Christian van der Werf1, Krystien V Lieve1, J Martijn Bos2,3,4, Conor M Lane2,3,4, Isabelle Denjoy5, Ferran Roses-Noguer6, Takeshi Aiba7, Yuko Wada8, Jodie Ingles9,10,11, Ida S Leren12, Boris Rudic13,14, Peter J Schwartz15, Alice Maltret16, Frederic Sacher17, Jonathan R Skinner18,19, Andrew D Krahn20, Thomas M Roston20,21,22, Jacob Tfelt-Hansen23, Heikki Swan24, Tomas Robyns25, Seiko Ohno8,26, Jason D Roberts27, Maarten P van den Berg28, Janneke A Kammeraad29, Vincent Probst30, Prince J Kannankeril31, Nico A Blom32,33, Elijah R Behr34,35, Martin Borggrefe13,14, Kristina H Haugaa12, Christopher Semsarian9,10,11, Minoru Horie8, Wataru Shimizu7,36, Janice A Till6, Antoine Leenhardt5, Michael J Ackerman2,3,4, Arthur A Wilde1,37. 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, USA. 3. Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, 200 First St SW, Rochester, MN, USA. 4. Department of Molecular Pharmacology and Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, 200 First St SW, Rochester, MN, USA. 5. Service de Cardiologie et CNMR Maladies Cardiaques Héréditaires Rares, Hôpital Bichat, 46 Rue Henri Huchard, Paris, France. 6. Department of Cardiology, Royal Brompton Hospital, Sydney St, Chelsea, London, UK. 7. Division of Arrhythmia and Electrophysiology, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Centre, 5 Chome-7-1 Fujishirodai, Suita, Osaka, Japan. 8. Department of Cardiovascular Medicine, Shiga University of Medical Science, Seta Tsukinowacho, Otsu, Japan. 9. Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney, Locked Bag 6, Newtown, Sydney, Australia. 10. Faculty of Medicine and Health, The University of Sydney, Locked Bag 6, Newtown, Sydney, Australia. 11. Department of Cardiology, Royal Prince Alfred Hospital, Locked Bag 6, Newtown, Sydney, Australia. 12. Department of Cardiology, Centre for Cardiological Innovation, Oslo University Hospital, Rikshospitalet, Sognsvannsveien 20, Oslo, Norway. 13. Department of Cardiology, University Medical Centre Mannheim, Theodor-Kutzer-Ufer 1 - 3, Mannheim, Germany. 14. German Centre for Cardiovascular Research (DZHK), Partner Site Heidelberg/Mannheim, Mannheim, Germany. 15. Istituto Auxologico Italiano, IRCCS, Center for Cardiac Arrhythmias of Genetic Origin, Via Pier Lombardo 22, Milan, Italy. 16. Hôpital Necker-Enfants-Malades, Cardiologie Pédiatrique, 149 Rue de Sèvres, Paris, France. 17. LIRYC Institute, Bordeaux University Hospital, Bordeaux University, Avenue du Haut Lévêque, 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, BC, Canada. 21. BC Children's Hospital, 4480 Oak St, Vancouver, BC, Canada. 22. Department of Pediatrics, University of British Columbia, 4480 Oak Street, Vancouver, BC, Canada. 23. Department of Cardiology, Rigshospitalet, Blegdamsvej 9, Copenhagen, Denmark. 24. Heart and Lung Centre, Helsinki University Hospital and Helsinki University, Tukholmankatu 8 A Helsinki, Finland. 25. Department of Cardiovascular Diseases, University Hospitals Leuven, Herestraat 49, Leuven, Belgium. 26. Department of Bioscience and Genetics, National Cerebral and Cardiovascular Centre, 5 Chome-7-1 Fujishirodai, Suita, Osaka, Japan. 27. Section of Cardiac Electrophysiology, Division of Cardiology, Department of Medicine, Western University, 339 Windermere Road, B6-129B, London, ON, Canada. 28. Department of Cardiology, University of Groningen, University Medical Centre Groningen, Hanzeplein 1, 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, USA. 32. Department of Pediatric Cardiology, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Meibergdreef 9, AZ Amsterdam, The Netherlands. 33. Department of Pediatric Cardiology, Willem-Alexander Children's Hospital, Leiden University Medical Centre, Albinusdreef 2, ZA Leiden, The Netherlands. 34. Molecular and Clinical Sciences Research Institute, St. George's, University of London, Cranmer Terrace, London, UK. 35. Cardiology Clinical Academic Group, St. George's University Hospitals NHS Foundation Trust, Cranmer Terrace, London, 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, 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. Published on behalf of the European Society of Cardiology. All rights reserved.
