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

Area of Science:

• Cardiology
• Electrophysiology
• Genetics

Background:

• Catecholaminergic polymorphic ventricular tachycardia (CPVT) presents management challenges, particularly for patients with drug-refractory cardiac events (CEs).
• Left cardiac sympathetic denervation (LCSD) is a potential therapeutic option for severe cases.

Purpose of the Study:

• To evaluate the efficacy of LCSD in 162 CPVT patients with drug-refractory CEs.
• To assess LCSD outcomes in a specific subpopulation compliant with medical therapy and those experiencing syncope.

Main Methods:

• Retrospective analysis of 162 CPVT patients (51% female, 80% probands, 79% RYR2 positive) who underwent LCSD.
• Focus on a main subanalysis of 118 patients compliant to therapy, including 41 with syncope on medical therapy.

Main Results:

• Before LCSD, 85% experienced CEs, 52% despite medical therapy, and 27% had prior sudden cardiac arrest (SCA).
• Post-LCSD (median 48 months), 17% experienced CEs, with only 3.6% of the compliant subpopulation having CEs, including 1 sudden cardiac death (SCD).
• LCSD reduced implantable cardioverter-defibrillator (ICD) shocks by 67% and electrical storms by 80%, improving quality of life.

Conclusions:

• LCSD is effective in reducing cardiac events in CPVT patients, including those with syncope on medical therapy.
• Less than 5% of compliant, symptomatic CPVT patients may require an ICD after LCSD.
• Implantable cardioverter-defibrillators (ICDs) may not reliably prevent sudden cardiac death in all CPVT patients.