The patient is an 8-week-old boy born with cyanotic congenital heart disease and severe dextroversion of the heart. Echocardiographic diagnosis of hypoplastic right heart syndrome was made at a local hospital, and the patient was then transferred to the New York University Medical Center. This video depicts the key catheterization and echocardiographic findings in this case as well as operative repair with replacement of the right ventricular outflow tract, pulmonary valve and main pulmonary artery with a 14-mm human homograft, ligation of the ductus arteriosus, closure of the atrial septal defect, and separation of fused tricuspid papillary muscles. Subsequent follow-up echoes are shown as well.