Echocardiographic features in familial amyloidosis with polyneuropathy.
Twenty-two patients with the Swedish variant of familial amyloidosis with polyneuropathy were studied by M-mode and two-dimensional echocardiography. These patients had few symptoms consistent with cardiac disease but, nevertheless, echocardiograms of only two of them, both with a short history of the disease, were considered normal in all aspects. The most common abnormality was increased thickness of the interventricular septum found in 20 (91%) of the patients. This septal hypertrophy was asymmetric in 12 (55%) of them. Two-dimensional echocardiography revealed a characteristic hyperrefractile appearance of the myocardium in 15 (68%) of the patients. Thus, echocardiography showed a characteristic pattern in these patients. This study also indicates that cardiac amyloidosis can be diagnosed in the preclinical, asymptomatic state by M-mode and two-dimensional echocardiography.
D000328:Adult; D000368:Aged; D000686:Amyloidosis / Q000175:diagnosis* / Q000235:genetics; D004452:Echocardiography; D005260:Female; D006331:Heart Diseases / Q000175:diagnosis; D006801:Humans; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis* / Q000235:genetics