title: Successful parental use of an automated external defibrillator for an infant with long-QT syndrome.
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.
authors.affiliation: 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
authors.forename: Abhay
authors.initials: A
authors.lastname: Divekar
authors.forename: Reeni
authors.initials: R
authors.lastname: Soni
mesh_terms: D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D003131:Combined Modality Therapy; D016757:Death, Sudden, Cardiac / Q000517:prevention & control; D047548:Defibrillators; D017147:Defibrillators, Implantable; D004554:Electric Countershock; D004562:Electrocardiography; D004630:Emergencies; D005260:Female; D005392:First Aid; D006801:Humans; D007223:Infant; D008133:Long QT Syndrome / Q000150:complications / Q000188:drug therapy / Q000628:therapy*; D008801:Mexiletine / Q000627:therapeutic use; D010290:Parents; D010353:Patient Education as Topic; D016171:Torsades de Pointes / Q000188:drug therapy / Q000209:etiology; D014693:Ventricular Fibrillation / Q000209:etiology / Q000628:therapy
chemical_list: D000889:Anti-Arrhythmia Agents; D008801:Mexiletine