European Heart Journal Advance Access originally published online on October 9, 2006
European Heart Journal 2007 28(15):1854-1861; doi:10.1093/eurheartj/ehl306
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Outcome of implantable cardioverter defibrillators in adults with congenital heart disease: a multi-centre study
1 Department of Cardiology, Thoraxcentre, Erasmus MC, Dr Molewaterplein 40, 3015 GD Rotterdam, The Netherlands
2 Department of Cardiology, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands
3 Department of Cardiology, University Hospitals Leuven, Leuven, Belgium
4 Department of Cardiology, Leiden University Medical Centre, Leiden The Netherlands
5 Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands
6 University Medical Centre Utrecht, Utrecht, The Netherlands
7 Department of Cardiology, University Medical Centre Nijmegen, Nijmegen, The Netherlands
Received 30 May 2006; revised 28 August 2006; accepted 21 September 2006; online publish-ahead-of-print 9 October 2006.
* Corresponding author. Tel: +31 10 463 2432; fax: +31 10 463 5498. E-mail address: s.c.yap{at}erasmusmc.nl
Aims: To investigate outcome and complications of implantable cardioverter defibrillators (ICDs) in adults with congenital heart disease (CHD) and to identify predictors of (in-) appropriate shocks.
Methods and results: Sixty-four CHD patients 
18 years at first ICD implantation [63% tetralogy of Fallot (TOF) and age at implantation 37 ± 13 years] were identified using the Dutch adult CHD registry and a Belgian tertiary care centre database. Median follow-up duration was 3.7 years. Early complications included pocket haematoma (n = 3), lead failure (n = 2), and pneumothorax (n = 2). Late complications occurred in 11 (17%) patients, including lead failure (n = 6) and and electrical storm (n = 3). Overall, 30 device-related re-interventions were performed in 20 patients (31%), including four premature generator changes and seven lead replacements. Half of the patients received one or more shocks, and 46 shocks in 15 patients (23%) were classified as appropriate. One hundred and sixty shocks in 26 patients (41%) were classified as inappropriate. No predictors of (in-)appropriate shocks were identified, except TOF being associated with less appropriate shocks than patients with other CHD (HR 0.29, P = 0.02).
Conclusion: The ICD provided effective therapy in a quarter of adults with CHD with low complication rates. The incidence of inappropriate shocks, however, appeared to be excessive and warrants further attention.
Key Words: Congenital heart disease • Implantable defibrillators • Sudden cardiac death • Tetralogy of Fallot • Adults
This paper was guest edited by Prof. Michael A. Gatzoulis, Royal Brompton Hospital, London, UK
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