Clinical Physiology of Circulation

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


Современные представления о структурно-функциональных и генетических особенностях синдрома некомпактного миокарда левого желудочка

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Link: Clinical Physiology of Blood Circulaiton. 2013; (): -

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Abstract

The left ventricular myocardial noncompaction syndrome is a rare congenital cardiomyopathy, it refers to diseases the etiology of which is not enough studied. The disease affects both children and adults. This genetically heterogeneous congenital cardiomyopathy is observed in the population with a frequency of about 0.05 % and classified as a primary genetic cardiomyopathy according to American Heart Association. Clinical research has confirmed the congenital character of the disease and its mainly autosomal dominant inheritance. Left ventricular noncompaction syndrome is characterized by excessive trabeculation combined with deep inter-trabecular spaces communicating with the left ventricular chamber. The universally recognized reason of the left ventricular noncompaction is a failure of normal myocardial development during embryogenesis. But there still remained many questions regarding this disease which shall substantiate the further research and observation.

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