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 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

doi:10.1093/eurheartj/ehz309

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

Afdeling for Retspatologi

26 Citationer (Scopus)

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.

Originalsprog	Engelsk
Tidsskrift	European Heart Journal
Vol/bind	40
Udgave nummer	35
Sider (fra-til)	2953–2961
ISSN	0195-668X
DOI	https://doi.org/10.1093/eurheartj/ehz309
Status	Udgivet - 14 sep. 2019

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10.1093/eurheartj/ehz309

Citationsformater

van der Werf, C., Lieve, K. V., Bos, J. M., Lane, C. M., Denjoy, I., Roses-Noguer, F., Aiba, T., Wada, Y., Ingles, J., Leren, I. S., Rudic, B., Schwartz, P. J., Maltret, A., Sacher, F., Skinner, J. R., Krahn, A. D., Roston, T. M., Tfelt-Hansen, J., Swan, H., ... Wilde, A. A. (2019). Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest. European Heart Journal, 40(35), 2953–2961. https://doi.org/10.1093/eurheartj/ehz309

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 et al.
I: European Heart Journal, Bind 40, Nr. 35, 14.09.2019, s. 2953–2961.

Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › peer review

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', European Heart Journal, bind 40, nr. 35, s. 2953–2961. https://doi.org/10.1093/eurheartj/ehz309

@article{150da5b7316e45cf864540c8a12744c6,

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

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.",

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

year = "2019",

month = sep,

day = "14",

doi = "10.1093/eurheartj/ehz309",

language = "English",

volume = "40",

pages = "2953–2961",

journal = "European Heart Journal",

issn = "0195-668X",

publisher = "Oxford University Press",

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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

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

SN - 0195-668X

VL - 40

SP - 2953

EP - 2961

JO - European Heart Journal

JF - European Heart Journal

IS - 35

ER -

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

Abstract

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