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Pediatrics. 2006 Aug;118(2):e526-9. Epub 2006 Jun 30.

Successful parental use of an automated external defibrillator for an infant with long-QT syndrome.

Author information

1
Section of Pediatric Cardiology, Department of Pediatrics, Health Sciences Center, University of Manitoba, FE-241, 685 William Ave, Winnipeg, Manitoba, Canada R3E 0Z2. adivekar@exchange.hsc.mb.ca

Abstract

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.

PMID:
16816007
DOI:
10.1542/peds.2006-0129
[Indexed for MEDLINE]
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