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

Section of Forensic Pathology

26 Citations (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.

Original language	English
Journal	European Heart Journal
Volume	40
Issue number	35
Pages (from-to)	2953–2961
ISSN	0195-668X
DOIs	https://doi.org/10.1093/eurheartj/ehz309
Publication status	Published - 14 Sept 2019

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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.
In: European Heart Journal, Vol. 40, No. 35, 14.09.2019, p. 2953–2961.

Research output: Contribution to journal › Journal article › Research › 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, vol. 40, no. 35, pp. 2953–2961. https://doi.org/10.1093/eurheartj/ehz309

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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}",

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doi = "10.1093/eurheartj/ehz309",

language = "English",

volume = "40",

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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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