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DMD – cardiac prophylaxis — RACP Paediatrics MCQ

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ModerateNeurologyDMD – cardiac prophylaxisRACP Paediatrics

A 12-year-old with DMD has lost ambulation. His latest echocardiogram shows normal LV function (EF 60%). When should cardiac pharmacoprophylaxis be initiated?

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Correct answer: A — ACE inhibitor prophylaxis

ACE inhibitors (or ARBs) are recommended for cardiac protection in DMD starting by age 10 years or at the onset of any decline in cardiac function, whichever comes first. Early initiation may delay the onset of cardiomyopathy. Regular echocardiographic surveillance (annually from diagnosis) is essential as cardiomyopathy develops in virtually all DMD patients.

Reference: RCH Melbourne – 2023 – DMD CPG; RACP Paediatric Curriculum – Neurology/Cardiology