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

Eur Heart J. 2007 Nov;28(21):2661-6. doi: 10.1093/eurheartj/ehm398. Epub 2007 Sep 24.


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.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Age Factors
  • Cause of Death
  • Child
  • Child, Preschool
  • Ebstein Anomaly / complications
  • Ebstein Anomaly / mortality*
  • Epidemiologic Methods
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male