Surgical trends and outcomes of neonatal Ebstein's anomaly: Multi-institutional study

Abstract

Objective

Ebstein's anomaly is a rare condition that can be diagnosed at any age. Symptomatic neonatal Ebstein's anomaly represents a challenging clinical entity with high morbidity and mortality. This study aims to describe treatment modalities and outcomes in neonatal Ebstein's anomaly.

Methods

We performed a review of the Pediatric Health Information System from 2004 to 2023. Patients with an Ebstein's anomaly diagnosis who were admitted within the first 2 days of life and who underwent a surgical intervention during that admission were included.

Results

A total of 330 patients were identified; 181 (55%) were female, 155 (47%) were non-Hispanic White, the median age at the initial Ebstein's anomaly surgery was 6 (interquartile range, 3-12) days, and the median birth weight was 2.9 (interquartile range, 2.5-3.2) kg. Median follow-up time was 1.8 (interquartile range, 0.1-6.8) years. Overall, 186 patients (56%) underwent isolated aortopulmonary shunt, 81 patients (25%) received the Starnes procedure, 58 patients (18%) received tricuspid valve repairs, and 5 patients (2%) received tricuspid valve replacements. Median length of stay was 38 (interquartile range, 24-74) days with no difference across procedures (P = .137). In-hospital mortality was 25% (n = 81), with tricuspid valve replacements (n = 4, 80%) having the highest. In multivariable Cox analysis, Black race (hazard ratio [HR], 2.7; 95% CI, 1.43-5.19; P = .002) and tricuspid valve replacements (HR, 8.2; 95% CI, 2.6-25.4; P < .001) had increased risk of mortality; older age at surgery (HR, 0.98; 95% CI, 0.97-0.99; P = .044) and higher birth weight (HR, 0.99; 95% CI, 0.99-0.99; P = .005) had a decreased risk for mortality. Overall survival at 10 years was 71% (95% CI, 66.0-76.3), and freedom from any cardiac reintervention at 10 years was 18% (95% CI, 12.7-25.8).

Conclusions

In a large multicenter dataset, surgery for neonatal Ebstein's anomaly is associated with significant mortality. Older age at surgery and higher birth weight are associated with decreased mortality. The initial approach does not dictate the need for a single ventricle pathway. These data can help counsel patients/families and help guide surveillance protocols and long-term management in this population.