Heart failure & cardiomyopathiesCohort study
Emery–Dreifuss muscular dystrophy Type 1 is associated with a high risk of malignant ventricular arrhythmias and end-stage heart failure
- Retrospective cohort study
- multicentre
- 12 international cardiomyopathy units
Authors' conclusion
Male EMD variant-carriers have a risk of progressive heart failure and ventricular arrhythmias similar to that of male LMNA variant-carriers.
Study context
- Population
- Consecutively referred EMD variant-carriers (Emery-Dreifuss muscular dystrophy type 1)38 males · 21 females
- Exposure
- EMDCARDIAC vs LMNACARDIAC
- Outcome
- Malignant ventricular arrhythmia (MVA) and end-stage HF (ESHF) incidence
Main finding
As reported in the abstract
MVA and ESHF incidence · median follow-up 65.0 months
| Group | Malignant ventricular arrhythmia (MVA) | End-stage heart failure (ESHF) |
|---|---|---|
| EMDCARDIAC | 4.8 per 100 person-years | 2.4 per 100 person-years |
| LMNACARDIAC | 6.6 per 100 person-years | 5.9 per 100 person-years |
- EMDCARDIAC vs LMNACARDIAC: log-rank P = .49 — MVA
- EMDCARDIAC vs LMNACARDIAC: log-rank P = .09 — ESHF
- Male EMD variant-carriers who developed MVA
- Nine (23.7%)
- Male EMD variant-carriers who developed ESHF
- five (13.2%)
Observational data: associations, not effects.
Further findings
MVA incidence rate · log-rank; similar
P = 0.49
ESHF incidence rate · log-rank
P = 0.09
- Cardiac phenotype in female EMD variant-carriers: Female EMD variant-carriers 42.8%
Original publication
Emery–Dreifuss muscular dystrophy Type 1 is associated with a high risk of malignant ventricular arrhythmias and end-stage heart failure. Eur Heart J. Published 28 August 2023. doi:10.1093/eurheartj/ehad561
Summarised from the published abstract; every number is checked against it