A 5-year-old child with known unrepaired VSD presents with increasing cyanosis and clubbing over the past year. Echocardiography now shows right-to-left shunting across the VSD and severe pulmonary hypertension. PVR/SVR ratio is 0.8. This child's condition is best characterised as:
- A Pulmonary arterial hypertension secondary to VSD
- B Primary pulmonary hypertension
- C Large restrictive VSD with bidirectional shunting
- D Eisenmenger syndrome ✓
Explanation
Eisenmenger syndrome refers to the development of severe, irreversible pulmonary arterial hypertension from initially left-to-right shunts (VSD, ASD, PDA) that causes shunt reversal (right-to-left), resulting in cyanosis. Surgical correction is contraindicated at this stage because the pulmonary vasculature disease is fixed. A PVR/SVR ≥0.5 with cyanosis defines Eisenmenger physiology. Medical therapy with pulmonary vasodilators (bosentan, sildenafil) may improve exercise tolerance but does not cure the condition.
Reference: Ghai Essential Pediatrics, 10th ed.
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Written and medically reviewed by the StethoPrep medical team.