Arrhythmias & electrophysiology

An international multicenter cohort study on implantable cardioverter-defibrillators for the treatment of symptomatic children with catecholaminergic polymorphic ventricular tachycardia

Heart RhythmPublished International multicentre cohort study

Authors' conclusion

SCD events occurred only in patients without an ICD and mostly in those not on optimal medical therapy.

Study context

Population
Symptomatic children (<19 years) with catecholaminergic polymorphic ventricular tachycardia (CPVT) (RYR2 variants, phenotype-positive) without history of sudden cardiac arrest at phenotype diagnosis
Participants
235 children
Exposure
ICD vs no ICD
  • ICDn = 73
  • No ICDn = 162reference
Outcome
SCD
Follow-up
Median 8.0 years (IQR 4.3-13.4 years)

Main finding

As reported in the abstract

median follow-up 8.0 years

ICD

0

events

Sudden cardiac death (SCD) occurred overall in 7 patients
3.0%
Of SCD cases, noncompliant with medications
4 (57.1%)

Observational data: associations, not effects.

Secondary outcomes

OutcomeHR (95% CI)P value
Composite SCD, sudden cardiac arrest or appropriate ICD shocks (without arrhythmic syncope)5.85 (3.40–10.09)P < 0.0001
Composite SCD, sudden cardiac arrest or appropriate ICD shocks (with arrhythmic syncope)2.55 (1.50–4.34)P = 0.0005

Safety

ICD shocks and device-related complications
31 (42.5%) · Appropriate shocks · Inappropriate shocks 18 (24.7%) · Device-related complications 21 (28.8%)

Original publication

An international multicenter cohort study on implantable cardioverter-defibrillators for the treatment of symptomatic children with catecholaminergic polymorphic ventricular tachycardia. Heart Rhythm. Published 7 April 2024. doi:10.1016/j.hrthm.2024.04.006

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