exposure to drugs or toxins may cause congenital heart disease. may cause congenital heart disease.

Maternal rubella infection is associated with Maternal rubella infection is associated with persistent ductus arteriosus, pulmonary valvular persistent ductus arteriosus, pulmonary valvular and/or artery stenosis, and atrial septal defect.and/or artery stenosis, and atrial septal defect.

Maternal alcohol misuse is associated with septal Maternal alcohol misuse is associated with septal defects, and maternal lupus erythematosus with defects, and maternal lupus erythematosus with congenital complete heart block.congenital complete heart block.

Genetic or chromosomal abnormalities such as Genetic or chromosomal abnormalities such as Down's syndrome may cause septal defects, and Down's syndrome may cause septal defects, and gene defects have also been identified as causing gene defects have also been identified as causing specific abnormalities, e.g. Marfan's and DiGeorge's specific abnormalities, e.g. Marfan's and DiGeorge's (deletion in chromosome 22q) syndromes. (deletion in chromosome 22q) syndromes.

PERSISTENT DUCTUS ARTERIOSUSPERSISTENT DUCTUS ARTERIOSUS

AetiologyAetiology:: During fetal life, before the lungs begin to During fetal life, before the lungs begin to

function, most of the blood from the function, most of the blood from the pulmonary artery passes through the ductus pulmonary artery passes through the ductus arteriosus into the aorta. arteriosus into the aorta.

Normally the ductus closes soon after birth Normally the ductus closes soon after birth but sometimes it fails to do so. but sometimes it fails to do so.

Persistence of the ductus is more common Persistence of the ductus is more common in in femalesfemales. .

Since the pressure in the aorta is higher than Since the pressure in the aorta is higher than that in the pulmonary artery (PA), there will that in the pulmonary artery (PA), there will be a continuous arteriovenous shunt.be a continuous arteriovenous shunt.

Clinical featuresClinical features With small shunts there may be no With small shunts there may be no

symptoms for years, but when the ductus is symptoms for years, but when the ductus is largelarge, growth and development may be , growth and development may be retarded.retarded.

cardiac failurecardiac failure may eventually ensue, may eventually ensue, dyspnoea being the first symptom. dyspnoea being the first symptom.

A continuous A continuous 'machinery' murmur'machinery' murmur is heard is heard with late systolic accentuation, maximal in with late systolic accentuation, maximal in the second left intercostal space below the the second left intercostal space below the clavicle.clavicle.

It is frequently accompanied by a thrill. It is frequently accompanied by a thrill. Pulses are increased in volume. Pulses are increased in volume.

Enlargement of the pulmonary artery Enlargement of the pulmonary artery may be detected radiologicaly. may be detected radiologicaly.

The ECG is usually normal.The ECG is usually normal.

Persistent ductus with reversed shuntingPersistent ductus with reversed shunting: : If pulmonary vascular resistance increases, If pulmonary vascular resistance increases,

pulmonary artery pressure rises and may pulmonary artery pressure rises and may continue to do so until it equals or exceeds continue to do so until it equals or exceeds aortic pressure. aortic pressure.

The shunt through the defect may then reverse, The shunt through the defect may then reverse, causing central cyanosis causing central cyanosis (Eisenmenger's (Eisenmenger's syndrome), syndrome), which may be more apparent in the which may be more apparent in the feet and toes than in the upper part of the body. feet and toes than in the upper part of the body.

The murmur becomes quieter, may be confined The murmur becomes quieter, may be confined to systole or may disappear. to systole or may disappear.

The ECG shows evidence of right ventricular The ECG shows evidence of right ventricular hypertrophy. hypertrophy.

Management: Management: It is now usual practice to close a patent It is now usual practice to close a patent

ductus at cardiac catheterisation with an ductus at cardiac catheterisation with an implantable occlusive device.implantable occlusive device.

Closure should be undertaken in infancy if Closure should be undertaken in infancy if the shunt is significant and pulmonary the shunt is significant and pulmonary resistance not elevated,resistance not elevated,

but this may be delayed until later childhood but this may be delayed until later childhood in those with smaller shunts, for whom in those with smaller shunts, for whom closure remains advisable to reduce the risk closure remains advisable to reduce the risk of endocarditis. of endocarditis.

