One-stage unifocalization followed by staged Fontan operation

Area of Science:

• Pediatric Cardiology
• Congenital Heart Surgery
• Cardiovascular Surgery

Background:

• Single ventricle physiology presents complex surgical challenges.
• Pulmonary atresia with major aortopulmonary collaterals requires multi-stage palliation.
• Absent central pulmonary artery necessitates reconstructive strategies.

Observation:

• A staged surgical approach was employed for a neonate with single ventricle, pulmonary atresia, MAPCAs, and absent central pulmonary artery.
• The first stage involved one-stage unifocalization, central pulmonary artery creation, and a modified Blalock-Taussig shunt at 1 month.
• The second stage included the Glenn operation and pulmonary arterial augmentation at 9 months, with Fontan completion at 22 months.

Findings:

• The staged approach facilitated continuous pulmonary artery development, evidenced by an increasing pulmonary arterial index and stable pulmonary arterial pressure.
• Surgical interventions, including unifocalization and pulmonary artery augmentation without prosthetic material, promoted significant pulmonary artery growth.
• Successful completion of the extracardiac conduit Fontan operation was achieved.

Implications:

• This case demonstrates the efficacy of a staged surgical strategy for complex single ventricle congenital heart disease.
• Early unifocalization and repeat pulmonary artery interventions are crucial for promoting pulmonary vascular growth.
• This approach offers a viable alternative for achieving Fontan completion in challenging pediatric cardiac cases.