Korean J Pediatr.  2013 Mar;56(3):125-129. 10.3345/kjp.2013.56.3.125.

Implantable cardioverter defibrillator therapy in pediatric and congenital heart disease patients: a single tertiary center experience in Korea

• Jin BK
• Bang JS
• Choi EY
• Kim GB
• Kwon BS
• Bae EJ
• Noh CI
• Choi JY
• Kim WH

• KMID: 2284283
• DOI: http://doi.org/10.3345/kjp.2013.56.3.125

Abstract

PURPOSE
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.
CONCLUSION
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.

Keyword

Implantable defibrillators; Congenital heart defects; Long QT syndrome; Child

MeSH Terms

Adolescent
Cardiomyopathies
Channelopathies
Child
Convulsive Therapy
Death, Sudden
Death, Sudden, Cardiac
Defibrillators
Defibrillators, Implantable
Early Diagnosis
Follow-Up Studies
Heart
Heart Arrest
Heart Defects, Congenital
Heart Diseases
Humans
Korea
Long QT Syndrome
Pediatrics
Retrospective Studies
Shock
Sympathectomy
Syncope
Tachycardia, Supraventricular
Tachycardia, Ventricular
Tachycardia, Ventricular