Arrhythmias & electrophysiology
Atrial Fibrillation and Flutter in a Contemporary Cohort of Patients With Myotonic Muscular Dystrophy
Authors' conclusion
There was a high prevalence of AF/AFL in this single-institution MMD cohort. The CHA2DS2-VASc score may not be an effective tool in this population.
Study context
- Population
- Patients with myotonic muscular dystrophy (MMD1 or MMD2) referred for routine electrophysiology evaluation between January 2013 and September 2023patients seen for new electrophysiology clinic visits for myotonic muscular dystrophy (MMD) cardiac evaluation
- Participants
- 120 patients
- Exposure
- Associations between patient factors and incident atrial fibrillation (AF)/atrial flutter (AFL)
- MMD1n = 96
- MMD2n = 24
- Outcome
- Age at MMD diagnosis
Methodological detailssingle-institution
- Single-institution
Main finding
As reported in the abstract
- Median age at MMD diagnosis (Q1-Q3: 21.8-51.0 years)
- 34.6 years
- Younger age at diagnosis for MMD1 patients
- P < 0.001
Observational data: associations, not effects.
Further findings
Predictors of incident AF/AFL
| Estimate | |
|---|---|
| Female MMD1 patientsLess likely to develop AF/AFL | HR 0.08 |
| Diagnosis of MMD1 at a younger ageAssociated with incident AF/AFL | HR 0.95 per year |
| Greater CTG nucleotide repeatsAssociated with incident AF/AFL | HR 1.09 per 50 repeats |
| Outcome | MMD1 | MMD2 | P value |
|---|---|---|---|
| AF or AFL diagnosed during the study period | 31 of 96 events | 6 of 24 events | P = 0.656 |
- AF or AFL diagnosed during the study period — AF or AFL, all patients
- 31%
AF and AFL
- AF
- 34 (28%)
- AFL
- 9 (8%)
- Both AF and AFL, of the 37 with AF/AFL
- 6 of 37
Anticoagulation (AC) at initial AF/AFL diagnosis
- AC prescribed, of the 37 with AF/AFL
- 41% (n = 15 of 37)
- CHA2DS2-VASc score higher in patients who received AC
- P < 0.001
Catheter ablation
- Underwent catheter ablation (of patients with AF/AFL)
- Twelve (32%)
Safety
Stroke in the AF/AFL group
- Stroke (no AC, CHA2DS2-VASc score 0)
- One
Original publication
Atrial Fibrillation and Flutter in a Contemporary Cohort of Patients With Myotonic Muscular Dystrophy. JACC Clin Electrophysiol. Published 22 January 2025. doi:10.
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