Strategy for pulmonary atresia and intact ventricular septum

Pulmonary atresia with an intact ventricular septum is characterized by varying degrees of right ventricular cavity hypoplasia. This factor is critical in determining the most appropriate surgical approach for each patient. We describe a patient who underwent definitive biventricular surgical repair in early infancy. We used an atrial septal defect patch with a one-way valve and performed a right ventricular overhaul after a balloon valvotomy.