BACKGROUND: Currently recognized types of @entity2783 include primary, familial and secondary, each of which may affect the heart. There may be differences in the heart response to the deposition of amyloid fibrils in these three forms of the disease. MATERIALS AND METHODS: Over a period of 10 years (1985-95), 28 consecutive @entity1 with primary, 11 with secondary and 17 with @entity14756 were studied at the Departments of Cardiology of Laiko and Hammersmith Hospitals. The diagnosis of @entity2783 was confirmed by biopsies of subcutaneous fat, rectum, kidney, bone marrow, gum or sural nerve. Diagnosis of cardiac involvement was based on typical electrocardiographic and echocardiographic findings. RESULTS: The left ventricular fractional shortening (%) was reduced in primary compared with familial or secondary @entity2783 (29.8 +/- 10.2 vs. 36.2 +/- 6.5 vs. 36 +/- 5.9, P < 0.05). The transmitral flow velocity pattern was compatible with abnormal relaxation in most @entity1 in the three groups [primary 16 (57%), familial 11 (64. 7%), secondary 6 (54.5%), P = NS]. @entity67 (right ventricular dP/dt < 220 mmHg s-1 or tricuspid annulus systolic excursion < 10 mm) was present in 8 (28.6%), 2 (11. 8%) and 0 @entity1 . @entity1 with @entity12884 were followed up for 15 +/- 6 months. There were 12 deaths, and repeat echocardiography in the survivors revealed a significant deterioration of left ventricular systolic function (fractional shortening = 23.6% +/- 8.8%, P < 0.05 vs. baseline). CONCLUSION: @entity12884 is characterized by more severe cardiac involvement than the familial or secondary @entity2783 and has an ominous course.

Echocardiographic features of primary, secondary and XXXX .

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients'] @entity2783 :: ('MESH:D000686', 'Disease') :: ['amyloidosis'] @entity67 :: ('MESH:D018754', 'Disease') :: ['Right ventricular systolic dysfunction'] @entity12884 :: ('MESH:C531616', 'Disease') :: ['primary amyloidosis', 'Primary amyloidosis'] @entity14756 :: ('MESH:D028226', 'Disease') :: ['familial amyloidosis']
@entity14756 :: (MESH:D028226,Disease) :: ['familial amyloidosis']
