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An International Multicenter Cohort Study on Implantable Cardioverter Defibrillators for the Treatment of Symptomatic Children with Catecholaminergic Polymorphic Ventricular Tachycardia
Journal article   Peer reviewed

An International Multicenter Cohort Study on Implantable Cardioverter Defibrillators for the Treatment of Symptomatic Children with Catecholaminergic Polymorphic Ventricular Tachycardia

Avani Lamba, Thomas M. Roston, Puck J. Peltenburg, Dania Kallas, Sonia Franciosi, Krystien V.V. Lieve, Prince J. Kannankeril, Minoru Horie, Seiko Ohno, Ramon Brugada, …
Heart rhythm, Vol.21(10), pp.1767-1776
10/2024
DOI: 10.1016/j.hrthm.2024.04.006
PMID: 38588993
url
https://lirias.kuleuven.be/retrieve/85e05526-ea35-4ef7-9b0a-78aadfd5602fView
Open Access

Abstract

Catecholaminergic polymorphic ventricular tachycardia (CPVT) may cause sudden cardiac death (SCD) despite medical therapy. Therefore, implantable cardioverter defibrillators (ICDs) are commonly advised. However, there are limited data on the outcomes of ICD use in children. To compare the risk of arrhythmic events in pediatric CPVT patients with and without ICDs. We compared the risk of SCD in RYR2 variant and phenotype positive symptomatic CPVT patients with and without ICDs, who were <19 years of age and had no history of sudden cardiac arrest (SCA) at phenotype diagnosis. The primary outcome was SCD; secondary outcomes were composite incidences of SCD, SCA, appropriate ICD shocks, with/without arrhythmic syncope. The study included 235 patients, 73 (31.1%) with ICDs and 162 (68.9%) without ICDs. Over a median follow-up of 8.0 years (IQR 4.3-13.4), SCD occurred in 7 (3.0%) patients, of which 4 (57.1%) were non-compliant with medications and none had an ICD. ICD patients had a higher risk of both secondary composite outcomes (without syncope: HR 5.85 (CI 3.40-10.09); p<0.0001; with syncope: HR 2.55 (CI 1.50-4.34); p=0.0005). Thirty-one (42.5%) patients with an ICD experienced appropriate shocks, 18 (24.7%) inappropriate shocks, and 21 (28.8%) device-related complications. SCD events occurred only in the no ICD group, in those not on optimal medical therapy. ICD patients had a high risk of appropriate and inappropriate shocks, which may be reduced with appropriate device programming. Severe ICD complications were common and risks versus benefits of ICDs need to be considered.
 Catecholaminergic Polymorphic Ventricular Tachycardia Implantable cardioverter defibrillator Inherited arrhythmia Ryanodine Receptor Sudden cardiac death Ventricular tachycardia

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