An overview of heart rhythm disorders and management in myotonic dystrophy type 1.

Myotonic dystrophy type 1 (DM1) is the commonest adult form of muscular dystrophy, presenting with a constellation of systemic findings secondary to a CTG triplet expansion of the non-coding region of the DMPK gene. Cardiac involvement is frequent, with conduction disease, 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 aim to outline the current knowledge on the pathophysiology of rhythm abnormalities in DM patients 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.