Author + information
- Received October 10, 2012
- Revision received December 29, 2012
- Accepted January 2, 2013
- Published online April 9, 2013.
- Barry J. Maron, MD⁎,⁎ (, )
- Paolo Spirito, MD†,
- Michael J. Ackerman, MD, PhD‡,
- Susan A. Casey, RN⁎,
- Christopher Semsarian, MB, BS, PhD§,
- N.A. Mark Estes III, MD∥,
- Kevin M. Shannon, MD¶,
- Euan A. Ashley, PhD#,
- Sharlene M. Day, MD⁎⁎,
- Giuseppe Pacileo, MD††,
- Francesco Formisano, MD†,
- Emmanuela Devoto, MD†,
- Aristidis Anastasakis, MD‡‡,
- J. Martijn Bos, MD, PhD‡,
- Anna Woo, MD§§,
- Camillo Autore, MD∥∥,
- Robert H. Pass, MD¶¶,
- Giuseppe Boriani, MD, PhD##,
- Ross F. Garberich, MS⁎,
- Adrian K. Almquist, MD⁎,
- Mark W. Russell, MD⁎⁎,
- Luca Boni, MD, PhD⁎⁎⁎,
- Stuart Berger, MD†††,
- Martin S. Maron, MD∥ and
- Mark S. Link, MD∥
- ↵⁎Reprint requests and correspondence:
Dr. Barry J. Maron, Minneapolis Heart Institute Foundation, 920 East 28th Street, Suite 620, Minneapolis, Minnesota 55407
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.
This study was funded in part by a grant from Medtronic, Inc. (which manufactures implantable cardioverter-defibrillators) but is not industry sponsored. Medtronic provided only support for investigator professional time and had no role in the design and conduct of the study; the collection, analysis, or interpretation of data; or the preparation, review, or approval of the manuscript. Statistical analysis was performed at the Minneapolis Heart Institute Foundation by one of the authors (Mr. Garberich). Dr. Ackerman is a consultant for Boston Scientific Corporation, Medtronic, Inc., and St. Jude Medical. Dr. Estes has received research funding and consulting fees from Boston Scientific Corporation and consulting fees from Medtronic, Inc. Dr. Boriani has received speaker's fees from Medtronic, Inc. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received October 10, 2012.
- Revision received December 29, 2012.
- Accepted January 2, 2013.
- American College of Cardiology Foundation