Arrhythmias & electrophysiologyCohort study

Pediatric Catecholaminergic Polymorphic Ventricular Tachycardia

JACC Clin ElectrophysiolPublished

  • retrospective multicenter study
  • 3 tertiary centers in Türkiye

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

Main finding

As reported in the abstract

Median arrhythmic event rate, before LCSD/BCSD vs during the first 2 postprocedural years (paired cohort) · first 2 postprocedural years

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

Deaths

Patients died
Seven
Documented treatment nonadherence present in all deaths

Original publication

Pediatric Catecholaminergic Polymorphic Ventricular Tachycardia. JACC Clin Electrophysiol. Published 22 September 2026. doi:10.1016/j.jacep.2026.08.020

Summarised from the published abstract; every number is checked against it