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European Heart Journal Advance Access published online on October 9, 2006
European Heart Journal, doi:10.1093/eurheartj/ehl306
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European Heart Journal © The European Society of Cardiology 2006; all rights reserved
Received May 30, 2006
Revised August 28, 2006
Accepted September 21, 2006

Clinical research

Outcome of implantable cardioverter defibrillators in adults with congenital heart disease: a multi-centre study

Sing-Chien Yap 1 *, Jolien W. Roos-Hesselink 1, Elke S. Hoendermis 2, Werner Budts 3, Hubert W. Vliegen 4, Barbara J.M. Mulder 5, Arie P. van Dijk 6, Martin J. Schalij 4, and Willem Drenthen 2

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; University Medical Centre, Utrecht, The Netherlands
6 Department of Cardiology, University Medical Centre Nijmegen, Nijmegen, The Netherlands

* To whom correspondence should be addressed.
Sing-Chien Yap, E-mail: s.c.yap{at}erasmusmc.nl


  Abstract

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.

Keywords: Congenital heart disease; Implantable defibrillators; Sudden cardiac death; Tetralogy of Fallot; Adults.
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