TY  - EJOU
AU  - Geerdink, Lianne M. 
AU  - Sarvaas, Gideon J. du Marchie 
AU  - Kuipers, Irene M. 
AU  - Helbing, Willem A. 
AU  - Delhaas, Tammo 
AU  - Heide, Henriette ter 
AU  - Rozendaal, Lieke 
AU  - Korte, Chris L. de 
AU  - Singh, Sandeep K. 
AU  - Ebels, Tjark 
AU  - Hazekamp, Mark G. 
AU  - Haas, Felix 
AU  - Bogers, Ad J. J. C. 
AU  - Kapusta, Livia 

TI  - Surgical outcome in pediatric patients with Ebstein’s anomaly: A multicenter, long-term study
T2  - Structural and Congenital Heart Disease

PY  - 2017
VL  - 12
IS  - 1
SN  - 3071-1738

AB  - <b>Objective:</b> Surgical outcomes of pediatric patients with Ebstein’s anomaly are often described as
part of all-age-inclusive series. Our objective is to focus on patients treated surgically in childhood
(0-18 y). We study the intended treatment (biventricular or 1.5 ventricle repair or univentricular
palliation), freedom from unplanned reoperation and survival of this specific age group, in a nationwide study.<br/>
<b>Design:</b> Records of all Ebstein’s anomaly patients born between 1980 and 2013 were reviewed.
Demographic variables, intraoperative procedures and postoperative outcomes were analyzed.<br/>
<b>Results:</b> Sixty-three patients underwent 109 operations. Median follow-up after diagnosis was
121 months (range 0-216 months). Twenty-nine (46%) patients required surgery in the first year
of life, including 21 who required neonatal surgery. The intended treatment was biventricular
(n = 37, 59%) and 1.5 ventricle (n = 5, 8%) repair or univentricular (n = 21, 33%) palliation. The
one-, five-, and 10-year freedom from unplanned reoperation was 89%, 79%, and 75% respectively. There were nine (14%) in hospital deaths (within 30 d after surgery). Causes of death were
low cardiac output syndrome, cardiac failure, hypoxemia, pulmonary hypertension or an unknown
cause. There were no late deaths.<br/>
<b>Conclusions:</b> Surgery in childhood represents the worse spectrum of disease, biventricular repair
is often not applied. Patients often face revision surgery. Mortality is limited to the immediate
postsurgical period.
KW  - congenital
KW  -  Ebstein’s anomaly
KW  -  neonates
KW  -  outcome
KW  -  surgery

DO  -