Arrhythmias & electrophysiologyCohort study
Sudden cardiac death and major arrhythmic events in patients with Fabry disease: Risk stratification in the French Heart Fabry cohort multicenter study
- retrospective, multicenter study
Authors' conclusion
The incidence of arrhythmic events is high in this FD cohort. A risk prediction for MAE was developed, which may improve preventive therapeutic decisions.
Study context
- Population
- Adult patients with Fabry disease (FD) in the French Heart Fabry cohortadult patients with FD included, 145 patients with follow-up after diagnosis · Follow-up after diagnosis 145
- Participants
- 160 patients
- Exposure
- 0 risk factors vs 1 risk factor vs 2 risk factors
Main finding
As reported in the abstract
Arrhythmic and/or conduction disturbances, predominantly supraventricular arrhythmias · over their lifespan
Patients with arrhythmic and/or conduction disturbances
69 (43%)
Observational data: associations, not effects.
Further findings
Predictors of MAE (multivariate analysis)
| Comparison | hazard ratio (95% CI) | P value | |
|---|---|---|---|
| nonsustained ventricular tachycardia | 9.37 (1.57–56.06) | P = 0.014 | |
| age >55 years | 16.99 (1.98–156) | P = 0.012 | |
| 0.050.21520Hazard ratio (log scale) |
Cumulative incidence
| Outcome | 0 risk factors | 1 risk factor | 2 risk factors | Estimate | P value |
|---|---|---|---|---|---|
| 5-year cumulative incidence of MAE by number of risk factors | 1.8% (95% CI 0.2–6.5) | 7.9% (95% CI 1.7–21.4) | 44.4% (95% CI 13.7–78.8) |
MAE (sudden cardiac death, sustained ventricular arrhythmia, high-degree atrioventricular block, or appropriate implantable cardioverter-defibrillator therapy) · Over 5 years of follow-up after diagnosis
- MAE occurred in 145 patients followed up
- 9 (6.25%)
Model discrimination for predicting MAE
- C-index
- 0.82 (95% CI 0.61-0.96)
- Optimism-corrected C-index after bootstrap internal validation
- 0.81 (95% CI 0.60-1)
- indicating stable discriminative performance with minimal overfitting
Original publication
Sudden cardiac death and major arrhythmic events in patients with Fabry disease: Risk stratification in the French Heart Fabry cohort multicenter study. Heart Rhythm. Published 15 May 2026. doi:10.1016/j.hrthm.2026.05.021
Summarised from the published abstract; every number is checked against it