Published online June 30, 2006
PEDIATRICS Vol. 118 No. 2 August 2006, pp. e526-e529 (doi:10.1542/peds.2006-0129)
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EXPERIENCE AND REASON

Successful Parental Use of an Automated External Defibrillator for an Infant With Long-QT Syndrome

Abhay Divekar, MBBS, MD and Reeni Soni, MD

Section of Pediatric Cardiology, Department of Pediatrics, Health Sciences Center, University of Manitoba, Winnipeg, Manitoba, Canada

Congenital long-QT syndrome with 2:1 atrioventricular block presenting in the perinatal period is rare, has a poor prognosis, and leads to high risk for lethal ventricular arrhythmic events. An implantable cardioverter-defibrillator seems to be the most effective treatment in the prevention of arrhythmic sudden cardiac death in patients with long-QT syndrome. Technical limitations and risks associated with implantable cardioverter-defibrillators in asymptomatic infants is considered too great to justify use for primary prophylaxis against sudden cardiac death. In this case report we describe the first successful parental use of an automated external defibrillator prescribed for primary prophylaxis against sudden cardiac death in an infant with long-QT syndrome.

Key Words: automated external defibrillator • long-QT syndrome • sudden cardiac death

Abbreviations: LQTS, long-QT syndrome • AED, automated external defibrillator • SCD, sudden cardiac death • QTc, corrected QT interval • EKG, electrocardiogram • ICD, implantable cardioverter-defibrillator

Accepted Feb 2, 2006.