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Updated: Jul 11, 2026
Assessment of Child Anthropometry in a Large Epidemiologic Study
Published on: February 2, 2017
Ebstein's anomaly: factors associated with death in childhood and adolescence: a multi-centre, long-term study
Livia Kapusta1, Rhona M Eveleigh, Sumayah E Poulino
1Children's Heart Centre, University Medical Centre St Radboud, PO Box 9101, 6500 HB Nijmegen, The Netherlands. l.kapusta@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 (< or =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.
