Arrhythmias & electrophysiologyCohort study

How Many Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) Patients Need an Implantable Cardioverter-defibrillator (ICD)?

JACC Clin ElectrophysiolPublished

  • retrospective study
  • worldwide

Authors' conclusion

Our data suggest that probably <5% of symptomatic CPVT patients compliant to medical therapy require an ICD after LCSD. ICDs do not reliably prevent SCD.

Study context

Population
CPVT patients who underwent left cardiac sympathetic denervation (LCSD) (51% female, 80% probands, 79% RYR2 positive)CPVT patients who underwent LCSD, main subanalysis n = 118 (symptomatic without a high-risk genotype and compliant to therapy), of whom 41 had syncope on medical therapy · main subanalysis 118 · syncope on medical therapy (of the 118) 41
Participants
162 patients

Main finding

As reported in the abstract

≥1 CE after LCSD · median of 48 months (Q1-Q3: 12-111 months) after LCSD

LCSD

17%

28 of 162 of 162

of the 28 with cardiac events (CEs), during noncompliance
10 of 28 (36%)

Observational data: associations, not effects.

Further findings

CEs after LCSD, main subanalysis (n = 118)

CEs after LCSD among the 118
13%
3 sudden cardiac arrest (SCAs) and 1 sudden cardiac death (SCD) despite an ICD among the 118
3%

CEs after LCSD, those with syncope on medical therapy (n = 41)

CEs after LCSD among the 41
6 (15%)
including the SCD despite an ICD
3%

ICD shocks and electrical storms

reduction in ICD shocks
67%
reduction in electrical storms
80%

Baseline before LCSD

≥1 CE before LCSD
n = 139; 85%
CEs despite medical therapy
84 (52%)
previous SCA
43 (27%)
received a beta-blocker
93%
beta-blocker nonselective
85%
both a beta-blocker and a class I antiarrhythmic drug
53%
ICD before LCSD
55% (89)

Original publication

How Many CPVT Patients Need an ICD? JACC Clin Electrophysiol. Published 14 May 2026. doi:10.1016/j.jacep.2026.03.027

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