Successful palliation and Fontan completion in a patient with double outlet right ventricle and intact ventricular septum

We report successful staged palliation of a rare cardiac anomaly: double outlet right ventricle with intact ventricular septum. Management included neonatal atrial septostomy, pulmonary artery banding, septectomy, lobectomy for congenital emphysema, and left ventricular exclusion to decompress the left ventricle. The patient progressed to a bidirectional Glenn and to a Fontan circulation, demonstrating a rare favourable outcome.