Substantial Operative and Nonoperative Mortality Persists in Neonatal Ebstein Anomaly: A 20-Year Multi-Institutional Analysis

Background

We sought to characterize the epidemiology, treatment patterns, and outcomes of neonatal Ebstein anomaly using a national database.

Methods

In this descriptive analysis, the pediatric health information system (PHIS) database was queried for the demographics, treatment, and outcomes of neonates with Ebstein anomaly using International Classification of Disease codes between 2004 and 2024. The surgical and nonoperative cohorts were each divided into two eras: era 1 = 2004-2014, era 2 = 2015-2024.

Results

Among 1323 neonates identified, 173 (13%) underwent surgical repair and 1150 (87%) received nonoperative management. Overall hospital mortality was 26.6% (46/173) (operative) and 12.8% (147/1150) (nonoperative). Nonoperative mortality significantly decreased over time (18.4% [34/185] vs 11.7% [113/965], p = .01), while operative mortality did not improve (20.5% [9/44] vs 28.7% [37/129], p = .38).

In the operative cohort, 73% (126/173) required mechanical ventilation (MV) < 14 days and 27% (47/173) required MV ≥14 days, with no mortality difference. In the nonoperative cohort, 60% (690/1150) avoided intubation. In the nonoperative cohort, compared with nonintubated patients (mortality:8.4%), mortality odds increased significantly with MV for 7 to 13 days (21%; OR 8.5, p < .01) and ≥14 days (26%; OR 7.8, p < .01). High-volume centers (≥75th percentile for Ebstein admissions) had significantly lower operative mortality compared with low-volume centers (19.6% vs 27.5%, OR 0.39, p = .04) but equivalent nonoperative mortality. Only 26.6% (46/173) of surgical patients were treated at high-volume centers.

Conclusions

Significant mortality persists in neonatal Ebstein anomaly (27% [46/173] surgical, 13% [147/1150] nonsurgical), with improvement over time only in nonsurgical patients. Nonsurgical patients requiring intubation >7 days have >20% mortality, warranting strategy reconsideration. Higher-volume centers appear to demonstrate superior operative survival, perhaps supporting patient transfer for surgical management.