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. Published on behalf of the European Society of Cardiology. All rights reserved.
Authors: Arthur A M Wilde; Christopher Semsarian; Manlio F Márquez; Alireza Sepehri Shamloo; Michael J Ackerman; Euan A Ashley; Back Sternick Eduardo; Héctor Barajas-Martinez; Elijah R Behr; Connie R Bezzina; Jeroen Breckpot; Philippe Charron; Priya Chockalingam; Lia Crotti; Michael H Gollob; Steven Lubitz; Naomasa Makita; Seiko Ohno; Martín Ortiz-Genga; Luciana Sacilotto; Eric Schulze-Bahr; Wataru Shimizu; Nona Sotoodehnia; Rafik Tadros; James S Ware; David S Winlaw; Elizabeth S Kaufman; Takeshi Aiba; Andreas Bollmann; Jong-Il Choi; Aarti Dalal; Francisco Darrieux; John Giudicessi; Mariana Guerchicoff; Kui Hong; Andrew D Krahn; Ciorsti Mac Intyre; Judith A Mackall; Lluís Mont; Carlo Napolitano; Pablo Ochoa Juan; Petr Peichl; Alexandre C Pereira; Peter J Schwartz; Jon Skinner; Christoph Stellbrink; Jacob Tfelt-Hansen; Thomas Deneke Journal: J Arrhythm Date: 2022-05-31
Authors: Arthur A M Wilde; Christopher Semsarian; Manlio F Márquez; Alireza Sepehri Shamloo; Michael J Ackerman; Euan A Ashley; Eduardo Back Sternick; Héctor Barajas-Martinez; Elijah R Behr; Connie R Bezzina; Jeroen Breckpot; Philippe Charron; Priya Chockalingam; Lia Crotti; Michael H Gollob; Steven Lubitz; Naomasa Makita; Seiko Ohno; Martín Ortiz-Genga; Luciana Sacilotto; Eric Schulze-Bahr; Wataru Shimizu; Nona Sotoodehnia; Rafik Tadros; James S Ware; David S Winlaw; Elizabeth S Kaufman; Takeshi Aiba; Andreas Bollmann; Jong Il Choi; Aarti Dalal; Francisco Darrieux; John Giudicessi; Mariana Guerchicoff; Kui Hong; Andrew D Krahn; Ciorsti MacIntyre; Judith A Mackall; Lluís Mont; Carlo Napolitano; Juan Pablo Ochoa; Petr Peichl; Alexandre C Pereira; Peter J Schwartz; Jon Skinner; Christoph Stellbrink; Jacob Tfelt-Hansen; Thomas Deneke Journal: Europace Date: 2022-09-01 Impact factor: 5.486
Authors: Peter J Schwartz; Michael J Ackerman; Charles Antzelevitch; Connie R Bezzina; Martin Borggrefe; Bettina F Cuneo; Arthur A M Wilde Journal: Nat Rev Dis Primers Date: 2020-07-16 Impact factor: 52.329
Authors: Małgorzata Stępień-Wojno; Joanna Ponińska; Elżbieta K Biernacka; Bogna Foss-Nieradko; Tomasz Chwyczko; Paweł Syska; Rafał Płoski; Zofia T Bilińska Journal: Diagnostics (Basel) Date: 2020-06-27
Authors: Pier D Lambiase; Lars Eckardt; Dominic A Theuns; Timothy R Betts; Andreas L Kyriacou; Elizabeth Duffy; Reinoud Knops Journal: Heart Rhythm O2 Date: 2020-10-28