Implantable Cardioverter-Defibrillators in Previously Undiagnosed Patients with Catecholaminergic Polymorphic Ventricular Tachycardia Resuscitated from Sudden Cardiac Arrest

Sep 2, 2026

Implantable cardioverter-defibrillators (ICDs) in catecholaminergic polymorphic ventricular tachycardia (CPVT) may fail to terminate arrhythmias and can precipitate fatal electrical storms. The study evaluated ICD efficacy and complications in patients who experienced a sentinel sudden cardiac arrest (SCA) event before CPVT was diagnosed. Among 136 SCA patients subsequently diagnosed with CPVT, guideline-directed therapy was initiated, and ICDs were implanted in 79 (58.1%); the primary endpoint was sudden cardiac death.

LanguageEnglish

CategoryGeneral

FormatDOCX

Pages42

Reading Time15 min

AccessFree to download

Abstract

Aims

Methods and Results

Implantable Cardioverter-Defibrillators in Previously Undiagnosed Patients with Catecholaminergic Polymorphic Ventricular Tachycardia Resuscitated from Sudden Cardiac Arrest Christian van der Werf1, *, †, Krystien V. Lieve1, *, †, J. Martijn Bos2, Conor M. Lane2, Isabelle Denjoy3, †, Ferran Roses-Noguer4, Takeshi Aiba5, Yuko Wada6, Jodie Ingles7, Ida S. Leren8, Boris Rudic9, Peter J. Schwartz10, †, Alice Maltret11, Frederic Sacherl2, Jonathan R. Skinner13, Andrew D. Krahn14, Thomas M. Roston14, 15, Jacob Tfelt-Hansen16, Heikki Swan17, Tomas Robyns18, †, Seiko Ohno6, 19, Jason D. Roberts20, Maarten P. van den Berg21, Janneke A. Kammeraad22, Vincent Probst23, †, Prince J. Kannankeril24, Nico A. Blom25, Elijah R. Behr26, †, Martin Borggrefe9, Kristina H. Haugaa8, Christopher Semsarian7, Minoru Horie6, Wataru Shimizu5, 27, Janice A. Till4, Antoine Leenhardt3, †, Michael J. Ackerman2, ‡ and Arthur A. Wilde1, 28, †, ‡ *Drs Van der Werf and Lieve are co-first authors. †European Reference Network ‘ERN GUARD-heart’ ‡Drs. Ackerman and Wilde are co-senior authors.1Amsterdam UMC, University of Amsterdam, Heart Centre; Department of Clinical and Experimental Cardiology, Amsterdam Cardiovascular Sciences, Amsterdam, the Netherlands 2Departments of Cardiovascular Medicine (Division of Heart Rhythm Services), Pediatric and Adolescent Medicine (Division of Pediatric Cardiology), and Molecular Pharmacology & Experimental Therapeutics (Windland Smith Rice Sudden Death Genomics Laboratory), Mayo Clinic, Rochester, MN, United States 3Service de Cardiologie et CNMR Maladies Cardiaques Héréditaires Rares, Hôpital Bichat, Paris, France 4Department of Cardiology, Royal Brompton Hospital, London, United Kingdom 5Division of Arrhythmia and Electrophysiology, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Centre, Suita, Osaka, Japan 6Department of Cardiovascular Medicine, Shiga University of Medical Science, Otsu, Japan 7Agnes Ginges Centre for Molecular Cardiology, Centenary Institute, Sydney, New South Wales, Australia; Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia 8Department of Cardiology, Centre for Cardiological Innovation, Oslo University Hospital, Rikshospitalet, and University of Oslo, Oslo, Norway 9Department of Cardiology, University Medical Centre Mannheim, Mannheim, Germany; German Centre for Cardiovascular Research (DZHK), Partner Site Heidelberg/Mannheim, Mannheim, Germany 10Istituto Auxologico Italiano, IRCCS, Center for Cardiac Arrhythmias of Genetic Origin, Milan, Italy 11Cardiologie Pédiatrique, Hôpital Necker-Enfants-Malades, Paris, France 12LIRYC Institute, Bordeaux University Hospital, Bordeaux University, Bordeaux, France 13Cardiac Inherited Disease Group New Zealand, Green Lane Paediatric and Congenital Cardiac Services, Starship Children's Hospital, Auckland New Zealand; The University of Auckland, Department of Paediatrics Child and Youth Health, Auckland, New Zealand 14Heart Rhythm Services, Division of Cardiology, University of British Columbia, Vancouver, BC, Canada 15BC Children's Hospital and Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada 16Department of Cardiology, Rigshospitalet, Copenhagen, Denmark 17Heart and Lung Centre, Helsinki University Hospital and Helsinki University, Helsinki, Finland 18Department of Cardiovascular Diseases, University Hospitals Leuven, Leuven, Belgium 19Department of Bioscience and Genetics, National Cerebral and Cardiovascular Centre, Suita, Osaka 20Section of Cardiac Electrophysiology, Division of Cardiology, Department of Medicine, Western University, London, Ontario, Canada 21Department of Cardiology, University of Groningen, University Medical Centre Groningen, Groningen, the Netherlands 22Department of Pediatric Cardiology, Sophia Children’s Hospital, Erasmus University Medical Centre, Rot

FAQs

What was the study aim regarding ICD use in CPVT patients?

To assess the efficacy and complications of ICDs in CPVT patients who had sudden cardiac arrest before CPVT diagnosis and treatment.

How many patients were analyzed and how many received an ICD?

136 patients were analyzed, and an ICD was implanted in 79 patients (58.1%).

What treatments were initiated after CPVT diagnosis?

Guideline-directed therapy including beta-blockers, flecainide, and/or left cardiac sympathetic denervation was started.

Sep 2, 2026

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