Myotonic dystrophy type 1 (DM1) is the most common adult form of muscular dystrophy, presenting with a constellation of systemic findings secondary to a CTG triplet expansion of the noncoding region of the DMPK gene. Cardiac involvement is frequent, with conduction disease and supraventricular and ventricular arrhythmias being the most prevalent cardiac manifestations, often developing from a young age. The development of cardiac arrhythmias has been linked to increased morbidity and mortality, with sudden cardiac death well described. Strategies to mitigate risk of arrhythmic death have been developed. In this review, we outline the current knowledge on the pathophysiology of rhythm abnormalities in patients with myotonic dystrophy and summarize available knowledge on arrhythmic risk stratification. We also review management strategies from an electrophysiological perspective, attempting to underline the substantial unmet need to address residual arrhythmic risks for this population.

1 型强直性肌营养不良症 (DM1) 是最常见的成人型肌营养不良症，表现为继发于 DMPK 基因非编码区 CTG 三联体扩增的一系列全身性表现。心脏受累很常见，传导疾病和室上性和室性心律失常是最常见的心脏表现，通常从年轻时开始。心律失常的发展与发病率和死亡率的增加有关，心源性猝死有很好的描述。已经制定了减轻心律失常性死亡风险的策略。在这篇综述中，我们概述了目前关于强直性营养不良患者节律异常的病理生理学知识，并总结了关于心律失常风险分层的现有知识。