AKT · Child health/Congenital heartlow yield

Eisenmenger syndrome

Reversal of a long-standing left-to-right shunt to right-to-left (pulmonary hypertension)

Overview

The end-stage of a large uncorrected left-to-right shunt (VSD, ASD, PDA, AVSD): chronic pulmonary overcirculation causes irreversible pulmonary hypertension until pulmonary pressure exceeds systemic, REVERSING the shunt to right-to-left → CYANOSIS. Once established it is irreversible and contraindicates shunt closure; managed with pulmonary vasodilators and ultimately heart-lung transplant. Pregnancy is very high-risk.

Recognise

Late CYANOSIS, clubbing, polycythaemia (secondary), reduced exercise tolerance in someone with a previously acyanotic shunt
Signs of pulmonary hypertension (loud P2, right heart failure); haemoptysis, arrhythmia, paradoxical emboli
Develops from an uncorrected/late-corrected large VSD/ASD/PDA/AVSD

Red flags

Established Eisenmenger CONTRAINDICATES shunt closure (the shunt is now offloading the right heart) — the window was missed
Pregnancy carries very high maternal mortality; avoid

Differentials & how to tell them apart

Cyanotic CHD (primary)Eisenmenger is acquired from a long-standing acyanotic shunt that reversed

Other pulmonary hypertensioncontext of a pre-existing shunt

Investigations

Echocardiography + right heart catheterisation (pulmonary pressures, reversed shunt); oxygen saturations; FBC (secondary polycythaemia).

Management

Pulmonary vasodilators + supportive care; heart-lung transplant (do NOT close the shunt)

1Recognise late cyanosis in someone with an uncorrected shunt; confirm pulmonary hypertension/reversed shunt. Specialist pulmonary hypertension therapy; supportive care.Gate: Once Eisenmenger is established, the shunt must NOT be closed (it is now decompressing the right heart) — the lesson is to close large shunts EARLY before this happens; pregnancy is extremely high-risk
2Pulmonary vasodilators; manage complications (polycythaemia, arrhythmia, haemoptysis); heart-lung transplant; contraception/pregnancy counselling.

Pulmonary vasodilators (e.g. bosentan, sildenafil) — specialist pulmonary hypertension therapy

Supportive (avoid dehydration/venesection unless indicated); transplant — heart-lung transplant is the definitive option

Key points

Late cyanosis in a previously acyanotic shunt = Eisenmenger (shunt reversed from pulmonary hypertension). Irreversible — don't close the shunt; the teaching point is closing large shunts early. Pregnancy is dangerous.

Monitor & prognosis

Pulmonary pressures, saturations, complications.

Progressive; transplant for end-stage.

Source: RCPCH; pulmonary hypertension guidelines