title: Efficacy of an implantable cardioverter-defibrillator in a neonate with LQT3 associated arrhythmias.
abstract: We present a case in which LQTS induced severe prenatal and neonatal arrhythmias. LQT3 was diagnosed (mutation R1623Q). Short-acting beta-blockers were ineffective as well as sotalol and mexiletine in preventing recurrent ventricular arrhythmias. An ICD was implanted at the age of 7 months (weight and length of the infant at implantation 6 kg and 60 cm respectively). Flecainide was prescribed in addition to the ICD implantation. After an appropriate shock the flecainide plasma levels were shown to be subtherapeutic. Readjustment of the flecainide dose resulted in adequate plasma levels. No further shocks occurred during a further 17 months follow-up period. The combination of an active can with a subcutaneous patch proved feasible, and lifesaving shocks occurred at 7 months after implantation.
authors.affiliation: Department of Pediatric Cardiology, Erasmus MC-Sophia, 3015 GJ Rotterdam, The Netherlands. a.tenharkel@erasmusmc.nl
authors.forename: Arend D J
authors.initials: AD
authors.lastname: Ten Harkel
authors.forename: Maarten
authors.initials: M
authors.lastname: Witsenburg
authors.forename: Peter L
authors.initials: PL
authors.lastname: de Jong
authors.forename: Luc
authors.initials: L
authors.lastname: Jordaens
authors.forename: Marjolein
authors.initials: M
authors.lastname: Wijman
authors.forename: Arthur A M
authors.initials: AA
authors.lastname: Wilde
mesh_terms: D017147:Defibrillators, Implantable; D004554:Electric Countershock; D004562:Electrocardiography; D006330:Heart Defects, Congenital / Q000628:therapy*; D006801:Humans; D007231:Infant, Newborn; D008133:Long QT Syndrome / Q000151:congenital / Q000628:therapy*