Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest

Eur Heart J. 2019 Sep 14;40(35):2953-2961. doi: 10.1093/eurheartj/ehz309.


Aims: In patients with catecholaminergic polymorphic ventricular tachycardia (CPVT), implantable cardioverter-defibrillator (ICD) shocks are sometimes ineffective and may even trigger fatal electrical storms. We assessed the efficacy and complications of ICDs placed in patients with CPVT who presented with a sentinel event of sudden cardiac arrest (SCA) while undiagnosed and therefore untreated.

Methods and results: We analysed 136 patients who presented with SCA and in whom CPVT was diagnosed subsequently, leading to the initiation of guideline-directed therapy, including β-blockers, flecainide, and/or left cardiac sympathetic denervation. An ICD was implanted in 79 patients (58.1%). The primary outcome of the study was sudden cardiac death (SCD). The secondary outcomes were composite outcomes of SCD, SCA, appropriate ICD shocks, and syncope. After a median follow-up of 4.8 years, SCD had occurred in three patients (3.8%) with an ICD and none of the patients without an ICD (P = 0.1). SCD, SCA, or appropriate ICD shocks occurred in 37 patients (46.8%) with an ICD and 9 patients (15.8%) without an ICD (P < 0.0001). Inappropriate ICD shocks occurred in 19 patients (24.7%) and other device-related complications in 22 patients (28.9%).

Conclusion: In previously undiagnosed patients with CPVT who presented with SCA, an ICD was not associated with improved survival. Instead, the ICD was associated with both a high rate of appropriate ICD shocks and inappropriate ICD shocks along with other device-related complications. Strict adherence to guideline-directed therapy without an ICD may provide adequate protection in these patients without all the potential disadvantages of an ICD.

Keywords: Catecholaminergic polymorphic ventricular tachycardia; Implantable cardioverter-defibrillator; Secondary prevention; Sudden cardiac arrest; Sudden cardiac death.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cardiopulmonary Resuscitation*
  • Death, Sudden, Cardiac / prevention & control*
  • Defibrillators, Implantable* / adverse effects
  • Electrocardiography
  • Follow-Up Studies
  • Guideline Adherence
  • Risk Factors
  • Tachycardia, Ventricular / diagnosis*
  • Tachycardia, Ventricular / therapy*
  • Treatment Outcome

Supplementary concepts

  • Polymorphic catecholergic ventricular tachycardia