Arrhythmias & electrophysiology
Sudden cardiac death and major arrhythmic events in patients with Fabry disease: Risk stratification in the French Heart Fabry cohort 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)followed for 5 years after diagnosis, 160 adult patients included (mean age 41.0 years, 47% male)
- Participants
- 145 patientsEvents: 9 (6.25%)
- Predictors
- Nonsustained ventricular tachycardia and age >55 years
- Predicted outcome
- Major arrhythmic events (MAEs): sudden cardiac death, sustained ventricular arrhythmia, high-degree atrioventricular block, or appropriate implantable cardioverter-defibrillator therapy
Methodological detailsFrench Heart Fabry cohort, bootstrap internal validation
- French Heart Fabry cohort
- Bootstrap internal validation
Model performance
As reported in the abstract
C-index
Model for predicting MAE
0.82(95% CI 0.61-0.96)
Optimism-corrected after bootstrap internal validation
0.81(95% CI 0.60-1)
Further findings
Predictors of MAE (multivariate analysis)
| 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
0 risk factors
1.8%(95% CI 0.2–6.5)
1 risk factor
7.9%(95% CI 1.7–21.4)
2 risk factors
44.4%(95% CI 13.7–78.8)
MAE, internal validation
- Stable discriminative performance with minimal overfitting
Arrhythmic and/or conduction disturbances over lifespan
- Patients with arrhythmic and/or conduction disturbances, predominantly supraventricular arrhythmias
- 69 (43%)
- Of 160 included patients
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.
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