Pharmacological treatment in the neonatal Pharmacological treatment in the neonatal period a prostaglandin synthetase inhibitor period a prostaglandin synthetase inhibitor (indometacin or ibuprofen) may be used in (indometacin or ibuprofen) may be used in the first week of lifethe first week of life

However, in the presence of a congenital defect with However, in the presence of a congenital defect with impaired lung perfusion (e.g. severe pulmonary impaired lung perfusion (e.g. severe pulmonary stenosis and left-to-right shunt through the ductus), stenosis and left-to-right shunt through the ductus), it may be advisable to improve oxygenation by it may be advisable to improve oxygenation by keeping the ductus open with prostaglandin keeping the ductus open with prostaglandin treatment.treatment.

COARCTATION OF THE AORTACOARCTATION OF THE AORTA Aetiology : Narrowing of the aorta most Aetiology : Narrowing of the aorta most

commonly occurs in the region where the commonly occurs in the region where the ductus arteriosus joins the aorta, i.e. at the ductus arteriosus joins the aorta, i.e. at the isthmus just below the origin of the left isthmus just below the origin of the left subclavian arterysubclavian artery

The condition is The condition is twicetwice as common as common in males in males as in females and occurs in 1 in 4000 as in females and occurs in 1 in 4000 children.children.

It is associated with other abnormalities, of It is associated with other abnormalities, of which the most frequent are which the most frequent are bicuspid aortic bicuspid aortic valve and valve and 'berry'berry' aneurysms of the cerebral ' aneurysms of the cerebral circulation. circulation.

Acquired coarctation of the aorta is Acquired coarctation of the aorta is rare but may follow trauma or occur rare but may follow trauma or occur complication of a progressive arteritis complication of a progressive arteritis Takayasu's disease.Takayasu's disease.

Clinical features and investigations Clinical features and investigations Aortic coarctation is an important cause of Aortic coarctation is an important cause of

cardiac failurecardiac failure in the newborn, but symptoms in the newborn, but symptoms are often absent when it is detected in older are often absent when it is detected in older children or adults.children or adults.

Headaches may occur from hypertension Headaches may occur from hypertension proximal to the coarctation.proximal to the coarctation.

weakness or cramps in the legs may result weakness or cramps in the legs may result from decreased circulation in the lower part from decreased circulation in the lower part of the body. of the body.

The blood pressure is raised in the upper The blood pressure is raised in the upper body body but normal or low in the legs. but normal or low in the legs.

The femoral pulses are weak, and delayed in The femoral pulses are weak, and delayed in comparison with the radial pulse. comparison with the radial pulse.

A systolic murmur A systolic murmur is usually heard is usually heard posteriorly, over the coarctation.posteriorly, over the coarctation.

There may also be an ejection click and There may also be an ejection click and systolic murmur in the aortic area due to a systolic murmur in the aortic area due to a bicuspid aortic valve.bicuspid aortic valve.

As a result of the aortic narrowing, As a result of the aortic narrowing, collaterals form, mainly involving the collaterals form, mainly involving the periscapular, internal mammary and periscapular, internal mammary and intercostal arteries.intercostal arteries.

These may result in localized bruits. These may result in localized bruits.

Chest X-ray in early childhood is often Chest X-ray in early childhood is often normal but at a later age may show normal but at a later age may show changes in the contour of the aorta changes in the contour of the aorta (indentation of the descending aorta, (indentation of the descending aorta, '3 '3 signsign') ') and notching and notching of the under-of the under-surfaces of the ribs from collaterals. surfaces of the ribs from collaterals.

MRI is ideal for demonstrating the MRI is ideal for demonstrating the lesion.lesion.

The ECG may show left ventricular The ECG may show left ventricular hypertrophyhypertrophy

Management Management In untreated cases, death may occur from left In untreated cases, death may occur from left

ventricular failure, dissection of the aorta or ventricular failure, dissection of the aorta or cerebral hemorrhage. cerebral hemorrhage.

Surgical correction is advisable in all but the Surgical correction is advisable in all but the mildest cases. mildest cases.

If this is done sufficiently early in childhood, If this is done sufficiently early in childhood, persistent hypertension can be avoided.persistent hypertension can be avoided.

Patients repaired in late childhood or adult Patients repaired in late childhood or adult life often remain hypertensive or develop life often remain hypertensive or develop recurrent hypertension later in life. recurrent hypertension later in life.

Recurrence of stenosis may occur as the Recurrence of stenosis may occur as the child grows, and this may be managed by child grows, and this may be managed by balloon dilatation, which can also be used as balloon dilatation, which can also be used as the primary treatment in some cases.the primary treatment in some cases.

