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Updated: Mar 15, 2026
Surgical Correction for Pediatric Epiblepharon and Trichiasis
Published on: July 8, 2025
Surgical outcome in pediatric patients with Ebstein's anomaly: A multicenter, long-term study
Lianne M Geerdink1,2, Gideon J du Marchie Sarvaas3, Irene M Kuipers4
1Department of Pediatric Cardiology, Radboud University Medical Center, Nijmegen, the Netherlands.
Insights
Pediatric Ebstein's anomaly surgery outcomes show high rates of revision surgery, with most deaths occurring shortly after initial operations. Biventricular repair is less common in this age group.
Area of Science:
- Pediatric Cardiac Surgery
- Congenital Heart Disease
- Congenital Heart Defects
Background:
- Ebstein's anomaly surgical outcomes are often reported in mixed-age groups.
- Focusing on pediatric patients (0-18 years) provides specific insights into this population's surgical management.
Purpose of the Study:
- To analyze surgical outcomes in pediatric patients with Ebstein's anomaly.
- To evaluate intended surgical treatments, freedom from reoperation, and survival rates in this specific age group.
Main Methods:
- Nationwide review of Ebstein's anomaly patients born between 1980 and 2013.
- Analysis of demographic data, surgical procedures, and postoperative outcomes.
Main Results:
- Sixty-three pediatric patients underwent 109 operations with a median follow-up of 121 months.
- 46% required surgery within the first year of life; biventricular repair was performed in 59%, 1.5 ventricle repair in 8%, and univentricular palliation in 33%.
- One-, five-, and 10-year freedom from unplanned reoperation was 89%, 79%, and 75%, respectively, with 14% in-hospital mortality.
Conclusions:
- Childhood surgery for Ebstein's anomaly often involves severe disease, with biventricular repair being less frequent.
- Pediatric patients frequently require revision surgeries, and mortality is concentrated in the immediate postoperative period.
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.
