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

Darren Turner¹, Amir Mehdizadeh-Shrifi¹, Muhammad Faateh¹

¹Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

World Journal for Pediatric & Congenital Heart Surgery

|June 17, 2026

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Neonatal Ebstein anomaly shows persistent high mortality, with improvements only in nonoperative cases. Surgical outcomes did not improve, and high-volume centers had better operative survival, suggesting potential benefits of patient transfer.

Area of Science:

Pediatric Cardiology
Congenital Heart Disease Epidemiology
Neonatal Surgery Outcomes

Background:

Ebstein anomaly is a rare congenital heart defect affecting the tricuspid valve.
Characterizing its impact on neonates is crucial for improving care.
National data provides a broad overview of treatment and outcomes.

Purpose of the Study:

To analyze the epidemiology, treatment patterns, and outcomes of neonatal Ebstein anomaly.
To compare outcomes between surgical and nonoperative management strategies.
To identify factors influencing mortality in this population.

Main Methods:

Descriptive analysis of the Pediatric Health Information System (PHIS) database (2004-2024).
Inclusion of neonates diagnosed with Ebstein anomaly using ICD codes.

Keywords:

Ebstein anomaly congenital heart disease neonatal cardiac surgery tricuspid valve anomaly

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Stratification of cohorts by treatment (surgical vs. nonoperative) and time eras.

Main Results:

1323 neonates identified; 13% surgical, 87% nonoperative.
Overall mortality: 26.6% surgical, 12.8% nonoperative.
Nonoperative mortality decreased over time (p=0.01); operative mortality did not (p=0.38).
Nonoperative patients needing >7 days mechanical ventilation had >20% mortality.
High-volume centers showed lower operative mortality (p=0.04).

Conclusions:

Significant mortality persists in neonatal Ebstein anomaly, with no improvement in surgical outcomes.
Nonoperative management shows improving survival, but prolonged ventilation increases mortality risk.
Higher-volume centers are associated with better surgical survival, supporting centralized care.