An overview of pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries

The management of infants and children with pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries has proven to be challenging. Therapeutic approaches have included unifocalization, shunting, coiling of collateral vessels, and heart/lung transplantation. Results have been variable and frustrating. This review will cover the anatomy, physiology, initial medical management, subsequent surgical management, and controversies surrounding various therapeutic approaches. The evolving understanding of genetic considerations in this condition will also be explored.