Surgical Outcome in Paediatric Patients with Ebstein's Anomaly: A Multicenter, Long-Term Study

Objectives: To review outcomes for a large Dutch cohort of pediatric patients with Ebstein's anomaly treated surgically in childhood (0–18 years). We focus on the intended treatment (biventricular or 1.5 ventricle repair or univentricular palliation), freedom from unplanned reoperation and survival in this specific age group.

Methods: Records of all Ebstein anomaly patients born between January 1980 and July 2013 were reviewed. Demographic variables, surgical reports and postoperative outcomes were analyzed.

Results: Of the 176 included patients, 112 were not operated during childhood, whereas the other 64 patients underwent 110 operations. Median follow-up of this surgical subgroup after diagnosis was 115 months (range: 0–216). Thirty (47%) patients required surgery in the first year of life, including 21 who required neonatal surgery. The intended treatment was biventricular (n = 37, 58%) and 1.5 ventricle (n = 5, 8%) repair or univentricular (n = 22, 34%) palliation. The 1-, 5-, and 10-year freedoms from unplanned reoperation were 88, 78, and 73%, respectively. There were 10 (16%) in-hospital deaths (9 within 30 days and 1 at 33 days after surgery). Causes of death were low cardiac output syndrome, cardiac failure, hypoxemia, pulmonary hypertension, or an unknown cause. There were no late deaths.

Conclusion: Surgery in childhood represents the worse spectrum of disease, biventricular repair can often not be applied. Patients often face revision surgery. Mortality is limited to the immediate post-surgical period.