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Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy

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Sex Differences in Cardiac Involvement in Adults With Myotonic Dystrophy Type 1: A Multicenter Study.

Alberto Alen¹, Carmen Muñoz², Marc Soriano-Amores³

¹Área del Corazón, Hospital Universitario Central Asturias, Oviedo, Spain.

|May 27, 2026

Summary

Males with myotonic dystrophy type 1 (DM1) experience more cardiac conduction abnormalities (CCAs) and atrial fibrillation (AF) than females. These sex differences in DM1 cardiac outcomes persist even after accounting for generational effects.

Keywords:

ICD arrythmia conduction abnormalities myotonic dystrophy pacemakers sex differences

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Area of Science:

Cardiology
Genetics
Neuromuscular Disorders

Background:

Myotonic dystrophy type 1 (DM1) causes progressive cardiac conduction abnormalities (CCAs) and arrhythmias.
While genetic anticipation and CTG repeat length impact DM1 severity, sex-based differences in cardiac involvement are not well understood.

Purpose of the Study:

To investigate sex differences in cardiac outcomes among adult DM1 patients.
To determine if these sex differences persist after adjusting for generational effects.

Main Methods:

Analysis of clinical data from 549 adult DM1 patients across 16 Spanish inherited cardiac disease clinics.
Primary endpoint: composite of CCA, device implantation, ventricular arrhythmias, and cardiac syncope.
Secondary endpoints: overall survival, atrial fibrillation (AF), and device implantation; birth cohort used as a proxy for genetic burden.

Main Results:

Males showed a higher cumulative incidence of the primary endpoint (sHR: 1.50), device implantation (sHR: 1.49), and AF (sHR: 2.19) compared to females.
Males more frequently presented with first-degree atrioventricular block; no significant sex difference in more severe AV block.
Sex disparities in cardiac outcomes persisted after generational adjustment (P < 0.05).

Conclusions:

Male sex is linked to increased risk of earlier CCAs, device implantation, and AF in DM1 patients, independent of generational/genetic factors.
Findings support the need for sex-informed risk stratification in DM1 management.