Arrhythmias & electrophysiologyDiagnostic or prognostic study
Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2: a validated risk stratification
- Prognostic model development and validation study
- derivation and independent external validation cohorts
- Cox regression
Authors' conclusion
These externally validated risk prediction models using clinical parameters accurately distinguished CPVT patients on β-blocker monotherapy at low and high risk for future AEs while treated with β-blockers.
Study context
- Population
- Patients with RYR2-mediated catecholaminergic polymorphic ventricular tachycardia (CPVT) on β-blocker monotherapy
- Participants
- 743 vs 129
- Comparison
- Derivation vs Validation
- Derivationn = 743
- Validationn = 129
- Outcome
- Arrhythmic events (AEs) (arrhythmic syncope, appropriate ICD shock, sudden cardiac arrest, SCD)
Performance
As reported in the abstract
Model discrimination for AE (optimism-corrected C-index); predictors: arrhythmic syncope or sudden cardiac arrest (SCA) before diagnosis, age at β-blocker initiation
| Test or model | Value | C-index for AE | Median follow-up |
|---|---|---|---|
| Derivation | 13.7% | 0.67 (95% CI 0.62-0.72) | 5.1 (IQR 7.7) years |
| Validation | 18.6% | 0.59 (95% CI 0.48-0.71) | 2.4 (IQR 4.4) years |
Further findings
| Outcome | Derivation | Validation |
|---|---|---|
| Near-fatal or fatal AEs (all AEs except arrhythmic syncope); fourth predictor: ventricular arrhythmia severity before β-blocker initiation | ||
| C-index | 0.74 (95% CI 0.68-0.80) | 0.60 (95% CI 0.47-0.72) |
- Calibration slope (derivation cohort)
- 1.00 (95% CI 0.59-1.41) · AE · nf/fAE 1.00 (95% CI 0.69-1.32)
Original publication
Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2: a validated risk stratification. Eur Heart J. Published 19 December 2025. doi:10.1093/eurheartj/ehaf965
Summarised from the published abstract; every number is checked against it