Current surgical therapy for Ebstein anomaly in neonates

Area of Science:

• Pediatric Cardiac Surgery
• Congenital Heart Disease
• Neonatal Cardiology

Background:

• Ebstein anomaly in neonates often leads to profound heart failure and historically poor outcomes.
• Surgical intervention is critical for severely symptomatic neonates with Ebstein anomaly.

Purpose of the Study:

• To report institutional experience with surgical management of Ebstein anomaly in severely symptomatic neonates.
• To evaluate the outcomes of different surgical strategies, including tricuspid valve repair and right ventricular exclusion.

Main Methods:

• Retrospective review of 16 neonates undergoing intervention for Ebstein anomaly (1992-2005).
• Surgical strategies included tricuspid valve repair or right ventricular exclusion with a fenestrated or nonfenestrated patch.
• A modified Blalock-Taussig shunt was used for pulmonary blood flow; the Starnes procedure evolved to include tricuspid valve patch fenestration.

Main Results:

• Hospital mortality was 31% (5/16).
• Survival was higher in the fenestrated tricuspid valve patch group (80%) compared to the nonfenestrated group (33%), though not statistically significant.
• Survivors of right ventricular exclusion underwent subsequent Fontan or bidirectional Glenn procedures.

Conclusions:

• Right ventricular exclusion with a fenestrated tricuspid valve patch, right atrioplasty, right ventriculoplasty, and a Blalock-Taussig shunt (Starnes procedure) effectively palliates neonates with critical Ebstein anomaly.
• This approach is suitable for neonates with a tricuspid valve unsuitable for repair.