Thomas M Roston1, Karolina Jones2, Nathaniel M Hawkins3, J Martijn Bos4, Peter J Schwartz5, Frances Perry2, Michael J Ackerman6, Zachary W M Laksman3, Padma Kaul7, Krystien V V Lieve8, Joseph Atallah9, Andrew D Krahn3, Shubhayan Sanatani10. 1. Department of Medicine, University of Alberta, Edmonton, Alberta, Canada; BC Children's Hospital, Division of Cardiology, Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada. 2. BC Children's Hospital, Division of Cardiology, Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada. 3. Division of Cardiology, University of British Columbia, Vancouver, British Columbia, Canada. 4. Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota; Department of Molecular Pharmacology & Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, Minnesota. 5. Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico Italiano, Milan, Italy. 6. Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota; Department of Molecular Pharmacology & Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, Minnesota; Department of Cardiovascular Medicine, Division of Heart Rhythm Services, Mayo Clinic, Rochester, Minnesota. 7. Canadian VIGOUR Centre, University of Alberta, Edmonton, Alberta, Canada; Department of Medicine, University of Alberta, Edmonton, Alberta, Canada. 8. Heart Centre, Department of Clinical and Experimental Cardiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands. 9. Stollery Children's Hospital, Division of Cardiology, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada. 10. BC Children's Hospital, Division of Cardiology, Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada. Electronic address: ssanatani@cw.bc.ca.
Abstract
BACKGROUND: The implantable cardioverter-defibrillator (ICD) may be associated with a high risk of complications in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT). However, ICDs in this population have not been systematically evaluated. OBJECTIVE: The purpose of this study was to characterize the use and outcomes of ICDs in CPVT. METHODS: We conducted a systematic review using Embase, MEDLINE, PubMed, and Google Scholar to identify studies that included patients with CPVT who had an ICD. RESULTS: Fifty-three studies describing 1429 patients with CPVT were included. In total, 503 patients (35.2%) had an ICD (median age 15.0 years; interquartile range 11.0-21.0 years). Among ICD recipients with a reported medication status, 96.7% were prescribed β-blockers and 13.2% flecainide. Sympathetic denervation was performed in 23.2%. Nearly half of patients received an ICD for primary prevention (47.3%), and 12.8% were prescribed optimal antiarrhythmic therapy. During follow-up, 40.1% had ≥1 appropriate shock, 20.8% experienced ≥1 inappropriate shock, 19.6% had electrical storm, and 7 patients (1.4%) died. An ICD-associated electrical storm was implicated in 4 deaths. Additional complications such as lead failure, endocarditis, or surgical revisions were observed in 96 of 296 patients (32.4%). A subanalysis of the 10 studies encompassing 330 patients with the most detailed ICD-related data showed similar trends. CONCLUSION: In this population with CPVT, ICDs were common and associated with a high burden of shocks and complications. The reliance on primary prevention ICDs, and poor uptake of adjuvant antiarrhythmic therapies, suggests that improved adherence to guideline-directed management could reduce ICD use and harm.
BACKGROUND: The implantable cardioverter-defibrillator (ICD) may be associated with a high risk of complications in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT). However, ICDs in this population have not been systematically evaluated. OBJECTIVE: The purpose of this study was to characterize the use and outcomes of ICDs in CPVT. METHODS: We conducted a systematic review using Embase, MEDLINE, PubMed, and Google Scholar to identify studies that included patients with CPVT who had an ICD. RESULTS: Fifty-three studies describing 1429 patients with CPVT were included. In total, 503 patients (35.2%) had an ICD (median age 15.0 years; interquartile range 11.0-21.0 years). Among ICD recipients with a reported medication status, 96.7% were prescribed β-blockers and 13.2% flecainide. Sympathetic denervation was performed in 23.2%. Nearly half of patients received an ICD for primary prevention (47.3%), and 12.8% were prescribed optimal antiarrhythmic therapy. During follow-up, 40.1% had ≥1 appropriate shock, 20.8% experienced ≥1 inappropriate shock, 19.6% had electrical storm, and 7 patients (1.4%) died. An ICD-associated electrical storm was implicated in 4 deaths. Additional complications such as lead failure, endocarditis, or surgical revisions were observed in 96 of 296 patients (32.4%). A subanalysis of the 10 studies encompassing 330 patients with the most detailed ICD-related data showed similar trends. CONCLUSION: In this population with CPVT, ICDs were common and associated with a high burden of shocks and complications. The reliance on primary prevention ICDs, and poor uptake of adjuvant antiarrhythmic therapies, suggests that improved adherence to guideline-directed management could reduce ICD use and harm.
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: Michael Veith; Ibrahim El-Battrawy; Gretje Roterberg; Laura Raschwitz; Siegfried Lang; Christian Wolpert; Rainer Schimpf; Xiaobo Zhou; Ibrahim Akin; Martin Borggrefe Journal: J Clin Med Date: 2020-03-25 Impact factor: 4.241
Authors: Estefanía Martínez-Barrios; Sergi Cesar; José Cruzalegui; Clara Hernandez; Elena Arbelo; Victoria Fiol; Josep Brugada; Ramon Brugada; Oscar Campuzano; Georgia Sarquella-Brugada Journal: Biomedicines Date: 2022-01-05