JACC. Clinical electrophysiology · 2026
Pediatric Catecholaminergic Polymorphic Ventricular Tachycardia: Clinical Characteristics, Genetic Spectrum, and Outcomes in a Multicenter Cohort.
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Aydemir Merve Maze, Nalbant Mustafa, Karacan Mehmet, Aydin Ülkü, Tunca Şahin Gülhan, Demirel Başgöze Aylin, Haydin Sertaç, Akkuş Murat, Hanözü Murat, Günlüoğlu Mehmet Zeki, Balik Recep Şiyar, Kafali Hasan Candaş, Akdeniz Celal, Ergül Yakup, Tuzcu Volkan
Аннотация
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.
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.