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Eisenmenger syndrome — RACP Paediatrics MCQ

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ModerateCardiologyEisenmenger syndromeRACP Paediatrics

A 15-year-old with an unrepaired large VSD presents with cyanosis, clubbing, and erythrocytosis (Hb 210 g/L). Echocardiography shows right-to-left shunting across the VSD with suprasystemic RV pressures. What is the most likely diagnosis?

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Correct answer: C — Eisenmenger syndrome

Long-standing left-to-right shunting from an unrepaired VSD causing irreversible pulmonary vascular disease with reversal to right-to-left shunting (cyanosis) is Eisenmenger syndrome. At this stage, the VSD is inoperable (closure would cause RV failure). Management is with pulmonary vasodilators, avoidance of dehydration, and eventual heart-lung transplantation.

Reference: RCH Melbourne – 2023 – Pulmonary Hypertension CPG; RACP Paediatric Curriculum – Cardiology