The contribution of channelopathy to the non-ischemic ventricular arrhythmias development

Authors: Bockeria L.A., Serguladze S.Yu., Kotanova E.S.

Company: Bakoulev National Medical Research Center for Cardiovascular Surgery, Moscow, Russian Federation

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Type:  Reviews


DOI: https://doi.org/10.24022/1997-3187-2022-16-1-49-60

For citation: Bockeria L.A., Serguladze S.Yu., Kotanova E.S. The contribution of channelopathy to the non-ischemic ventricular arrhythmias development. Creative Cardiology. 2022; 16 (1): 49–60 (in Russ.). DOI: 10.24022/1997-3187-2022-16-1-49-60

Received / Accepted:  21.11.2021 / 23.03.2022

Keywords: ventricular arrhythmias channelopathy non-ischemic genesis risk stratification

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Abstract

About 40% of patients died suddenly at the age of under 35, have no structural pathology of the heart according to autopsy data. Channelopathies are rare heterogeneous congenital heart rhythm disorders caused by mutations in genes encoding defective ion channel proteins and making up 50% cases of primary non-ischemic cardiomyopathies. Channelopathies include long QT syndrome, short QT syndrome, Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia. These conditions may be detected by genetic testing, which is especially important in asymptomatic persons younger than 40 years. The cardiomyocyte action potential is formed by a certain series of depolarizing and repolarizing ion currents mediated by ion channels. Changes in any of these currents and the appearance of free intracellular calcium make the myocardium vulnerable to polymorphic ventricular tachycardia or ventricular fibrillation. Each of the channelopathies has its own signature of an electrocardiogram, typical forms of manifestation, and a pathognomonically mutating gene. The risk stratification of the channelopathies has specific features, as well as different scales. This review will highlight the mechanisms of development, clinical manifestations and the risk stratification of ventricular arrhythmias associated with cardiac channelopathies.

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About Authors

  • Leo A. Bockeria, Academician of RAS and RAMS, President, ORCID
  • Sergey Yu. Serguladze, Dr. Med. Sci., Senior Researcher, Head of Department, ORCID
  • Evgeniya S. Kotanova, Cand. Med. Sci., Cardiologist, ORCID

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