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Hypertrophic cardiomyopathy (HCM) is a primary heart
muscle disorder caused, in the majority of cases, by mutations in genes encoding cardiac
sarcomeric proteins1. Its complex and varied
pathophysiology includes myocardial hypertrophy, myocyte disarray, interstitial fibrosis,
myocardial ischaemia, diastolic dysfunction, and abnormal peripheral vascular responses.
Although its most important complication, sudden death, was first recognised by Donald
Teare in the 1950's2, the identification and treatment of
patients at risk of dying suddenly remains an area of controversy.
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