Rapid two-stage Starnes procedure for a symptomatic neonate with Ebstein anomaly

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Surgical Innovation

Background:

• Ebstein anomaly is a rare congenital heart defect affecting the tricuspid valve, often leading to severe symptoms in neonates.
• Symptomatic neonates with Ebstein anomaly require timely and effective surgical intervention.
• Traditional surgical approaches may involve cardiopulmonary bypass, posing risks for critically ill newborns.

Observation:

• A novel rapid two-stage Starnes procedure was developed for a neonate diagnosed with Ebstein anomaly prenatally.
• The first stage, performed without cardiopulmonary bypass within 16 hours of birth, included pulmonary artery ligation, atrial and ventricular plication, a modified Blalock-Taussig shunt, and patent ductus arteriosus ligation.
• The second stage, at 12 days of age, involved the Starnes procedure using an autologous pericardial patch with a fenestration to close the tricuspid valve orifice.

Findings:

• The two-stage Starnes procedure was successfully implemented in a critically symptomatic neonate.
• The infant experienced an excellent postoperative course following both surgical stages.
• This approach demonstrates the feasibility and efficacy of a staged surgical strategy for severe Ebstein anomaly.

Implications:

• A rapid two-stage Starnes procedure represents a viable and effective therapeutic option for neonates with severe Ebstein anomaly.
• This technique may offer advantages by avoiding cardiopulmonary bypass in the initial emergency surgery.
• Further studies are warranted to evaluate the long-term outcomes and broader applicability of this surgical approach in pediatric cardiac surgery.