[A case report of a ventricular septal defect with congenital portosystemic venous shunt].

A three-month-old baby boy was scheduled for a ventricular septal defect (VSD) repair. The patient was complicated with pulmonary hypertension (PH) and congenital portosystemic venous shunt (CPSVS). Because it was unclear whether the CPSVS was the main cause of the PH or not, the PH was treated with ordinary methods for the management of anesthesia for VSD patients with PH. He underwent the repair of the VSD, and his postoperative course was uneventful. The mechanism of PH in patients with CPSVS is different from that in those with VSD. We speculated that his pulmonary arteries were not affected with the CPSVS, because no PH was observed after the repair of VSD. Fresh frozen plasma was effective for hemostasis during weaning from cardiopulmonary bypass, because he could not produce enough coagulation factors.