ATRIAL SEPTAL DEFECTATRIAL SEPTAL DEFECT

AetiologyAetiology: is one of the most common : is one of the most common congental heart defects.congental heart defects.

occurs twice as frequently in occurs twice as frequently in femalesfemales.. Most are Most are 'ostium secundum'ostium secundum' defects,' defects, involving the fossa ovalis which in utero wasinvolving the fossa ovalis which in utero was the foramen ovalethe foramen ovale

'Ostium primum' defects result from a defect 'Ostium primum' defects result from a defect in the in the

atrioventricular septum and are associatedatrioventricular septum and are associated with a 'cleft mitral valve' (split anterior with a 'cleft mitral valve' (split anterior

leafletleaflet). ).

Since the normal right ventricle is moreSince the normal right ventricle is more compliant than the left, a large volume of compliant than the left, a large volume of blood shunts through the defect from the left to blood shunts through the defect from the left to the right atrium and then to the right ventricle the right atrium and then to the right ventricle and pulmonary arteries and pulmonary arteries

As a result there is gradual enlargement of the As a result there is gradual enlargement of the right side of the heart and of the pulmonary right side of the heart and of the pulmonary arteries. arteries.

Pulmonary hypertension and shunt reversal Pulmonary hypertension and shunt reversal sometimes complicate atrial septal defect, but sometimes complicate atrial septal defect, but are less commonare less common

and tend to occur later in life than with other and tend to occur later in life than with other types of left-to-right shunt. types of left-to-right shunt.

Clinical features: Clinical features: Most children are Most children are freefree of symptoms for many years of symptoms for many years

and the condition is often detected at routine clinical and the condition is often detected at routine clinical examination or following a chest X-ray.examination or following a chest X-ray.

Dyspnoea, chest infections, cardiac failure and Dyspnoea, chest infections, cardiac failure and arrhythmias, arrhythmias, especially atrial fibrillation, are other especially atrial fibrillation, are other possible modes of presentation. possible modes of presentation.

wide fixed splitting of the second heart soundwide fixed splitting of the second heart sound: wide : wide because of delay in right ventricular ejection because of delay in right ventricular ejection (increased stroke volume and right bundle branch (increased stroke volume and right bundle branch block) and fixed because the septal defect equalises block) and fixed because the septal defect equalises left and right atrial pressures throughout theleft and right atrial pressures throughout the respiratory cycle.respiratory cycle.

a systolic flow murmur over the pulmonary a systolic flow murmur over the pulmonary valve.valve.

In children with a large shunt, there In children with a large shunt, there may be a diastolic flow murmur over may be a diastolic flow murmur over the tricuspid valve. Unlike a mitral flow the tricuspid valve. Unlike a mitral flow murmur, this is usually high-pitched.murmur, this is usually high-pitched.

The chest X-rayThe chest X-ray typically shows enlargement of the heart typically shows enlargement of the heart

and the pulmonary artery as well as and the pulmonary artery as well as pulmonary plethora. pulmonary plethora.

The ECG usually shows incomplete right The ECG usually shows incomplete right bundle branch block because right bundle branch block because right ventricular depolarisation is delayed as a ventricular depolarisation is delayed as a result of ventricular dilatation. (with a result of ventricular dilatation. (with a 'primum' defect there is also left axis 'primum' defect there is also left axis deviation). deviation).

Echocardiography can directly demonstrate Echocardiography can directly demonstrate the defect and typically shows RV dilatation, the defect and typically shows RV dilatation, RV hypertrophy and pulmonary artery RV hypertrophy and pulmonary artery dilatation. dilatation. The precise size and location of the defect can The precise size and location of the defect can be shown by transoesophageal echocardiography. be shown by transoesophageal echocardiography.

ManagementManagement Atrial septal defects in which pulmonary Atrial septal defects in which pulmonary

flow is increased 50% above systemic flow flow is increased 50% above systemic flow (i.e. flow ratio of 1.5:1) are often large enough (i.e. flow ratio of 1.5:1) are often large enough to be clinically recognisable and should be to be clinically recognisable and should be closed surgically. Closure can also be closed surgically. Closure can also be accomplished at cardiac catheterisation accomplished at cardiac catheterisation using implantable closure devices.using implantable closure devices.

The long-term prognosis thereafter is The long-term prognosis thereafter is excellent unless pulmonary hypertension excellent unless pulmonary hypertension has developed.has developed.

Severe pulmonary hypertension and shunt Severe pulmonary hypertension and shunt reversal are both contraindications to reversal are both contraindications to surgerysurgery

VENTRICULAR SEPTAL DEFECTVENTRICULAR SEPTAL DEFECT Aetiology: occurs as a result of incomplete Aetiology: occurs as a result of incomplete

septation of the ventricles. septation of the ventricles. Embryologically, the interventricular septum has a Embryologically, the interventricular septum has a

membranousmembranous and a and a muscularmuscular portion, and the latter portion, and the latter is further divided into is further divided into inflow, trabecular and outflow inflow, trabecular and outflow portions. portions.

