How Many CPVT Patients Need an ICD?: The Impact of Left Cardiac Sympathetic Denervation.

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.