Examine this patient's heart.

Examine this patient's cardiovascular system.



· Symptoms may not appear until early late childhood or early adulthood.

· Cyanosis (appears as right-to-left shunting develops).

· Dyspnoea on exercise and impaired exercise tolerance.

· Palpitations (common and usually due to atrial fibrillation or flutter).

· Angina of effort.

· Haemoptysis (may occur as a result of pulmonary infarction, or rupture of dilated pulmonary arteries, or

aorticopulmonary vessels).

· Syncope (owing to inadequate cardiac output or, less commonly, an arrhythmia).

· Symptoms of hyperviscosity including visual disturbances, fatigue, headache, dizziness, and


· Symptoms of heart thilure are uncommon until the disease is in its advanced stages.


· Clubbing of fingers and central cyanosis.

· 'a' waves in the JVP, 'v' wave if tricuspid regurgitation is also present.

· Left parasternal heave and palpable P2.

· Loud P2, pulmonary ejection click, early diastolic murmur of pulmonary regurgitation (Graham Steell


· Loud pansystolic murmur of tricuspid regurgitation.

· Listen carefully to the second sound. The clinical findings resulting from the underlying defect are as

follows: -VSD: single second sound

-ASD: fixed, wide split second sound

-PDA: reverse split of second sound and differential cyanosis where lower-limb cyanosis is marked.


This patient has Eisenmenger syndrome with a shunt at the ventricular level (lesion) which is congenital

in origin (aetiology), and severe pulmonary hypertension (functional status).


What do you understand by the term 'Eisenmenger syndrome'?]

Pulmonary hypertension with a reversed or bidirectional shunt. It matters very little where the shunt

happens to be (e.g. VSD, ASD, patent ductus arteriosus, persistent truncus arteriosus, single ventricle or

common atrioventricular canal).


What do you understand by the term 'Eisenmenger complex'?

Eisenmenger complex is a VSD with a right-to-left shunt in the absence of pulmonary stenosis. The

onset of Eisenmenger syndrome is often heralded by a softening of the murmur, a decrease in the left

heart size and an increase in the second pulmonic sound.

Mention some cyanotic heart diseases of infancy.

· Tetralogy of Fallot.

· Transposition of the great vessels.

· Tricuspid regurgitation.

· Total anomalous pulmonary venous connection.

What is the age of onset of Eisenmenger syndrome?

In the case of patent ductus arteriosus and VSD, about 80% occur in infancy, whereas in the case of ASD

over 90% occur in adult life.

What are the complications of Eisenmenger syndrome?

§ Haemoptysis.

§ Right ventricular failure.

§ ·Cerebrovascular accidents (as a result of paradoxical embolization, venous thrombosis of cerebral

vessels, or intracranial haemorrhage).

§ ·Sudden death.

§ ·Brain abscess.

§ ·Bleeding and thrombosis (patients at increased risks for both as a consequence of an abnormal

haemostasis secondary to chronic arterial desaturation).

§ ·Paradoxical embolization.

§ ·Infective endocarditis.

§ ·Hyperuricaemia.

§ ·Recurrent haemoptysis.

How would you investigate this patient?


· Right ventricular hypertrophy.

· Atrial arrhythmias, particularly in those with underlying atrial septal detect.

Chest radiograph

· Conspicuous dilatation of the pulmonary artery with narrowed 'pruned' peri-pheral vessels (due to

pulmonary hypertension).

· Slight to moderate enlargement of the heart (predominantly right ventricle) may be seen in atrial septal

defect, whereas the size of the heart is normal in ventricular septal defect or patent ductus arteriosus.


· Evidence of right ventricular overload and pulmonary hypertension.

· The underlying cardiac defect can be visualized although shunting may be difficult to demonstrate by

colour Doppler imaging because of low-velocity jet.

· Contrast echocardiography permits localization of shunt.

Cardiac catheterization

· To determine the extent and severity of pulmonary vascular disease and to quantify accurately the

magnitude of intracardiac shunting.

· Assessment of reversibility of shunting is done using pulmonary vasodilators (e.g. oxygen, inhaled

nitrous oxide, intravenous adenosine or epoprostenol).

What is the prognosis in these patients?

· Survival is 80% 10 years after diagnosis, 77% at 15 years, and 42% at 25 years.

· Death is usually sudden; other causes include heart failure, haemoptysis, brain abscess or stroke.

· Poor prognostic factors include syncope, clinically evident right ventricular systolic dysfunction, low

cardiac output, and severe hypoxaemia.

Is pregnancy safe in this patient?

Pregnancy is associated with a high incidence of early spontaneous abortion and rarely results in the

birth of a healthy child. Mortality of the mother is high (30-60%) in those with underlying VSD, particularly

in late pregnancy and the postpartum period. Pregnancy is, therefore, contraindicated and if it occurs is

best terminated at an early stage. If pregnancy proceeds to term, a vaginal delivery is the preferred route

with careful management of hydration, arrhythmias and hypoxaemia. Epidural anaesthesia is preferred

over general anaesthesia in complicated cases.

What treatment is available for Eisenmenger syndrome?

· Phlebotomy to avoid symptoms of polycythaemia.

· Long-term intravenous epoprostenol (Circulation 1999; 99(14): 1858-65; Ann Intern Med 1999;

130(9): 740-3).

· Combined heart-lung transplantation (limited success and hence patients should be carefully


Victor Eisenmenger was a German physician who described this condition in an infant in 1897. His

patient had cyanosis since infancy and a fairly good quality of life until he succumbed at the age of 32

years. The patient was active until the age of 29 years when he developed right heart failure and died

three years later following a massive haemoptysis. Post-mortem revealed a large VSD (2.5 cm), both the

left and right ventricles having equally thick walls. The pulmonary arteries revealed atheroma with multiple

thrombi leading to pulmonary infarctions.

Paul Wood, at the Brompton Hospital, in 1958 published a study of 127 patients and was the first to

suggest that Eisenmenger reaction occurred with defects other than at the ventricular level (BMJ 1958; 2.'