An international multicenter cohort study on implantable cardioverter-defibrillators for the treatment of symptomatic children with catecholaminergic polymorphic ventricular tachycardia

Avani Lamba; Thomas M Roston; Puck J Peltenburg; Dania Kallas; Sonia Franciosi; Krystien V V Lieve; Prince J Kannankeril; Minoru Horie; Seiko Ohno; Ramon Brugada; Takeshi Aiba; Peter Fischbach; Linda Knight; Jan Till; Sit-Yee Kwok; Vincent Probst; David Backhoff; Martin J LaPage; Anjan S Batra; Fabrizio Drago; Kristina Haugaa; Andrew D Krahn; Tomas Robyns; Heikki Swan; Terezia Tavacova; Joseph Atallah; Martin Borggrefe; Boris Rudic; Georgia Sarquella-Brugada; Ehud Chorin; Allison Hill; Janneke Kammeraad; Anna Kamp; Ian Law; James Perry; Jason D Roberts; Svjetlana Tisma-Dupanovic; Christopher Semsarian; Jonathan R Skinner; Jacob Tfelt-Hansen; Isabelle Denjoy; Antoine Leenhardt; Peter J Schwartz; Michael J Ackerman; Arthur A M Wilde; Christian van der Werf; Shubhayan Sanatani

doi:10.1016/j.hrthm.2024.04.006

An international multicenter cohort study on implantable cardioverter-defibrillators for the treatment of symptomatic children with catecholaminergic polymorphic ventricular tachycardia

Heart Rhythm. 2024 Apr 7:S1547-5271(24)02323-3. doi: 10.1016/j.hrthm.2024.04.006. Online ahead of print.

Authors

Avani Lamba¹, Thomas M Roston², Puck J Peltenburg³, Dania Kallas¹, Sonia Franciosi¹, Krystien V V Lieve³, Prince J Kannankeril⁴, Minoru Horie⁵, Seiko Ohno⁶, Ramon Brugada⁷, Takeshi Aiba⁸, Peter Fischbach⁹, Linda Knight⁹, Jan Till¹⁰, Sit-Yee Kwok¹¹, Vincent Probst¹², David Backhoff¹³, Martin J LaPage¹⁴, Anjan S Batra¹⁵, Fabrizio Drago¹⁶, Kristina Haugaa¹⁷, Andrew D Krahn¹⁸, Tomas Robyns¹⁹, Heikki Swan²⁰, Terezia Tavacova²¹, Joseph Atallah²², Martin Borggrefe²³, Boris Rudic²⁴, Georgia Sarquella-Brugada²⁴, Ehud Chorin²⁵, Allison Hill²⁶, Janneke Kammeraad²⁷, Anna Kamp²⁸, Ian Law²⁹, James Perry³⁰, Jason D Roberts³¹, Svjetlana Tisma-Dupanovic³², Christopher Semsarian³³, Jonathan R Skinner³⁴, Jacob Tfelt-Hansen³⁵, Isabelle Denjoy³⁶, Antoine Leenhardt³⁶, Peter J Schwartz³⁷, Michael J Ackerman³⁸, Arthur A M Wilde³, Christian van der Werf³, Shubhayan Sanatani³⁹

