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Burnout HCM — EECC MCQ

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HardCardiomyopathyBurnout HCMEECC

A 58-year-old man with HCM and LVEF 48% (reduced from 65% three years ago) develops progressive breathlessness. CMR shows extensive LGE involving 25% of the LV myocardium with LV cavity dilatation. What phase of HCM does this represent?

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Correct answer: C — HCM with systolic dysfunction (burnout/end-stage HCM) — characterised by progressive LV thinning, cavity dilatation, declining LVEF, and extensive fibrosis; associated with poor prognosis

Approximately 5-10% of HCM patients develop a 'burned-out' or end-stage phase characterised by: LV wall thinning (regression of hypertrophy), LV cavity dilatation, progressive systolic dysfunction (LVEF <50%), and extensive myocardial fibrosis (often >15-20% LGE by CMR). This phase mimics DCM and carries a poor prognosis with high rates of HF hospitalisation, arrhythmias, and death. The 2023 ESC Cardiomyopathy Guidelines recognise this as a distinct phenotypic evolution requiring: (1) standard HFrEF GDMT; (2) ICD assessment (high SCD risk from extensive scar); (3) transplant evaluation if refractory. Mavacamten is not indicated in the burnout phase (it further reduces contractility and is contraindicated when LVEF <55%).

Reference: ESC (2023): Guidelines on Cardiomyopathies