Surgical outcome in pediatric patients with Ebstein's anomaly: A multicenter, long-term study

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

*Bijbehorende auteur voor dit werk

OnderzoeksoutputAcademicpeer review

7 Citaten (Scopus)


ObjectiveSurgical 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.

DesignRecords of all Ebstein's anomaly patients born between 1980 and 2013 were reviewed. Demographic variables, intraoperative procedures and postoperative outcomes were analyzed.

ResultsSixty-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.

ConclusionsSurgery 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.

Originele taal-2English
Pagina's (van-tot)32-39
Aantal pagina's8
TijdschriftCongenital heart disease
Nummer van het tijdschrift1
StatusPublished - feb-2017

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