Background: The management of catecholaminergic polymorphic ventricular tachycardia (CPVT) patients with drug refractory cardiac events (CEs) is challenging. Objectives: This study sought to assess the efficacy of left cardiac sympathetic denervation (LCSD) in 162 CPVT patients, focusing on those symptomatic without a high-risk genotype and compliant to therapy (main subanalysis, n = 118) of whom 41 had syncope on medical therapy. CEs included syncope, sudden cardiac arrest (SCA), sudden cardiac death (SCD), and appropriate implantable cardioverter-defibrillator (ICD) interventions. Methods: A retrospective study including 162 CPVT patients (51% female, 80% probands, 79% RYR2 positive) who underwent LCSD worldwide. Results: Most (n = 139; 85%) of the 162 patients experienced ≥1 CE before LCSD, 84 (52%) had CEs despite medical therapy, and 43 (27%) had previous SCA. Overall, 93% received a beta-blocker (nonselective in 85%), 53% both a beta-blocker and a class I antiarrhythmic drug, and 55% (89) had an ICD before LCSD. During a median of 48 months (Q1-Q3: 12-111 months) after LCSD, 28 of 162 patients (17%) had ≥1 CE, including 10 of 28 (36%) during noncompliance. Of the 118 patients (main subanalysis), 13% suffered CEs after LCSD, including 3 SCAs and 1 SCD despite an ICD (3%). Of the 41 with syncope on medical therapy, 6 (15%) experienced CEs after LCSD, including the SCD despite an ICD (3%). LCSD improved quality of life by reducing ICD shocks by 67% and electrical storms by 80%. Conclusions: 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.
How Many CPVT Patients Need an ICD?
Dusi, VeronicaCo-first
;De Ferrari, Gaetano M.;
2026-01-01
Abstract
Background: The management of catecholaminergic polymorphic ventricular tachycardia (CPVT) patients with drug refractory cardiac events (CEs) is challenging. Objectives: This study sought to assess the efficacy of left cardiac sympathetic denervation (LCSD) in 162 CPVT patients, focusing on those symptomatic without a high-risk genotype and compliant to therapy (main subanalysis, n = 118) of whom 41 had syncope on medical therapy. CEs included syncope, sudden cardiac arrest (SCA), sudden cardiac death (SCD), and appropriate implantable cardioverter-defibrillator (ICD) interventions. Methods: A retrospective study including 162 CPVT patients (51% female, 80% probands, 79% RYR2 positive) who underwent LCSD worldwide. Results: Most (n = 139; 85%) of the 162 patients experienced ≥1 CE before LCSD, 84 (52%) had CEs despite medical therapy, and 43 (27%) had previous SCA. Overall, 93% received a beta-blocker (nonselective in 85%), 53% both a beta-blocker and a class I antiarrhythmic drug, and 55% (89) had an ICD before LCSD. During a median of 48 months (Q1-Q3: 12-111 months) after LCSD, 28 of 162 patients (17%) had ≥1 CE, including 10 of 28 (36%) during noncompliance. Of the 118 patients (main subanalysis), 13% suffered CEs after LCSD, including 3 SCAs and 1 SCD despite an ICD (3%). Of the 41 with syncope on medical therapy, 6 (15%) experienced CEs after LCSD, including the SCD despite an ICD (3%). LCSD improved quality of life by reducing ICD shocks by 67% and electrical storms by 80%. Conclusions: 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.
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