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Haemochromatosis Cardiomyopathy — RACP Adult Medicine MCQ

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HardCardiologyHaemochromatosis CardiomyopathyRACP Adult Medicine

A 55-year-old man with haemochromatosis (C282Y homozygous) who has not been adequately treated presents with new-onset diabetes, heart failure with dilated cardiomyopathy (LVEF 30%), and bronze skin. Cardiac MRI shows severe myocardial iron overload (T2* 5 ms). What is the most urgent treatment?

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Correct answer: D — IV desferrioxamine chelation therapy

Severe cardiac iron overload (T2* <10 ms on cardiac MRI) with cardiomyopathy is a medical emergency in haemochromatosis. IV desferrioxamine continuous infusion (40–60 mg/kg/day) is more effective than phlebotomy for rapid cardiac iron removal and can reverse cardiomyopathy. Phlebotomy alone is too slow for severe cardiac involvement. Concurrent heart failure therapy (ACEi/ARB, beta-blocker, diuretics, SGLT2i) should be commenced. Cardiac function can improve significantly with aggressive chelation, though it takes weeks to months.

Reference: eTG – 2025 – Haematology; EASL – 2010 – Haemochromatosis Guidelines