A 70-year-old man was admitted with syncope attack on exertion. Ventricular septal defect with pulmonary hypertension and left atrial myxoma were confirmed by ultrasonography and cardiac catheterization. Preoperative Pp/Ps was 0.95 and pulmonary vascular resistance was 16 units. Pulmonary vascular resistance decreased to 9.6 units by the administration of Isoproterenol and decreased to 8.5 units with PGE1. Patch closure of VSD and excision of left atrial myxoma were performed simultaneously. The patient recovered completely, although he suffered from pneumonia and jaundice due to liver congestion postoperatively. Cardiac catheterization before discharge revealed Pp/Ps 0.38 and PVR 10.1 units.