Surgical outcome in pediatric patients with Ebstein's anomaly: A multicenter, long-term study
Objective: 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. Design: Records of all Ebstein's anomaly patients born between 1980 and 2013 were reviewed. Demographic variables, intraoperative procedures and postoperative outcomes were analyzed. Results: 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. Conclusions: 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.
|Keywords||Congenital, Ebstein's anomaly, Neonates, Outcome, Surgery|
|Persistent URL||dx.doi.org/10.1111/chd.12404, hdl.handle.net/1765/93876|
|Journal||Congenital Heart Disease|
Geerdink, L.M, Du Marchie Sarvaas, G.J, Kuipers, I.M, Helbing, W.A, Delhaas, T, ter Heide, H. (Henriette), … Kapusta, L. (2016). Surgical outcome in pediatric patients with Ebstein's anomaly: A multicenter, long-term study. Congenital Heart Disease. doi:10.1111/chd.12404