Hemodynamic and angiocardiographic observations in familial amyloidosis with polyneuropahy.

Eleven patients with familial amyloidosis with polyneuropathy were studied by heart catheterization; eight of them also by right and left ventriculography and selective coronary angiography. Signs of congestive heart failure, often reported as typical of cardiac amyloidosis, were found in only one patient. The systolic function was relatively unimpaired, but in several patients hemodynamic data suggested restrictive cardiomyopathy. Six patients showed signs of right or left ventricular outflow tract obstruction. Thus, no uniform hemodynamic pattern could be identified, but the changes found indicate impaired diastolic function.