Abstract

The cardiovascular system was examined in 19 cases of familial amyloid polyneuropathy. In a group of patients with neurological involvement, various cardiac abnormalities were common, including orthostatic hypotension, prominent apex cardiographic A waves, abnormal apical systolic waves (bulges), systolic murmurs, mid-systolic clicks, QS waves, atrioventricular block, left bundlebranch block, and abnormalities of ejection time and pre-ejection period. Though there was one case with pronounced cardiac abnormality despite a normal neurological state, and though cardiovascular symptoms appeared later than neurological symptoms, the degree of cardiac involvement generally paralleled the severity of the neurological disorder.