Noncompaction of the left ventricular myocardium

Authors: E.Z. Golukhova, R.A. Shomakhov

Company: A.N. Bakoulev Scientific Center for Cardiovascular Surgery of Russian Academy of Medical Sciences


For citation: Golukhova EZ., Shomakhov RA. Noncompaction of the left ventricular myocardium. Kreativnaya kardiologiya. 2013; 1: 35-45 (in Russian)

Keywords: noncompaction of the left ventricular myocardium cardiomyopathy non coronary-associated ventricular arrhythmias heart failure

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Abstract

Noncompaction of the left ventricular myocardium (LVN) – is a rare form of cardiomyopathy, which characterizes by the pathological prominent trabeculation of the left ventricular myocardium with formation of two layers of myocardium, i.e. normal and «noncompact ». The disease is genetically determined and results from the failure of myocardial development during embryogenesis. Pathogenesis and clinical features have involved the three leading syndromes: heart failure, arrhythmias, thromboembolic syndrome. Diagnosis is based on echocardiography records and results of magnetic resonance imaging of the heart. The disease has had an unfavorable development and prognosis and more over the mortality in the adult population made up about 50 %. The treatment should be pathogenesis-related and include the therapy for heart failure, cardiac arrhythmias as well as prevention of thromboembolic complications. Heart transplantation is to be recommended for patients with terminal heart failure.

References

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Chief Editor

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