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

Christian van der Werf, Krystien V. Lieve, J. Martijn Bos, Conor M. Lane, Isabelle Denjoy, Ferran Roses-Noguer, Takeshi Aiba, Yuko Wada, Jodie Ingles, Ida S. Leren, Boris Rudic, Peter J. Schwartz, Alice Maltret, Frederic Sacher, Jonathan R. Skinner, Andrew D. Krahn, Thomas M. Roston, Jacob Tfelt-Hansen, Heikki Swan, Tomas RobynsSeiko Ohno, Jason D. Roberts, Maarten P. van den Berg, Janneke A. Kammeraad, Vincent Probst, Prince J. Kannankeril, Nico A. Blom, Elijah R. Behr, Martin Borggrefe, Kristina H. Haugaa, Christopher Semsarian, Minoru Horie, Wataru Shimizu, Janice A. Till, Antoine Leenhardt, Michael J. Ackerman, Arthur A. Wilde

Research output: Contribution to journalArticleAcademicpeer-review


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.
Original languageEnglish
Pages (from-to)2953-2961
JournalEuropean Heart Journal
Issue number35
Publication statusPublished - 14 Sep 2019

Cite this