Most congenital defects are 'Most congenital defects are 'perimembranousperimembranous', i.e. at ', i.e. at the junction of the membranous and muscular the junction of the membranous and muscular portions. portions.

V.S.D are V.S.D are the most common congenital the most common congenital cardiac cardiac defect, occurring once in 500 live births. defect, occurring once in 500 live births.

The defect may be isolated or part of complex The defect may be isolated or part of complex congenital heart disease. congenital heart disease.

Acquired ventricular septal defect may result from Acquired ventricular septal defect may result from rupture as a complication of acute myocardial rupture as a complication of acute myocardial infarction, or rarely from trauma.infarction, or rarely from trauma.

Clinical features Clinical features Flow from the high-pressure left ventricle to the low-Flow from the high-pressure left ventricle to the low-

pressure right ventricle during systole produces a pressure right ventricle during systole produces a pansystolic murmurpansystolic murmur usually heard best at the left usually heard best at the left sternal edge but radiating all over the precordium . sternal edge but radiating all over the precordium .

A small defect often produces a loud murmur A small defect often produces a loud murmur (maladie de Roger)(maladie de Roger) in the absence of other in the absence of other haemodynamic disturbance. haemodynamic disturbance.

Conversely, a large defect may produce a softer Conversely, a large defect may produce a softer murmur, particularly if pressure in the right ventricle murmur, particularly if pressure in the right ventricle is elevated. is elevated.

This may be found immediately after birth, while This may be found immediately after birth, while pulmonary vascular resistance remains high, or pulmonary vascular resistance remains high, or when the shunt is reversed in Eisenmenger's when the shunt is reversed in Eisenmenger's syndrome. syndrome.

Congenital ventricular septal defect may Congenital ventricular septal defect may present as:present as:

cardiac failure in infantscardiac failure in infants, , as as a murmura murmur with only minor haemodynamic with only minor haemodynamic

disturbance in older children or adults, disturbance in older children or adults, or rarely or rarely as Eisenmenger's as Eisenmenger's syndrome.syndrome. In a proportion of infants, the murmur gets In a proportion of infants, the murmur gets

quieter or disappears due to spontaneous quieter or disappears due to spontaneous closure of the defect. closure of the defect.

If cardiac failure complicates a large defect, it If cardiac failure complicates a large defect, it is usually absent in the immediate postnatal is usually absent in the immediate postnatal period and only becomes apparent in the first period and only becomes apparent in the first 4-6 weeks of life. 4-6 weeks of life.

The chest X-ray shows pulmonary plethora The chest X-ray shows pulmonary plethora ECG shows bilateral ventricular hypertrophy.ECG shows bilateral ventricular hypertrophy.

Management :Management : Small VSD require no specific Small VSD require no specific

treatment apart from endocarditis treatment apart from endocarditis prophylaxis. prophylaxis.

Cardiac failure in infancy is initially Cardiac failure in infancy is initially treated medically with digoxin and treated medically with digoxin and diuretics. diuretics.

Persisting failure is an indication for Persisting failure is an indication for surgical repair of the defect. surgical repair of the defect.

Percutaneous closure devices are Percutaneous closure devices are under development. under development.

Doppler echocardiography helps to Doppler echocardiography helps to predict the small septal defects that are predict the small septal defects that are likely to close spontaneously.likely to close spontaneously.

Surgical closure is contraindicated in fully Surgical closure is contraindicated in fully developed Eisenmenger's syndrome when heart-developed Eisenmenger's syndrome when heart-lung transplantation may be the only effective lung transplantation may be the only effective method of treatment. method of treatment.

PrognosisPrognosis

Except in the case of Eisenmenger's syndrome, Except in the case of Eisenmenger's syndrome, long-term prognosis is very good in congenital long-term prognosis is very good in congenital ventricular septal defect.ventricular septal defect.

Many patients with Eisenmenger's syndrome die in Many patients with Eisenmenger's syndrome die in the second or third decade of life, but a few survive the second or third decade of life, but a few survive to the fifth decade without transplantationto the fifth decade without transplantation

TETRALOGY OF FALLOTTETRALOGY OF FALLOT The tetralogy comprises (1) pulmonary The tetralogy comprises (1) pulmonary

stenosis, (2) overriding of the ventricular stenosis, (2) overriding of the ventricular septal defect by the aorta, (3) a ventricular septal defect by the aorta, (3) a ventricular septal defect and (4) right ventricular septal defect and (4) right ventricular hypertrophy.hypertrophy.

