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

88 Citations (Scopus)


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
Number of pages9
JournalEuropean Heart journal
Issue number35
Publication statusPublished - 14 Sept 2019

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