Pulmonary Atresia,Ventricular Septal Defect,and Major Aortopulmonary Collateral Arteries: The Natural History and Late Presentation

Abstract

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries represents a heterogenous and complex congenital heart defect. Treatment pathways vary widely depending on center expertise. The recommended approach in infancy is to achieve uniform pulmonary artery and capillary bed development, and a biventricular repair when feasible. The natural history of this condition is particularly relevant in low-middle income countries where early intervention is not possible due to poor access to cardiac surgical care. This group of patients may present in late childhood or as adults with symptoms of chronic cyanosis that may be associated with cardiac failure. The surgical approach to this group may range from complete correction to pulmonary artery rehabilitation or unifocalization, or consideration for heart-lung transplantation.