Eisenmenger syndrome
Eisenmenger syndrome is the end stage of a large uncorrected left-to-right cardiac shunt, in which chronic pulmonary overcirculation causes irreversible pulmonary vascular disease and shunt reversal to right-to-left with cyanosis. The defect can no longer be closed; management is specialized supportive care, pulmonary vasodilators, and avoidance of destabilizing insults.
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What this article covers
- Overview
Definition: Eisenmenger syndrome is the development of irreversible pulmonary vascular disease and pulmonary hypertension from a large chronic left-to-right cardiac shunt, causing the shunt to reverse to right-to-left with cyanosis.
- Epidemiology
Now less common where congenital shunts are repaired early.
- Risk factors
Large uncorrected left-to-right shunt (VSD, ASD, PDA, or complex disease).
- Pathophysiology
Chronic pulmonary overcirculation: a large left-to-right shunt exposes the pulmonary vasculature to high flow and pressure.
- Clinical features
Central cyanosis, clubbing, and exertional dyspnea.
- Differential diagnosis
Idiopathic pulmonary arterial hypertension: severe pulmonary hypertension with a structurally normal heart and no intracardiac shunt on echocardiography, the absence of an underlying congenital defect being the discriminator.
- Diagnosis
Establish by right-to-left (or bidirectional) shunting with severe pulmonary hypertension in a patient with a congenital shunt: the diagnostic basis is echocardiography demonstrating the shunt with…
- Management
First-line, specialized supportive care and avoidance of destabilizing insults: manage in an adult congenital heart disease center, avoid dehydration, systemic vasodilation, high altitude, and…
- Complications
Progressive right heart failure. Hyperviscosity, thrombosis, and paradoxical embolism, and bleeding (including hemoptysis).
- Prognosis
A serious, life-limiting condition, though survival into adulthood is common with care.
- Prevention
Timely closure of large shunts before pulmonary vascular disease develops (the key preventive measure).
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