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

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