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

van der Werf, C; Lieve, KV; Bos, JM; Lane, CM; Denjoy, I; Roses-Noguer, F; Aiba, T; Wada, Y; Ingles, J; Leren, IS; et al. van der Werf, C; Lieve, KV; Bos, JM; Lane, CM; Denjoy, I; Roses-Noguer, F; Aiba, T; Wada, Y; Ingles, J; Leren, IS; Rudic, B; Schwartz, PJ; Maltret, A; Sacher, F; Skinner, JR; Krahn, AD; Roston, TM; Tfelt-Hansen, J; Swan, H; Robyns, T; Ohno, S; Roberts, JD; van den Berg, MP; Kammeraad, JA; Probst, V; Kannankeril, PJ; Blom, NA; Behr, ER; Borggrefe, M; Haugaa, KH; Semsarian, C; Horie, M; Shimizu, W; Till, JA; Leenhardt, A; Ackerman, MJ; Wilde, AA (2019) Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest. Eur Heart J, 40 (35). pp. 2953-2961. ISSN 1522-9645 https://doi.org/10.1093/eurheartj/ehz309
SGUL Authors: Behr, Elijah Raphael

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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.

Item Type: Article
Additional Information: This is a pre-copyedited, author-produced version of an article accepted for publication in European Heart Journal following peer review. The version of record 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 Robyns, Seiko 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, Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest, European Heart Journal, Volume 40, Issue 35, 14 September 2019, Pages 2953–2961 is available online at: https://doi.org/10.1093/eurheartj/ehz309
Keywords: Catecholaminergic polymorphic ventricular tachycardia, Implantable cardioverter-defibrillator, Secondary prevention, Sudden cardiac arrest, Sudden cardiac death, Catecholaminergic polymorphic ventricular tachycardia, Implantable cardioverter-defibrillator, Secondary prevention, Sudden cardiac arrest, Sudden cardiac death, 1102 Cardiovascular Medicine And Haematology, Cardiovascular System & Hematology
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Journal or Publication Title: Eur Heart J
ISSN: 1522-9645
Language: eng
Dates:
DateEvent
14 September 2019Published
30 May 2019Published Online
26 April 2019Accepted
Publisher License: Publisher's own licence
Projects:
Project IDFunderFunder ID
113304045ZonMW Priority Medicines for Rare Diseases and Orphan DrugsUNSPECIFIED
1059156National Health and Medical Research Councilhttp://dx.doi.org/10.13039/501100000925
PubMed ID: 31145795
Go to PubMed abstract
URI: https://openaccess.sgul.ac.uk/id/eprint/110953
Publisher's version: https://doi.org/10.1093/eurheartj/ehz309

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