Ebstein's anomaly: factors associated with death in childhood and adolescence: a multi-centre, long-term study.
until further notice
SourceEuropean Heart Journal, 28, 21, (2007), pp. 2661-6
Article / Letter to editor
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Paediatrics - OUD tm 2017
Primary and Community Care
Epidemiology, Biostatistics & HTA
European Heart Journal
SubjectIGMD 1: Functional imaging; IGMD 5: Health aging / healthy living; NCEBP 14: Cardiovascular diseases; UMCN 1.5: Interventional oncology; UMCN 2.1: Heart, lung and circulation
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.
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