Twenty-eight patients with familial amyloidotic polyneuropathy, but without any symptoms consistent with cardiac dysfunction, were examined by echocardiography. The patients were subdivided into three groups according to the severity of the disease. Only 1 of the patients, with mild neuropathy, had an echocardiogram which was considered normal. The most prominent findings were increased thickness of the heart walls, highly refractile myocardial echoes, and decreased ventricular diastolic motility. The severity of the échocardiographie alterations increased with the severity of the disease. Fourteen patients were reexamined at least 3 years after the initial examination. No symptoms or signs of cardiac dysfunction had developed during the follow-up period. A certain,albeit modest, increase of frequency and extent of the échocardiographie findings occurred during follow-up. It is concluded that echocardiography is of diagnostic value in familial amyloidotic polyneuropathy. Serial examinations help to elucidate the course of the disease, but have limited clinical value.

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