Ebstein's anomaly: factors associated with death in childhood and adolescence: a multi-centre, long-term study.
OBJECTIVE
The objective of this study is to establish factors associated with death after diagnosis of Ebstein's anomaly (EA) during childhood and adolescence.


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.


CONCLUSIONS
The overall survival of patients with EA during childhood and adolescence has dramatically improved when compared with earlier reports.
Kapusta
Livia
L
Children's Heart Centre, University Medical Centre St Radboud, PO Box 9101, 6500 HB Nijmegen, The Netherlands. l.kapusta@cukz.umcn.nl
Eveleigh
Rhona M
RM
Poulino
Sumayah E
SE
Rijlaarsdam
Marry E
ME
du Marchie Sarvaas
Gideon J
GJ
Strengers
Jan L
JL
Delhaas
Tammo
T
de Korte
Chris L
CL
Feuth
Ton
T
Helbing
William A
WA
D000293:Adolescent; D000367:Age Factors; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D004437:Ebstein Anomaly / Q000150:complications / Q000401:mortality*; D004812:Epidemiologic Methods; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male