Aetiology Aetiology The embryological cause is abnormal The embryological cause is abnormal

development of the bulbar septum which development of the bulbar septum which separates the ascending aorta from the separates the ascending aorta from the pulmonary artery, and which normally aligns pulmonary artery, and which normally aligns and fuses with the outflow part of the and fuses with the outflow part of the interventricular septum. interventricular septum.

The defect occurs in about 1 in 2000 births The defect occurs in about 1 in 2000 births and is and is the most common cause of cyanosis the most common cause of cyanosis in infancy in infancy afterafter the first year of life. the first year of life.

Clinical features Clinical features Children Children are usually cyanosed are usually cyanosed but this may not be but this may not be

present in the neonate because it is only when right present in the neonate because it is only when right ventricular pressure rises to equal or exceed left ventricular pressure rises to equal or exceed left ventricular pressure that a large right-to-left shunt ventricular pressure that a large right-to-left shunt develops. develops.

The subvalvular component of the right ventricle The subvalvular component of the right ventricle outflow obstruction is dynamic, and may increase outflow obstruction is dynamic, and may increase suddenly under adrenergic stimulation.suddenly under adrenergic stimulation.

The affected child suddenly becomes increasingly The affected child suddenly becomes increasingly cyanosed, often after feeding or a crying attack, and cyanosed, often after feeding or a crying attack, and may become apnoeic and unconscious.may become apnoeic and unconscious.

These attacks are called These attacks are called 'Fallot's spells'. 'Fallot's spells'. In older children, Fallot's spells are uncommon but In older children, Fallot's spells are uncommon but

cyanosis becomes increasingly apparent, with cyanosis becomes increasingly apparent, with stunting of growth, digital clubbing and stunting of growth, digital clubbing and polycythemia. polycythemia.

Some children characteristically obtain relief by Some children characteristically obtain relief by squatting after exertion, which increases the squatting after exertion, which increases the afterload of the left heart and reduces the right-to-afterload of the left heart and reduces the right-to-left shunting. left shunting.

The natural history before the development of The natural history before the development of surgical correction was variable, but most patients surgical correction was variable, but most patients died in infancy or childhood.died in infancy or childhood.

On examinationOn examination the most characteristic feature is the the most characteristic feature is the

combination ofcombination of cyanosis cyanosis with a loud ejection with a loud ejection systolic murmusystolic murmur in the pulmonary area (as for r in the pulmonary area (as for pulmonary stenosis).pulmonary stenosis).

However, cyanosis may be absent in the However, cyanosis may be absent in the newborn or in patients with only mild right newborn or in patients with only mild right ventricular outflow obstruction (ventricular outflow obstruction ('acyanotic 'acyanotic tetralogy of Fallot'). tetralogy of Fallot').

Investigations Investigations The ECG shows right ventricular hypertrophy, The ECG shows right ventricular hypertrophy, chest X-ray shows an abnormally chest X-ray shows an abnormally small small

pulmonary artery and a 'boot-shaped' heart. pulmonary artery and a 'boot-shaped' heart. Echocardiography is diagnostic and Echocardiography is diagnostic and

demonstrates that the aorta is not continuous demonstrates that the aorta is not continuous with the anterior ventricular septumwith the anterior ventricular septum

Management Management

The definitive management is total correction of the The definitive management is total correction of the defect by defect by surgical relief of the pulmonary stenosis surgical relief of the pulmonary stenosis and closure of the ventricular septal defect. and closure of the ventricular septal defect.

Primary surgical correction may be undertaken prior Primary surgical correction may be undertaken prior to age 5, unless the pulmonary arteries are too to age 5, unless the pulmonary arteries are too hypoplastic, when a palliative shunt may be hypoplastic, when a palliative shunt may be performed (e.g. the Blalock-Taussig shunt, an performed (e.g. the Blalock-Taussig shunt, an anastomosis between the pulmonary artery and anastomosis between the pulmonary artery and subclavian artery). subclavian artery).

The prognosis after total correction is good, The prognosis after total correction is good, especially if the operation is performed in childhood. especially if the operation is performed in childhood.

OTHER CAUSES OF CYANOTIC CONGENITAL HEART OTHER CAUSES OF CYANOTIC CONGENITAL HEART DISEASEDISEASE

Tricupid atresiaTricupid atresia

Transposition of great vesselsTransposition of great vessels

Pulmonary atresiaPulmonary atresia

Ebstein s anomalyEbstein s anomaly