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Ebstein s anomaly: factors associated with death in childhood and adolescence: a multi-centre, long-term study Kapusta L; Eveleigh RM; Poulino SE; Rijlaarsdam ME; du Marchie Sarvaas GJ; Strengers JL; Delhaas T; de Korte CL; Feuth T; Helbing WAEur Heart J 2007[Nov]; 28 (21): 2661-6AIMS: 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.|Adolescent[MESH]|Age Factors[MESH]|Cause of Death[MESH]|Child[MESH]|Child, Preschool[MESH]|Ebstein Anomaly/complications/*mortality[MESH]|Epidemiologic Methods[MESH]|Female[MESH]|Humans[MESH]|Infant[MESH]|Infant, Newborn[MESH]|Male[MESH] |
