OBJECTIVES: The aim of this study was to determine the efficacy of implantable cardioverter-defibrillators (ICDs) in children and adolescents with hypertrophic cardiomyopathy (HCM). BACKGROUND: HCM is the most common cause of sudden death in the young. The availability of ICDs over the past decade for HCM has demonstrated the potential for sudden death prevention, predominantly in adult patients. METHODS: A multicenter international registry of ICDs implanted (1987 to 2011) in 224 unrelated children and adolescents with HCM judged at high risk for sudden death was assembled. Patients received ICDs for primary (n = 188) or secondary (n = 36) prevention after undergoing evaluation at 22 referral and nonreferral institutions in the United States, Canada, Europe, and Australia. RESULTS: Defibrillators were activated appropriately to terminate ventricular tachycardia or ventricular fibrillation in 43 of 224 patients (19%) over a mean of 4.3 ± 3.3 years. ICD intervention rates were 4.5% per year overall, 14.0% per year for secondary prevention after cardiac arrest, and 3.1% per year for primary prevention on the basis of risk factors (5-year cumulative probability 17%). The mean time from implantation to first appropriate discharge was 2.9 ± 2.7 years (range to 8.6 years). The primary prevention discharge rate terminating ventricular tachycardia or ventricular fibrillation was the same in patients who underwent implantation for 1, 2, or ≥3 risk factors (12 of 88 [14%], 10 of 71 [14%], and 4 of 29 [14%], respectively, p = 1.00). Extreme left ventricular hypertrophy was the most common risk factor present (alone or in combination with other markers) in patients experiencing primary prevention interventions (17 of 26 [65%]). ICD-related complications, particularly inappropriate shocks and lead malfunction, occurred in 91 patients (41%) at 17 ± 5 years of age. CONCLUSIONS: In a high-risk pediatric HCM cohort, ICD interventions terminating life-threatening ventricular tachyarrhythmias were frequent. Extreme left ventricular hypertrophy was most frequently associated with appropriate interventions. The rate of device complications adds a measure of complexity to ICD decisions in this age group.
OBJECTIVES: The aim of this study was to determine the efficacy of implantable cardioverter-defibrillators (ICDs) in children and adolescents with hypertrophic cardiomyopathy (HCM). BACKGROUND: HCM is the most common cause of sudden death in the young. The availability of ICDs over the past decade for HCM has demonstrated the potential for sudden death prevention, predominantly in adult patients. METHODS: A multicenter international registry of ICDs implanted (1987 to 2011) in 224 unrelated children and adolescents with HCM judged at high risk for sudden death was assembled. Patients received ICDs for primary (n = 188) or secondary (n = 36) prevention after undergoing evaluation at 22 referral and nonreferral institutions in the United States, Canada, Europe, and Australia. RESULTS: Defibrillators were activated appropriately to terminate ventricular tachycardia or ventricular fibrillation in 43 of 224 patients (19%) over a mean of 4.3 ± 3.3 years. ICD intervention rates were 4.5% per year overall, 14.0% per year for secondary prevention after cardiac arrest, and 3.1% per year for primary prevention on the basis of risk factors (5-year cumulative probability 17%). The mean time from implantation to first appropriate discharge was 2.9 ± 2.7 years (range to 8.6 years). The primary prevention discharge rate terminating ventricular tachycardia or ventricular fibrillation was the same in patients who underwent implantation for 1, 2, or ≥3 risk factors (12 of 88 [14%], 10 of 71 [14%], and 4 of 29 [14%], respectively, p = 1.00). Extreme left ventricular hypertrophy was the most common risk factor present (alone or in combination with other markers) in patients experiencing primary prevention interventions (17 of 26 [65%]). ICD-related complications, particularly inappropriate shocks and lead malfunction, occurred in 91 patients (41%) at 17 ± 5 years of age. CONCLUSIONS: In a high-risk pediatric HCM cohort, ICD interventions terminating life-threatening ventricular tachyarrhythmias were frequent. Extreme left ventricular hypertrophy was most frequently associated with appropriate interventions. The rate of device complications adds a measure of complexity to ICD decisions in this age group.
Authors: Rocio Hinojar; José Luis Zamorano; Ariana Gonzalez Gómez; Maria Plaza Martin; Amparo Esteban; Luis Miguel Rincón; Juan Carlos Portugal; José Julio Jimenez Nácher; Covadonga Fernández-Golfín Journal: Clin Cardiol Date: 2017-06-14 Impact factor: 2.882
Authors: Marwan M Refaat; Akl C Fahed; Sylvana Hassanieh; Mostafa Hotait; Mariam Arabi; Hadi Skouri; Jonathan G Seidman; Christine E Seidman; Fadi F Bitar; Georges Nemer Journal: Card Electrophysiol Clin Date: 2016-03
Authors: Steven E Lipshultz; E John Orav; James D Wilkinson; Jeffrey A Towbin; Jane E Messere; April M Lowe; Lynn A Sleeper; Gerald F Cox; Daphne T Hsu; Charles E Canter; Juanita A Hunter; Steven D Colan Journal: Lancet Date: 2013-09-03 Impact factor: 79.321
Authors: Martin S Maron; Ethan J Rowin; Benjamin S Wessler; Paula J Mooney; Amber Fatima; Parth Patel; Benjamin C Koethe; Mikhail Romashko; Mark S Link; Barry J Maron Journal: JAMA Cardiol Date: 2019-07-01 Impact factor: 14.676
Authors: Gabrielle Norrish; Henry Chubb; Ella Field; Karen McLeod; Maria Ilina; Georgia Spentzou; Jan Till; Piers E F Daubeney; Alan Graham Stuart; Jane Matthews; Dominic Hares; Elspeth Brown; Katie Linter; Vinay Bhole; Krishnakumar Pillai; Michael Bowes; Caroline B Jones; Orhan Uzun; Amos Wong; Arthur Yue; Shankar Sadagopan; Tara Bharucha; Norah Yap; Eric Rosenthal; Sujeev Mathur; Satish Adwani; Zdenka Reinhardt; Jasveer Mangat; Juan Pablo Kaski Journal: Europace Date: 2021-03-08 Impact factor: 5.214