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Eur Heart J. 2019 May 30. pii: ehz309. doi: 10.1093/eurheartj/ehz309. [Epub ahead of print]

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

Author information

1
Amsterdam UMC, University of Amsterdam, Heart Centre, and Department of Clinical and Experimental Cardiology, Amsterdam Cardiovascular Sciences, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.
2
Division of Heart Rhythm Services, Department of Cardiovascular Medicine, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.
3
Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.
4
Department of Molecular Pharmacology and Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.
5
Service de Cardiologie et CNMR Maladies Cardiaques Héréditaires Rares, Hôpital Bichat, 46 Rue Henri Huchard, 75877 Paris, France.
6
Department of Cardiology, Royal Brompton Hospital, Sydney St, Chelsea, London SW3 6NP, UK.
7
Division of Arrhythmia and Electrophysiology, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Centre, 5 Chome-7-1 Fujishirodai, Suita, Osaka 565-0873, Japan.
8
Department of Cardiovascular Medicine, Shiga University of Medical Science, Seta Tsukinowacho, 520-2192, Otsu, Japan.
9
Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney, Locked Bag 6, Newtown NSW 2042, Sydney, Australia.
10
Faculty of Medicine and Health, The University of Sydney, Locked Bag 6, Newtown NSW 2042, Sydney, Australia.
11
Department of Cardiology, Royal Prince Alfred Hospital, Locked Bag 6, Newtown NSW 2042, Sydney, Australia.
12
Department of Cardiology, Centre for Cardiological Innovation, Oslo University Hospital, Rikshospitalet, Sognsvannsveien 20, 0372 Oslo, Norway.
13
Department of Cardiology, University Medical Centre Mannheim, Theodor-Kutzer-Ufer 1 - 3, 68167 Mannheim, Germany.
14
German Centre for Cardiovascular Research (DZHK), Partner Site Heidelberg/Mannheim, Mannheim, Germany.
15
Centre for Cardiac Arrhythmias of Genetic Origin, IRCCS Istituto Auxologico Italiano, Via Mosè Bianchi, 90, 20149 Milan, Italy.
16
Hôpital Necker-Enfants-Malades, Cardiologie Pédiatrique, 149 Rue de Sèvres, 75015 Paris, France.
17
LIRYC Institute, Bordeaux University Hospital, Bordeaux University, Avenue du Haut Lévêque, 33600 Pessac- Bordeaux, France.
18
Cardiac Inherited Disease Group New Zealand, Green Lane Paediatric and Congenital Cardiac Services, Starship Children's Hospital, 2 Park Rd, Grafton, Auckland 1023 New Zealand.
19
Department of Paediatrics Child and Youth Health, The University of Auckland, Auckland, New Zealand.
20
Division of Cardiology, Heart Rhythm Services, University of British Columbia, 1033 Davie Street, Vancouver V6E 1M7, BC, Canada.
21
BC Children's Hospital, 4480 Oak St, Vancouver, BC V6H 3N1, Canada.
22
Department of Pediatrics, University of British Columbia, 4480 Oak Street, Vancouver, BC V6H 3V4, Canada.
23
Department of Cardiology, Rigshospitalet, Blegdamsvej 9, 2100 Copenhagen, Denmark.
24
Heart and Lung Centre, Helsinki University Hospital and Helsinki University, Tukholmankatu 8 A 00290 Helsinki, Finland.
25
Department of Cardiovascular Diseases, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium.
26
Department of Bioscience and Genetics, National Cerebral and Cardiovascular Centre, 5 Chome-7-1 Fujishirodai, Suita, Osaka 565-0873, Japan.
27
Section of Cardiac Electrophysiology, Division of Cardiology, Department of Medicine, Western University, 339 Windermere Road, B6-129B, London, ON N6A 5A5, Canada.
28
Department of Cardiology, University of Groningen, University Medical Centre Groningen, Hanzeplein 1, 9713 GZ Groningen, The Netherlands.
29
Department of Pediatric Cardiology, Sophia Children's Hospital, Erasmus University Medical Centre, Doctor Molewaterplein 40, 3015 GD Rotterdam, The Netherlands.
30
L'Institut du Thorax, Cardiologic Department and Reference Center for Hereditary Arrhythmic Diseases INSERM 1087, Boulevard Monod, Nantes, France.
31
Department of Pediatrics, Monroe Carell Jr Children's Hospital at Vanderbilt, Vanderbilt University Medical Centre, 2200 Children's Way, Nashville, TN 37232, USA.
32
Department of Pediatric Cardiology, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.
33
Department of Pediatric Cardiology, Willem-Alexander Children's Hospital, Leiden University Medical Centre, Albinusdreef 2, 2333 ZA Leiden, The Netherlands.
34
Molecular and Clinical Sciences Research Institute, St. George's, University of London, Cranmer Terrace, London SW17 0RE, UK.
35
Cardiology Clinical Academic Group, St. George's University Hospitals NHS Foundation Trust, Cranmer Terrace, London SW17 0RE, UK.
36
Department of Cardiovascular Medicine, Nippon Medical School, 1-1-5, Sendagi, Bunkyo-ku, Tokyo, Japan.
37
Brahim Centre of Excellence in Research of Hereditary Disorders, Princess Al-Jawhara Al, 7393 Al-Malae'b St, King Abdul Aziz University, Jeddah 22252, Kingdom of Saudi Arabia.

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.

KEYWORDS:

Catecholaminergic polymorphic ventricular tachycardia; Implantable cardioverter-defibrillator; Secondary prevention; Sudden cardiac arrest; Sudden cardiac death

PMID:
31145795
DOI:
10.1093/eurheartj/ehz309

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