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致心律失常性右室心肌病与运动活动:从病变心脏的分子通路到运动性心脏模拟的新认识。

Arrhythmogenic right ventricular cardiomyopathy and sports activity: from molecular pathways in diseased hearts to new insights into the athletic heart mimicry.

机构信息

Division of Cardiology, University Heart Center Zurich, Rämistrasse 100, CH-8091 Zurich, Switzerland.

Division of Cardiology, Johns Hopkins Hospital, 1800 Orleans St, Baltimore, MD 21287, USA.

出版信息

Eur Heart J. 2021 Mar 31;42(13):1231-1243. doi: 10.1093/eurheartj/ehaa821.

Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease associated with a high risk of sudden cardiac death. Among other factors, physical exercise has been clearly identified as a strong determinant of phenotypic expression of the disease, arrhythmia risk, and disease progression. Because of this, current guidelines advise that individuals with ARVC should not participate in competitive or frequent high-intensity endurance exercise. Exercise-induced electrical and morphological para-physiological remodelling (the so-called 'athlete's heart') may mimic several of the classic features of ARVC. Therefore, the current International Task Force Criteria for disease diagnosis may not perform as well in athletes. Clear adjudication between the two conditions is often a real challenge, with false positives, that may lead to unnecessary treatments, and false negatives, which may leave patients unprotected, both of which are equally inacceptable. This review aims to summarize the molecular interactions caused by physical activity in inducing cardiac structural alterations, and the impact of sports on arrhythmia occurrence and other clinical consequences in patients with ARVC, and help the physicians in setting the two conditions apart.

摘要

致心律失常性右室心肌病(ARVC)是一种与心脏性猝死风险较高相关的遗传性疾病。除其他因素外,体力活动已明确被确定为疾病表型表达、心律失常风险和疾病进展的一个重要决定因素。因此,目前的指南建议 ARVC 患者不应参加竞技或频繁的高强度耐力运动。运动引起的电和形态学的代偿性重塑(所谓的“运动员心脏”)可能模拟 ARVC 的几个典型特征。因此,目前用于疾病诊断的国际工作组标准在运动员中可能表现不佳。两种情况之间的明确判断常常是一个真正的挑战,假阳性可能导致不必要的治疗,假阴性可能使患者得不到保护,这两种情况都是不可接受的。本综述旨在总结体力活动引起心脏结构改变的分子相互作用,以及运动对 ARVC 患者心律失常发生和其他临床后果的影响,帮助医生区分这两种情况。

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