Definition of Eisenmenger’s Syndrome

Pulmonary hypertension with reversal of shunt is called Eisenmenger’s syndrome.

Causes of Eisenmenger’s Syndrome

In these cases, persistently raised pulmonary flow (due to left to right shunt) causes increased pulmonary resistance followed by pulmonary hypertension. This increases the pressure in the right ventricle leading to reversal of shunt (from right to the left side).

Clinical Features


• Dyspnoea.
• Fatigue.
• Syncope.
• Angina.
• Haemoptysis.
• Features of CCF.


• Central cyanosis. But in PDA, differential cyanosis occurs (cyanosis in toes, not in the hands).
• Clubbing. But in PDA, differential clubbing occurs (clubbing in toes, not in the hands).
• Pulse—low volume.
• Prominent ‘a’ wave in JVP.
• Other signs of pulmonary hypertension—palpable P2, left parasternal lift, epigastric pulsation.
• TR may occur.
• Original murmur of VSD, ASD or PDA—decrease in intensity, may disappear.


• Diuretic.
• Digoxin may be given in some cases.
• Venesection may be required, especially if there is polycythaemia.
• Heart-lung transplantation may be done.
• Surgery is contraindicated in Eisenmenger’s syndrome, as it aggravates right-sided heart

Causes of Death

• Right heart failure.
• Infective endocarditis.
• Pulmonary infarction.
• Cerebral thrombosis or abscess.
• Arrhythmia.