Neonatal Ebstein Anomaly: A 30-year Institutional Review

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Neonatal Surgery

Background:

• Ebstein anomaly is a rare congenital heart defect affecting the tricuspid valve.
• Long-term outcomes for neonates with Ebstein anomaly vary significantly based on management strategy and associated anomalies.

Purpose of the Study:

• To review a 30-year single-center experience managing neonates with Ebstein anomaly.
• To evaluate survival rates, need for reoperation, and functional status in this cohort.
• To identify factors influencing prognosis in neonates with Ebstein anomaly.

Main Methods:

• Retrospective review of 80 neonates diagnosed with Ebstein anomaly between 1985 and 2015.
• Analysis of early and late survival, reoperation rates, and functional status (New York Heart Association classification).
• Stratification of outcomes based on the need for medical management versus surgical or catheter-based intervention, and presence of pulmonary atresia.

Main Results:

• Overall hospital discharge survival was 86%, with a 15-year survival estimate of 67%.
• Neonates managed medically had significantly better 15-year survival (79%) compared to those requiring intervention (45%, P=0.005).
• Pulmonary atresia or critical stenosis occurred in 18 neonates; 34% of all patients required intervention, with 10-year freedom from reoperation for early surgical survivors being 16%.

Conclusions:

• Neonates with Ebstein anomaly who do not require intervention have a favorable prognosis.
• Significant mortality risk persists for neonates needing intervention, especially those with associated pulmonary atresia.
• Early and late outcomes are strongly influenced by the need for and type of intervention required.