Primary cardiac amyloidosis : a case presentation

Przybojewski, J. Z. ; Daniels, A. R. ; Van Der Walt, J. J. (1980-05)

The original publication is available at http://www.samj.org.za

CITATION: Przybojewski, J.Z., Daniels, A.R. & Van der Walt, J.J. 1980. Primary cardiac amyloidosis: a case presentation. South African Medical Journal, 10 May: 774-780.

Article

A young man with primary cardiac amyloidosis presented clinically as a case of 'restrictive' cardiomyopathy with an initial diagnosis of constrictive pericarditis, probably of tuberculous origin. Cardiac catheterization showed features compatible with restrictive cardiomyopathy or constrictive pericarditis but pericardial biopsy was negative. Percutaneous right ventricular endomyocardial biopsies established amyloid infiltration of the myocardium, but rectal, tongue and liver biopsies were all negative for amyloidosis. Benze-Jones protein was absent from the urine. Serum electrophoresis, and bone marrow examination all failed to detect possible multiple myelomatosis as a cause of secondary amyloidosis. Respiratory function tests showed a restrictive picture, probably secondary to chronic congestive cardiac failure. Thus, this case is the second example of primary cardiac amyloidosis in the world literature which has been diagnosed by right ventricular endomyocardial biopsy. Phonocardiographic features of early aortic valve closure are reported for the first time in amyloid restrictive cardiomyopathy. Since tuberculous constrictive pericarditis is an important cause of cardiac disease in this country, with effective therapy, the authors stress more frequent use of endomyocardial biopsy as a safe diagnostic tool to exclude other similar disease states. The patient was offered the possibility of a cardiac transplantation in view of the localized nature of his disease, but refused. He now continues to respond unsatisfactorily to antifailure therapy with, presumably, a poor prognosis.

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