Primary systemic amyloidosis with involvement of the heart valued like hypertrophic cardiomyopathy HTML

Authors: S.A. Boldueva, I.V. Yarmosh, N.S. Shvets, V.V. Grokhotova

Company: North-Western State Medical University named after I.I. Mechnikov of Ministry of Health of the Russian Federation, ulitsa Kirochnaya, 41, Saint-Petersburg, 191015, Russian Federation

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Heading: Case reports

UDC: 616.12-003.821:616.127-007.61
DOI: https://doi.org/10.24022/1997-3187-2018-12-3-275-283

For citation: Boldueva S.A., Yarmosh I.V., Shvets N.S., Grokhotova V.V. Primary systemic amyloidosis with involvement of the heart valued like hypertrophic cardiomyopathy. Creative Cardiology. 2018; 12 (3): 275–83 (in Russ.). DOI: 10.24022/1997-3187-2018-12-2-275-283

Received / Accepted:  16.03.2018/19.03.2018

Key Words: AL-amyloidosis heart failure left ventricular hypertrophy myocardial biopsy

Abstract

The article deals with the case of primary systemic amyloidosis with a clinical picture of congestive heart failure refractory to symptomatic therapy. For a long time the patient’s diagnosis was hypertrophic cardiomyopathy due to thickening of the myocardium by echocardiography. The diagnosis of AL-type amyloidosis was verified on the basis of myocardial biopsy using histological and immunohistochemical methods. Despite starting the pathogenetic therapy, the patient died six months later, two years after the first symptoms of the disease. This clinical case attracts attention of physicians to the problem of differential diagnosis of hypertrophic cardiomyopathy, which will provide early diagnosis and pathogenetic treatment of primary amyloidosis.

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About Authors

Chief Editor

Leo A. Bockeria, MD, PhD, DSc, Professor, Academician of Russian Academy of Sciences, Director of Bakoulev National Medical Research Center for Cardiovascular Surgery