Affiliations

¹ BC Children's Hospital, Division of Cardiology, Department of Pediatrics, The University of British Columbia, Vancouver, British Columbia, Canada.
² BC Children's Hospital, Division of Cardiology, Department of Pediatrics, The University of British Columbia, Vancouver, British Columbia, Canada; Center for Cardiovascular Innovation, Division of Cardiology, The University of British Columbia, Vancouver, British Columbia, Canada.
³ Amsterdam UMC, University of Amsterdam, Heart Centre; Department of Clinical and Experimental Cardiology, Amsterdam Cardiovascular Sciences, Heart Failure and Arrhythmias, Amsterdam, The Netherlands.
⁴ Department of Pediatrics, Monroe Carell Jr Children's Hospital at Vanderbilt, Vanderbilt University Medical Centre, Nashville, Tennessee.
⁵ Department of Cardiovascular Medicine, Shiga University of Medical Science, Otsu, Japan.
⁶ Department of Cardiovascular Medicine, Shiga University of Medical Science, Otsu, Japan; Department of Bioscience and Genetics, National Cerebral and Cardiovascular Centre, Suita, Japan.
⁷ Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain, Cardiovascular Genetics Center, Institut d'Investigació Biomèdica Girona (IDIBGI), University of Girona, Girona, Spain, Medical Science Department, School of Medicine, University of Girona, Girona, Spain, Cardiology Service, Hospital Josep Trueta, Girona, Spain.
⁸ Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Centre, Suita, Japan.
⁹ Sibley Heart Center, Children's Healthcare of Atlanta, Atlanta, Georgia.
¹⁰ Department of Cardiology, Royal Brompton Hospital, London, United Kingdom.
¹¹ Hong Kong Children's Hospital, Hong Kong, SAR, China.
¹² Université de Nantes, CHU Nantes, CNRS, INSERM, l'institut du thorax, Nantes, France.
¹³ University of Gottingen, Gottingen, Germany.
¹⁴ University of Michigan, Ann Arbor, Michigan.
¹⁵ Division of Cardiology, Department of Pediatrics, Irvine and Children's Hospital of Orange County, University of California, Orange, California.
¹⁶ Pediatric Cardiology and Cardiac Arrhythmias Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital, IRCCS, Palidoro-Rome, Italy.
¹⁷ ProCardio Center for Innovation, Department of Cardiology, Oslo University Hospital, Rikshospitalet, Oslo, Norway, Department of Cardiology, Karolinska University Hospital, Solna, Sweden, Department of Medicine, Huddinge, Karolinska Institute, Stockholm, Sweden.
¹⁸ Center for Cardiovascular Innovation, Division of Cardiology, The University of British Columbia, Vancouver, British Columbia, Canada.
¹⁹ Department of Cardiovascular Diseases, University Hospitals Leuven, Leuven, Belgium, Department of Cardiovascular Sciences, University of Leuven, Leuven, Belgium.
²⁰ Heart and Lung Centre, Helsinki University Hospital and Helsinki University, Helsinki, Finland.
²¹ Department of Pediatric Cardiology, Children's Heart Centre, Second Faculty of Medicine, Charles University in Prague; Motol University Hospital, Prague, Czech Republic.
²² Cardiology, Faculty of Medicine & Dentistry - Pediatrics Department, Stollery Children's Hospital, Edmonton, Alberta, Canada.
²³ Department of Medicine, University Medical Center Mannheim, Mannheim, Germany, German Center for Cardiovascular Research (DZHK), Partner Site Heidelberg/Mannheim, Heidelberg, Germany.
²⁴ Pediatric Arrhythmias, Inherited Cardiac Diseases and Sudden Death Unit, Hospital Sant Joan de Déu, Barcelona, Spain, Medical Science Department, School of Medicine, Universitat de Girona, Girona, Spain.
²⁵ Department of Cardiology, Tel Aviv Sourasky Medical Center and Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
²⁶ Children's Hospital Los Angeles, Los Angeles, California.
²⁷ Université de Nantes, CHU Nantes, CNRS, INSERM, l'institut du thorax, Nantes, France; Department of Pediatric Cardiology, Erasmus MC Sophia, Rotterdam, The Netherlands.
²⁸ Nationwide Children's Hospital, Columbus, Ohio.
²⁹ University of Iowa Stead Family Children's Hospital, Iowa City, Iowa.
³⁰ Rady Children's Hospital, San Diego, California.
³¹ Section of Cardiac Electrophysiology, Division of Cardiology, Department of Medicine, Western University, London, Ontario, Canada, Population Health Research Institute, Hamilton Health Sciences, and McMaster University, Hamilton, Ontario, Canada.
³² Nemours Children's Clinic, Orlando, Florida.
³³ Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney, Sydney, New South Wales, Australia, Faculty of Medicine and Health, The University of Sydney, Sydney, New South Wales, Australia.
³⁴ Cardiac Inherited Disease Group New Zealand, Green Lane Paediatric and Congenital Cardiac Services, Starship Children's Hospital, Auckland, New Zealand, Department of Paediatrics Child and Youth Health, The University of Auckland, Auckland, New Zealand.
³⁵ Department of Cardiology, Rigshospitalet, Copenhagen, Denmark, Department of Forensic Medicine, Faculty of Medical Sciences, University of Copenhagen, Copenhagen, Denmark.
³⁶ Service de Cardiologie et CNMR Maladies Cardiaques Héréditaires Rares, Hôpital Bichat, APHP, Université de Paris Cité, Paris, France.
³⁷ Istituto Auxologico Italiano, IRCCS, Center for Cardiac Arrhythmias of Genetic Origin, Milan, Italy.
³⁸ Departments of Cardiovascular Medicine, Pediatric and Adolescent Medicine, and Molecular Pharmacology & Experimental Therapeutics; Division of Heart Rhythm Services and Pediatric Cardiology, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, Minnesota.
³⁹ BC Children's Hospital, Division of Cardiology, Department of Pediatrics, The University of British Columbia, Vancouver, British Columbia, Canada. Electronic address: ssanatani@cw.bc.ca.

PMID: 38588993
DOI: 10.1016/j.hrthm.2024.04.006

Abstract

Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) may cause sudden cardiac death (SCD) despite medical therapy. Therefore, implantable cardioverter-defibrillators (ICDs) are commonly advised. However, there are limited data on the outcomes of ICD use in children.

Objective: The purpose of this study was to compare the risk of arrhythmic events in pediatric patients with CPVT with and without ICD.

Methods: We compared the risk of SCD in patients with RYR2 (ryanodine receptor 2) variants and phenotype-positive symptomatic patients with CPVT with and without ICD who were younger than 19 years and had no history of sudden cardiac arrest at phenotype diagnosis. The primary outcome was SCD; secondary outcomes were composite end points of SCD, sudden cardiac arrest, or appropriate ICD shocks with or without arrhythmic syncope.

Results: The study included 235 patients, 73 with ICD (31.1%) and 162 without ICD (68.9%). Over a median follow-up of 8.0 years (interquartile range 4.3-13.4 years), SCD occurred in 7 patients (3.0%), of whom 4 (57.1%) were noncompliant with medications and none had an ICD. Patients with ICD had a higher risk of both secondary composite outcomes (without syncope: hazard ratio 5.85; 95% confidence interval 3.40-10.09; P < .0001; with syncope: hazard ratio 2.55; 95% confidence interval 1.50-4.34; P = .0005). Thirty-one patients with ICD (42.5%) experienced appropriate shocks, 18 (24.7%) inappropriate shocks, and 21 (28.8%) device-related complications.

Conclusion: SCD events occurred only in the no ICD group and in those not on optimal medical therapy. Patients with ICD had a high risk of appropriate and inappropriate shocks, which may be reduced with appropriate device programming. Severe ICD complications were common, and risks vs benefits of ICDs need to be considered.

Keywords: Catecholaminergic polymorphic ventricular tachycardia; Implantable cardioverter-defibrillator; Inherited arrhythmia; Ryanodine receptor; Sudden cardiac death; Ventricular tachycardia.