title: Implantable cardioverter defibrillator therapy in pediatric and congenital heart disease patients: a single tertiary center experience in Korea.
abstract: OBJECTIVE
  The use of implantable cardioverter defibrillators (ICDs) to prevent sudden cardiac death is increasing in children and adolescents. This study investigated the use of ICDs in children with congenital heart disease.
  
  
  METHODS
  This retrospective study was conducted on the clinical characteristics and effectiveness of ICD implantation at the department of pediatrics of a single tertiary center between 2007 and 2011.
  
  
  RESULTS
  Fifteen patients underwent ICD implantation. Their mean age at the time of implantation was 14.5±5.4 years (range, 2 to 22 years). The follow-up duration was 28.9±20.4 months. The cause of ICD implantation was cardiac arrest in 7, sustained ventricular tachycardia in 6, and syncope in 2 patients. The underlying disorders were as follows: ionic channelopathy in 6 patients (long QT type 3 in 4, catecholaminergic polymorphic ventricular tachycardia [CPVT] in 1, and J wave syndrome in 1), cardiomyopathy in 5 patients, and postoperative congenital heart disease in 4 patients. ICD coils were implanted in the pericardial space in 2 children (ages 2 and 6 years). Five patients received appropriate ICD shock therapy, and 2 patients received inappropriate shocks due to supraventricular tachycardia. During follow-up, 2 patients required lead dysfunction-related revision. One patient with CPVT suffered from an ICD storm that was resolved using sympathetic denervation surgery.
  
  
  CONCLUSIONS
  The overall ICD outcome was acceptable in most pediatric patients. Early diagnosis and timely ICD implantation are recommended for preventing sudden death in high-risk children and patients with congenital heart disease.
authors.affiliation: Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
authors.forename: Bo Kyung
authors.initials: BK
authors.lastname: Jin
authors.forename: Ji Seok
authors.initials: JS
authors.lastname: Bang
authors.forename: Eun Young
authors.initials: EY
authors.lastname: Choi
authors.forename: Gi Beom
authors.initials: GB
authors.lastname: Kim
authors.forename: Bo Sang
authors.initials: BS
authors.lastname: Kwon
authors.forename: Eun Jung
authors.initials: EJ
authors.lastname: Bae
authors.forename: Chung Il
authors.initials: CI
authors.lastname: Noh
authors.forename: Jung Yun
authors.initials: JY
authors.lastname: Choi
authors.forename: Woong Han
authors.initials: WH
authors.lastname: Kim
keywords: Child; Congenital heart defects; Implantable defibrillators; Long QT syndrome
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