Scimitar syndrome in an infant with right lung hypoplasia, ventricular septal defect, and severe pulmonary hypertension

A 5-month-old boy was presented for surgical repair of scimitar syndrome associated with right lung hyperplasia, severe pulmonary hypertension, ventricular septal defect (VSD), and atrial septal defect. The calculated shunt fraction (Qp/Qs) was 3.1:1.0, pulmonary vascular resistance was 4.6, and the perfusion lung scan showed a marked decrease (11%) in right pulmonary blood flow. Surgical repair was performed through the right fourth intercostal space with the patient supine. The anomalous vein was divided and interposed with a short azygos vein graft, followed by closure of the VSD. Finally, the interposed azygos vein was anastomosed to the left atrium. Although pulmonary artery pressure was normalized at the 2-year follow-up, cardiac catheterization 6 months after the operation demonstrated right pulmonary vein obstruction.