Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest. (30th May 2019)

• Record Type: Journal Article
• Authors: van der Werf, Christian; Lieve, Krystien V; Bos, J Martijn; Lane, Conor M; Denjoy, Isabelle; Roses-Noguer, Ferran; Aiba, Takeshi; Wada, Yuko; Ingles, Jodie; Leren, Ida S; Rudic, Boris; Schwartz, Peter J; Maltret, Alice; Sacher, Frederic; Skinner, Jonathan R; Krahn, Andrew D; Roston, Thomas M; Tfelt-Hansen, Jacob; Swan, Heikki; Robyns, Tomas; Ohno, Seiko; Roberts, Jason D; van den Berg, Maarten P; Kammeraad, Janneke A; Probst, Vincent; Kannankeril, Prince J; Blom, Nico A; Behr, Elijah R; Borggrefe, Martin; Haugaa, Kristina H; Semsarian, Christopher; Horie, Minoru; Shimizu, Wataru; Till, Janice A; Leenhardt, Antoine; Ackerman, Michael J; Wilde, Arthur A;
• Is Part Of: European heart journal. Volume 40:Number 35(2019)
• Page Start: 2953
• Page End: 2961
• Publication Date: 2019-05-30
• Subjects: Catecholaminergic polymorphic ventricular tachycardia -- Implantable cardioverter-defibrillator -- Secondary prevention -- Sudden cardiac arrest -- Sudden cardiac death; Cardiology -- Periodicals; Heart -- Diseases -- Periodicals; 616.12005
• DOI: 10.1093/eurheartj/ehz309
• Languages: English
• ISSNs: 0195-668X

Abstract

Abstract: 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.