A 34 year old man presented with an inferior non-Q-wave myocardial infarction. Echocardiography showed a bicuspid aortic valve with aortic outflow obstruction. Left coronary cusp morphology was normal but the right coronary cusp was grossly distorted and replaced by a mobile echodense mass encroaching upon the aortic valve orifice. The aortic valve was replaced and pathological analysis of the excised valve showed primary amyloid infiltration of the right coronary cusp but a normal left coronary cusp. The mass adherent to the right coronary leaflet had the histological appearances of organised thrombus and this was assumed to be the source of coronary embolism. This is the first reported case of primary valvar amyloid presenting with clinical sequelae and it illustrates the need for careful clinical assessment in young patients presenting with acute ischaemic syndromes.
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