[A case of ventricular septal defect and patent ductus arteriosus associated with absent right pulmonary artery, scimitar syndrome and severe pulmonary hypertension].

A 8 month-old female was diagnosed with ventricular septal defect and patent ductus arteriosus associated with absent right pulmonary artery and scimitar syndrome. Cardiac catheterization revealed severe pulmonary hypertension as follows; 72 mmHg of mean pulmonary artery pressure, 0.56 Qp/Qs and 1.73 Pp/Ps. Temporary closure of the ductus reduced the mean pulmonary artery pressure from 72 to 40 mmHg. PDA was ligated and VSD was closed successfully. However, 4 months after initial operation she was readmitted due to infection of the hypoplastic right lung. Removal of the hypoplastic right lung was performed and postoperative course was uneventful.