Arrhythmias & electrophysiology
Long-Term Arrhythmic Follow-Up and Risk Stratification of Patients With Desmoplakin-Associated Arrhythmogenic Right Ventricular Cardiomyopathy
Authors' conclusion
DSP-TFC+ patients are at substantial risk for VAs. The ARVC risk calculator performs poorly in DSP-TFC+ patients suggesting need for a gene-specific risk algorithm.
Study context
- Population
- Patients with likely pathogenic/pathogenic desmoplakin (DSP) variants fulfilling definite 2010 arrhythmogenic right ventricular cardiomyopathy (ARVC) Task Force Criteria (DSP-TFC+)
- Participants
- 252 patients
- Outcome
- Ventricular arrhythmias (composite of sustained ventricular tachycardia (VT), appropriate ICD therapies, VF/SCD events in follow-up)
Methodological details20 institutions across 3 continents
- 20 institutions across 3 continents
Main finding
As reported in the abstract
Ventricular arrhythmias · 44.5 [IQR: 19.6-78.3] months
DSP-TFC+
37.3%
94 events
Observational data: associations, not effects.
Further findings
| Outcome | Values | aHR | P value |
|---|---|---|---|
| Predictors of VA during first 5-year follow-up · strongest association | 2.097 | P = 0.004 | |
| Incident VA in patients without VA at diagnosis (n = 204) | No VA at diagnosis 32.8% · Annual rate 7.37%/y |
- Predictors of ventricular arrhythmias (VA) during first 5-year follow-up — Age not associated with VA
- P = 0.723
- Predictors of VA during first 5-year follow-up — Male sex not associated with VA
- P = 0.200
VA risk with LV involvement (n = 194)
- Higher VA risk with LV involvement
- log-rank P = 0.0239
ARVC risk calculator performance for VA prediction
- C-statistic overall
- 0.604 [0.594-0.614]
- C-statistic with LV disease
- 0.558 [0.556-0.560]
Original publication
Long-Term Arrhythmic Follow-Up and Risk Stratification of Patients With Desmoplakin-Associated Arrhythmogenic Right Ventricular Cardiomyopathy. JACC Adv. Published 2 February 2024. doi:10.
Summarised from the published abstract; every number is checked against it