Cardiac amyloidosis: hemodynamic, echocardiographic and endomyocardial biopsy studies.
Six patients with cardiac amyloidosis (four males, two females; age 27-60 years) were evaluated by us. Four patients presented with congestive heart failure, while one patient each presented with effort angina and giddiness. Extracardiac clues to the diagnosis in the form of involvement of other systems were present in only two patients. The electrocardiogram was abnormal in four patients while three exhibited roentgenographic evidence of cardiomegaly or pulmonary venous hypertension. Echocardiography suggested the diagnosis of amyloidosis in only two patients, restrictive cardiomyopathy in two other patients and dilated and hypertrophic cardiomyopathy in one patient each. Cardiac catheterisation and angiography suggested restrictive heart disease in four patients and hypertrophic cardiomyopathy in one. One patient, whose initial haemodynamic study was normal, had features of dilated cardiomyopathy at repeat study after 11 months. Endomyocardial biopsies showed amyloid deposits in all patients. We emphasise the varied clinical manifestation of cardiac amyloidosis and the need for a high index of suspicion. The diagnosis can be safely and reliably confirmed by endomyocardial biopsy.
Talwar
K K
KK
Department of Cardiology, All India Institute of Medical Sciences, New Delhi.
Kumar
V
V
Agarwal
R
R
Chopra
P
P
Wasir
H S
HS
D000328:Adult; D000686:Amyloidosis / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D001706:Biopsy; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D004452:Echocardiography; D004562:Electrocardiography; D004699:Endocardium / Q000473:pathology; D005260:Female; D006439:Hemodynamics; D006801:Humans; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology