Staged Treatment for Critically Ill Neonates With Ebstein Anomaly: Cone Repair After Starnes Procedure

Area of Science:

• Pediatric Cardiac Surgery
• Congenital Heart Disease
• Cardiovascular Surgery

Background:

• The Starnes procedure historically led to a single ventricle pathway due to right ventricular (RV) cavity reduction.
• Critical neonatal Ebstein anomaly presents challenges for biventricular repair.

Purpose of the Study:

• To describe an up-to-date staged surgical repair for critical neonatal Ebstein anomaly.
• To emphasize surgical details of the Starnes procedure, intermediate interventions, and Cone repair post-Starnes.

Main Methods:

• A staged treatment approach involving tricuspid valve (TV) repair after the Starnes procedure.
• Inclusion of a Glenn procedure with pulmonary valve (PV) commissurotomy as an intermediate stage for patients with pulmonary atresia (PAt).
• Description of surgical maneuvers during Starnes and Glenn procedures to facilitate subsequent steps and promote RV growth.

Main Results:

• Successful achievement of 2-ventricle or one-and-a-half ventricle repair in 17 patients.
• Routine application of staged treatment with Cone repair after Starnes since 2019.

Conclusions:

• The described staged repair facilitates biventricular or one-and-a-half ventricle repair in critical neonatal Ebstein anomaly.
• This approach offers an alternative to the traditional single ventricle pathway for selected patients.