Arrhythmias & electrophysiology
Pediatric Catecholaminergic Polymorphic Ventricular Tachycardia: Clinical Characteristics, Genetic Spectrum, and Outcomes in a Multicenter Cohort
Authors' conclusion
LCSD/BCSD was associated with a substantial reduction in arrhythmic burden, but observational treatment allocation and center-specific management preclude conclusions regarding comparative effectiveness. Sustained treatment adherence remains essential.
Study context
- Population
- Pediatric patients with catecholaminergic polymorphic ventricular tachycardia (CPVT), 2012–2025pediatric patients with CPVT
- Participants
- 92 patients
- Exposure
- Paired preprocedural and postprocedural event rates
- Left cardiac sympathetic denervation (LCSD)n = 25
- Bilateral cardiac sympathetic denervation (BCSD)n = 25
- Outcome
- Median arrhythmic event rate, before LCSD/BCSD vs during the first 2 postprocedural years (paired cohort)
- Follow-up
- First 2 postprocedural years
Methodological details3 tertiary centers in Türkiye
- 3 tertiary centers in Türkiye
Main finding
As reported in the abstract
P < 0.001
Observational data: associations, not effects.
Further findings
First 2-year event rates, LCSD vs BCSD · first 2 years
- Did not differ significantly between procedures
Age at symptom onset and diagnosis
Years
- Median age at symptom onset
- 9.25 (IQR: 5.88-12.0)
- Median age at diagnosis
- 11.6 (IQR: 8.64-14.41)
Genetic testing
- Genetically tested patients
- 87
- Pathogenic or likely pathogenic variant, of 87 tested
- 74 (85.1%)
- Ryanodine receptor 2 variants, of 87 tested
- 41 (47.1%)
- Calsequestrin 2 variants, of 87 tested
- 24 (27.6%)
Sympathetic denervation
- Patients who underwent sympathetic denervation
- Fifty
Safety
- Patients died
- Seven
- Documented treatment nonadherence present in all deaths
Original publication
Pediatric Catecholaminergic Polymorphic Ventricular Tachycardia: Clinical Characteristics, Genetic Spectrum, and Outcomes in a Multicenter Cohort. JACC Clin Electrophysiol. Published 22 September 2026. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →