ORIGINAL ARTICLE Multicenter Study of the Effectiveness of Implantable Cardioverter Defibrillators in Children and Young Adults With Heart Disease Nicholas H. Von Bergen • Dianne L. Atkins • Macdonald Dick II • David J. Bradley • Susan P. Etheridge • Elizabeth V. Saarel • Peter S. Fischbach • Seshadri Balaji • Narayanswami Sreeram • William N. Evans • Ian H. Law Received: 26 August 2010 / Accepted: 7 December 2010 / Published online: 6 January 2011 Ó Springer Science+Business Media, LLC 2010 Abstract Implantable cardioverter defibrillators (ICDs) are being used with increasing frequency in children and young adults. Our aim was to examine the appropriateness and frequency of ICD discharges in children and young adults, to compare the effectiveness of ICDs when placed for primary or secondary prevention, and to provide time- dependent analysis of ICD discharges. Data were collected from seven institutions on 210 patients \ 30 years of age who underwent ICD implantation from October 1992 to January 2007. Median age at implant was 15.4 years with a follow-up average of 3.3 years. Heart disease was catego- rized as electrical (n = 90, 42%), cardiomyopathic (n = 62, 30%), or congenital heart disease (n = 58, 28%). ICDs are increasingly placed for primary prevention. There are increased appropriate ICD discharges for ICDs placed for secondary prevention (52%) versus primary prevention (14%) at 5 years. There is no difference in the risk of inappropriate discharges between primary and secondary prevention indications. There is an increased risk for inappropriate therapy in the congenital heart disease pop- ulation. An increasing number of ICDs are being placed for primary prevention in young patients, a marked shift in practice during the last two decades. The benefits of ICDs remain greater in secondary than in primary-prevention patients. In both groups, approximately 25% of patients received inappropriate discharges within 5 years of implant. Patients with congenital heart disease are the most affected by inappropriate discharges. Keywords Implantable cardioverter defibrillator Á Congenital heart disease Á Pediatrics Á Long QT syndrome Á Defibrillation Á Sudden cardiac death Introduction Implantable cardioverter defibrillators (ICDs) are used in adults with potential lethal arrhythmias related to ischemic heart disease and decreased heart function. In addition, the use of ICDs in younger patients is currently expanding due to generator and lead technology improvements, greater understanding of genetic channelopathies, and an increas- ing number of children with congenital heart disease N. H. Von Bergen (&) Á D. L. Atkins Á I. H. Law Department of Pediatric Cardiology, Carver College of Medicine, University of Iowa Children’s Hospital, University of Iowa, Iowa City, IA, USA e-mail: nicholas-vonbergen@uiowa.edu M. Dick II Á D. J. Bradley Department of Pediatric Cardiology, University of Michigan Congenital Heart Center, University of Michigan, Ann Arbor, MI, USA D. J. Bradley Á S. P. Etheridge Á E. V. Saarel Department of Pediatric Cardiology, Primary Children’s Medical Center and The University of Utah School of Medicine, Salt Lake City, UT, USA P. S. Fischbach Department of Pediatric Cardiology, Sibley Heart Center, Atlanta, GA, USA S. Balaji Department of Pediatric Cardiology, Oregon Heath Sciences, Portland, OR, USA N. Sreeram Department of Pediatric Cardiology, Heart Center, University Hospital of Cologne, Cologne, Germany W. N. Evans Á I. H. Law Department of Pediatric Cardiology, Las Vegas Children’s Heart Center, Las Vegas, NV, USA 123 Pediatr Cardiol (2011) 32:399–405 DOI 10.1007/s00246-010-9866-7