Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest
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Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest. / 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.
I: European Heart Journal, Bind 40, Nr. 35, 14.09.2019, s. 2953–2961.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest
AU - van der Werf, Christian
AU - Lieve, Krystien V
AU - Bos, J Martijn
AU - Lane, Conor M
AU - Denjoy, Isabelle
AU - Roses-Noguer, Ferran
AU - Aiba, Takeshi
AU - Wada, Yuko
AU - Ingles, Jodie
AU - Leren, Ida S
AU - Rudic, Boris
AU - Schwartz, Peter J
AU - Maltret, Alice
AU - Sacher, Frederic
AU - Skinner, Jonathan R
AU - Krahn, Andrew D
AU - Roston, Thomas M
AU - Tfelt-Hansen, Jacob
AU - Swan, Heikki
AU - Robyns, Tomas
AU - Ohno, Seiko
AU - Roberts, Jason D
AU - van den Berg, Maarten P
AU - Kammeraad, Janneke A
AU - Probst, Vincent
AU - Kannankeril, Prince J
AU - Blom, Nico A
AU - Behr, Elijah R
AU - Borggrefe, Martin
AU - Haugaa, Kristina H
AU - Semsarian, Christopher
AU - Horie, Minoru
AU - Shimizu, Wataru
AU - Till, Janice A
AU - Leenhardt, Antoine
AU - Ackerman, Michael J
AU - Wilde, Arthur A
N1 - Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2019. For permissions, please email: journals.permissions@oup.com.
PY - 2019/9/14
Y1 - 2019/9/14
N2 - 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.
AB - 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.
U2 - 10.1093/eurheartj/ehz309
DO - 10.1093/eurheartj/ehz309
M3 - Journal article
C2 - 31145795
VL - 40
SP - 2953
EP - 2961
JO - European Heart Journal
JF - European Heart Journal
SN - 0195-668X
IS - 35
ER -
ID: 225424816