Pediatric Catecholaminergic Polymorphic Ventricular Tachycardia

BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited arrhythmia with a high risk of life-threatening ventricular arrhythmias in childhood. OBJECTIVES: This study sought to characterize pediatric CPVT and evaluate contemporary therapeutic outcomes, including left cardiac sympathetic denervation (LCSD) and bilateral cardiac sympathetic denervation (BCSD). METHODS: This retrospective multicenter study included 92 pediatric patients with CPVT from 3 tertiary centers in Türkiye between 2012 and 2025. Arrhythmic events included aborted cardiac arrest, sudden cardiac death, appropriate implantable cardioverter-defibrillator shocks, arrhythmic syncope, and ventricular tachycardia. Paired preprocedural and postprocedural event rates were compared in patients with an adequate preprocedural observation period. RESULTS: Median age at symptom onset was 9.25 years (IQR: 5.88-12.0), and median age at diagnosis was 11.6 years (IQR: 8.64-14.41). Among 87 genetically tested patients, 74 (85.1%) had a pathogenic or likely pathogenic variant; ryanodine receptor 2 and calsequestrin 2 variants were identified in 41 (47.1%) and 24 (27.6%), respectively. Fifty patients underwent sympathetic denervation (LCSD, n = 25; BCSD, n = 25). In the paired cohort (n = 35), median arrhythmic event rate decreased from 3.20 events/year (IQR: 1.74-8.76) before LCSD/BCSD to 0.00 events/year (IQR: 0.00-1.35) during the first 2 postprocedural years (P < 0.001). First 2-year event rates did not differ significantly between procedures. Seven patients died; documented treatment nonadherence was present in all. CONCLUSIONS: 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.