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Restrictive cardiomyopathy — RACP Paediatrics MCQ

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HardCardiologyRestrictive cardiomyopathyRACP Paediatrics

A 3-year-old presents with exercise intolerance and syncope. Echocardiogram shows a non-dilated left ventricle with severe diastolic dysfunction, biatrial enlargement, and restrictive filling pattern. Systolic function is preserved. What is the most likely type of cardiomyopathy?

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Correct answer: E — Restrictive cardiomyopathy

Restrictive cardiomyopathy is characterised by impaired ventricular filling (diastolic dysfunction) with preserved systolic function, biatrial enlargement, and normal or reduced ventricular volumes. It is the rarest form of cardiomyopathy in children and carries the worst prognosis. Many children require cardiac transplantation.

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