Arrhythmias & electrophysiologyCohort study
Sex Differences in Cardiac Involvement in Adults With Myotonic Dystrophy Type 1
- cohort
- 16 inherited cardiac disease clinics in Spain
Authors' conclusion
In this large DM1 cohort, male sex was associated with a higher cumulative incidence of earlier CCA, device implantantion, and AF, beyond generational/genetic effects. These findings support sex-informed risk stratification.
Study context
- Population
- Adult DM1 patients followed at inherited cardiac disease clinics in SpainDM1 patients analyzed
- Participants
- 549 patients
- Exposure
- males compared to females
- Outcome
- Composite of:
- Lifetime CCA development
- Device implantation
- Major ventricular arrhythmias
- Cardiac syncope
- Follow-up
- Lifetime
Main finding
As reported in the abstract
Subdistribution hazard ratio
1.50
95% CI 1.12–2.00
P = 0.006
males vs females
sHR 1.50 (95% CI 1.12–2.00); P = 0.006
Observational data: associations, not effects.
Secondary outcomes
| Outcome | sHR (95% CI) | P value | |
|---|---|---|---|
| Device implantation · higher cumulative incidence in males | 1.49 (1.04–2.14) | P = 0.029 | |
| AF · higher cumulative incidence in males | 2.19 (1.36–3.53) | P = 0.001 | |
| 0.20.5125Subdistribution hazard ratio (log scale) |
First-degree atrioventricular block
- Device implantation: persisted after generational adjustment (P < 0.05)
- Atrial fibrillation (AF): persisted after generational adjustment (P < 0.05)
- First-degree atrioventricular block: P = 0.037
Second- or third-degree atrioventricular block or QRS ≥120 ms
- no significant difference
Overall survival
- Overall survival was similar between sexes
- P = 0.472
Original publication
Sex Differences in Cardiac Involvement in Adults With Myotonic Dystrophy Type 1. JACC Adv. Published 28 April 2026. doi:10.1016/j.jacadv.2026.102771
Summarised from the published abstract; every number is checked against it