Aortic atresia with normal left ventricle. Single-stage repair in the neonate

Four to seven percent of infants born with aortic atresia have a normal-sized left ventricle in association with a ventricular septal defect and a normal mitral valve. In contrast to the more common group of infants with aortic atresia whose left ventricle is hypoplastic, this important subgroup has potential for complete operative correction involving both the right and left ventricles. Previous reports of surgical management of these infants have described early palliative procedures to control systemic and pulmonary blood flow. Our approach has been to perform complete repair at one stage in the neonate. Continuity between the proximal main pulmonary artery and aorta is established, a patch on the ventricular septal defect baffles left ventricular blood to the pulmonary valve, and a homograft conduit achieves right ventricular-pulmonary artery continuity. Three of four neonates who underwent single-stage repair were discharged from the hospital in good condition. Achieving a physiologically normal circulation simplifies the postoperative management of this condition and is to be preferred over neonatal palliation.