Primary amyloid heart disease presenting as hypertrophic obstructive cardiomyopathy.
This report describes the unusual presentation of a patient with primary cardiac amyloidosis. Initial clinical symptoms and hemodynamic studies, including Technetium-99m-pyrophosphate scintigraphy, suggested hypertrophic obstructive cardiomyopathy, but endomyocardial biopsy revealed diffuse amyloid infiltration. Only two other cases of left ventricular outflow tract obstruction due to cardiac amyloidosis have been reported. The false-negative technetium-99m-pyrophosphate scintigram in this patient argues for the use of endomyocardial biopsy to aid in the diagnosis of left ventricular hypertrophy.
D000686:Amyloidosis / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D001706:Biopsy; D006328:Cardiac Catheterization; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis* / Q000000981:diagnostic imaging; D003937:Diagnosis, Differential; D011756:Diphosphates; D004452:Echocardiography; D004699:Endocardium / Q000473:pathology; D005260:Female; D006801:Humans; D008875:Middle Aged; D011877:Radionuclide Imaging; D013667:Technetium; D016698:Technetium Tc 99m Pyrophosphate
D011756:Diphosphates; D016698:Technetium Tc 99m Pyrophosphate; D013667:Technetium