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.
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.
Authors: Hyung Yoon Kim; Shin Yi Jang; Ju Ryoung Moon; Eun Kyoung Kim; Sung A Chang; Jinyoung Song; June Huh; I Seok Kang; Ji Hyuk Yang; Tae Gook Jun; Seung Woo Park Journal: J Korean Med Sci Date: 2016-11 Impact factor: 2.153
Authors: Tammo Delhaas; Gideon J du Marchie Sarvaas; Marry E Rijlaarsdam; Jan L Strengers; Rhona M Eveleigh; Sumayah E Poulino; Chris L de Korte; Livia Kapusta Journal: Pediatr Cardiol Date: 2010-02 Impact factor: 1.655