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Europace 2005 7(1):77-84; doi:10.1016/j.eupc.2004.09.007
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© 2005 The European Society of Cardiology. Published by Elsevier Ltd. All rights reserved.

CASE REPORT

Efficacy of an implantable cardioverter-defibrillator in a neonate with LQT3 associated arrhythmias

Arend D.J. Ten Harkela,*, Maarten Witsenburga, Peter L. de Jongb, Luc Jordaensc, Marjolein Wijmand and Arthur A.M. Wildee

aDepartment of Pediatric Cardiology, Erasmus MC-Sophia Dr. Molewaterplein 60, 3015 GJ Rotterdam, The Netherlands; bDepartment of Cardiothoracic Surgery, Erasmus MC Dr. Molewaterplein 60, 3015 GJ Rotterdam, The Netherlands; cDepartment of Cardiology, Erasmus MC Dr. Molewaterplein 60, 3015 GJ Rotterdam, The Netherlands; dDepartment of Gynaecology, Erasmus MC-Sophia Dr. Molewaterplein 60, 3015 GJ Rotterdam, The Netherlands; eDepartment of Cardiology, AMC Amsterdam The Netherlands

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.

Key Words: ICD implantation, neonatal, LQT3

*Corresponding author. Tel.: +31 10 463 6363; fax: +31 10 463 6772. E-mail address: a.tenharkel{at}erasmusmc.nl (A.D.J. Ten Harkel).


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