Hypertrophic cardiomyopathy is a primary disorder of the myocardium characterised by disproportionate hypertrophy of the ventricular wall. It is the most common genetic cardiac disease with an incidence of 1 in 500 and it is diagnosed most commonly using transthoracic echocardiography.

This review article discusses: the diagnosis of hypertrophic cardiomyopathy; the differential diagnoses; the characteristic histological signs found at postmortem and/or myectomy and the clinical symptoms and signs.

Current recommendations for myectomy of first degree relatives, based on the ACC/ESC guidelines, are discussed as well as general management and then specific management for various subgroups and symptomatic patients.