National Centre for Sports Cardiology, St Vincent's Hospital, Melbourne, Vic, Australia; Department of Medicine, University of Melbourne at St Vincent's Hospital (Melbourne), Melbourne, Vic, Australia.
National Centre for Sports Cardiology, St Vincent's Hospital, Melbourne, Vic, Australia; Baker Heart & Diabetes Institute, Melbourne, Vic, Australia.
Heart Lung Circ. 2020 Apr;29(4):547-555. doi: 10.1016/j.hlc.2019.12.007. Epub 2019 Dec 26.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a group of cardiomyopathies associated with ventricular arrhythmias predominantly arising from the right ventricle, sudden cardiac death and right ventricular failure, caused largely due to inherited mutations in proteins of the desmosomal complex. Whilst long recognised as a cause of sudden cardiac death (SCD) during exercise, it has recently been recognised that intense and prolonged exercise can worsen the disease resulting in earlier and more severe phenotypic expression. Changes in cardiac structure and function as a result of exercise training also pose challenges with diagnosis as enlargement of the right ventricle is commonly seen in endurance athletes. Advice regarding restriction of exercise is an important part of patient management, not only of those with established disease, but also in individuals known to carry gene mutations associated with development of ARVC.
致心律失常性右心室心肌病（ARVC）是一组与室性心律失常相关的心肌病，主要起源于右心室，可导致心源性猝死和右心衰竭，主要由桥粒蛋白复合物的基因突变引起。尽管 ARVC 早已被认为是运动时心源性猝死（SCD）的原因，但最近人们认识到，剧烈和长时间的运动可能会使病情恶化，导致更早和更严重的表型表达。运动训练导致的心脏结构和功能的变化也给诊断带来了挑战，因为耐力运动员的右心室增大很常见。关于限制运动的建议是患者管理的重要组成部分，不仅针对已确诊疾病的患者，而且针对已知携带与 ARVC 发展相关基因突变的个体。
