Coronary artery fistulas in pulmonary atresia and ventricular septal defect

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Cardiac Surgery

Background:

• Pulmonary atresia with ventricular septal defect (PA/VSD) presents complex and variable anatomy.
• Coronary artery-to-pulmonary artery fistulas are a rare anomaly contributing to pulmonary blood flow in PA/VSD.
• Management strategies for this combined defect require careful consideration.

Purpose of the Study:

• To describe the management and outcomes of patients with PA/VSD and coronary artery-to-pulmonary artery fistula.
• To evaluate the efficacy of direct fistula closure and single-stage intracardiac repair.

Main Methods:

• Retrospective review of 4 pediatric patients treated between 1996 and present.
• Surgical management involved direct internal closure of the coronary-pulmonary fistula.
• Simultaneous total intracardiac repair was performed in all cases.

Main Results:

• Four patients with PA/VSD and coronary-pulmonary fistulas were treated.
• Fistulas originated from left coronary, right coronary, or solitary coronary systems, all draining into the main pulmonary artery.
• One patient experienced mortality; the remaining three had successful single-stage repairs.

Conclusions:

• Direct internal closure of coronary artery-to-pulmonary artery fistulas is a viable strategy in PA/VSD.
• This approach facilitates successful single-stage intracardiac repair in pediatric patients with favorable anatomy.
• Coronary-pulmonary fistulas require specific management within the context of PA/VSD repair.