Primary systemic amyloidosis with involvementof the heart valued like hypertrophic cardiomyopathy

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

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

For correspondence:  Sign in or register.

Type:  Case reports


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

Keywords: AL-amyloidosis heart failure left ventricular hypertrophy myocardial biopsy

Полнотекстовая версия:  



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.


  1. Muchtar E., Buadi F.K., Dispenzieri A., Gertz M.A. Immunoglobulin light-chain amyloidosis: from basics to new developments in diagnosis, prognosis and therapy. Acta Haematol. 2016; 135: 172–90. DOI: 10.1159/000443200.
  2. Pinney J.H., Smith C.J., Taube J.B., Lachmann H.J., Venner C.P., Gibbs S.D. еt al. Systemic amyloidosis in England: an epidemiological study. Br. J. Haematol. 2013; 161: 525–32. DOI: 10.1111/bjh.12286.
  3. Wechalekar A.D., Gillmore J.D., Bird J., Cavenagh J., Hawkins S., Kazmi M. еt al. Guidelines on the diagnosis and investigation of AL amyloidosis. Br. J. Haematol. 2015; 168 (2): 207–18. DOI: 10.1111/bjh.13155.
  4. Palladini G., Merlini G. What is new in diagnosis and management of light chain amyloidosis? Blood. 2016; 128 (2): 159–68. DOI: 10.1182/blood-2016-01-629790.
  5. Dubrey S.W., Hawkins P.N., Falk R.H. Amyloid diseases of the heart: assessment, diagnosis, and referral. Heart. 2011; 97: 75–84. DOI:10.1136/hrt.2009.190405.
  6.  Boldueva S.A., Samokhvalova M.V., Bogdanova E.B., Solov'eva T.S. Primary systemic amyloidosis with involvement of the heart and coronary arteries. Kardiologiia. 2010; 6: 92–4. (in Russ.).
  7. Myasnikov R.P., Andreenko E.Yu., Kushunina D.V., Maksimova O.B., Beregovskaya S.A., Mershina E.A. et al. Cardiac amyloidosis: modern aspects of diagnosis and treatment (clinical observation). Clinical and Experimental Surgery. Petrovsky Journal. 2014; 4: 72–82. (in Russ.).
  8. Boldueva S.A., Makhnov A.P., Tret'yakova N.S. Clinical observation of primary systemic amyloidosis with the expressed implications of a heart failure and a hemorrhagic syndrome. Problems of Women Health. 2016; 11 (4): 61–6 (in Russ.).

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