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European Heart Journal Advance Access published online on September 24, 2007
European Heart Journal, doi:10.1093/eurheartj/ehm398
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© The European Society of Cardiology 2007. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

Ebstein's anomaly: factors associated with death in childhood and adolescence: a multi-centre, long-term study

Livia Kapusta1,*, Rhona M. Eveleigh1, Sumayah E. Poulino1, Marry E. Rijlaarsdam2, Gideon J. du Marchie Sarvaas3, Jan L. Strengers4, Tammo Delhaas5, Chris L. de Korte1, Ton Feuth1 and William A. Helbing6

1 Children's Heart Centre, University Medical Centre St Radboud, PO Box 9101, 6500 HB Nijmegen, The Netherlands
2 Centre of Congenital Heart Disease Amsterdam Leiden, The Netherlands
3 University Medical Centre Groningen, The Netherlands
4 University Medical Centre Utrecht, The Netherlands
5 University Hospital Maastricht, The Netherlands
6 Erasmus Medical Centre Rotterdam, The Netherlands

Received 19 March 2007; revised 23 July 2007; accepted 24 August 2007.

* Corresponding author. Tel: +31 24 3619060; fax: +31 24 3619052. E-mail address: l.kapusta{at}cukz.umcn.nl

Aims: The objective of this study is to establish factors associated with death after diagnosis of Ebstein's anomaly (EA) during childhood and adolescence.

Methods and results: This study is a retrospective chart review. All paediatric patients were diagnosed with EA and followed in tertiary-care university hospitals between 1980 and 2005. Factors associated with death were obtained using the Cox regression and log-rank tests. Of the 93 patients with EA, 18 (19%) died and 75 (81%) survived. The median age at EA diagnosis and follow-up was 0 (range 0–162) and 86 months (range 0–216), respectively. After 35 months of diagnosis, the Kaplan–Meier survival probability remains stable at 80%. Young age at presentation (≤12 months), hepatomegaly, the need for medication (diuretics and Prostin) and mechanical ventilation at presentation, pulmonary valve defects (defined as moderate-to-severe pulmonary stenosis and pulmonary atresia), patent arterial duct, and ventricular septal defect were significantly associated with death.

Conclusion: The overall survival of patients with EA during childhood and adolescence has dramatically improved when compared with earlier reports.

Key Words: Ebstein • Children • Risk factors • Death • Survival


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