[A successful surgical case of atrial septal defect with congenital factor XI deficiency].

We experienced a successful surgical case, who was a 16-year-old boy suffered from atrial septal defect with congenital factor XI deficiency. This disorder is an inherited disorder of blood coagulation characterized by a defect of the intrinsic pathway of thrombin formation and a mild to moderate bleeding tendency, but it can result in severe bleeding in surgical procedures. Open heart surgery in this disorders is rare. Preoperatively we prepared 4000 cc of fresh frozen plasma, but operated without using it. It would appear that the possibility of open heart surgery with congenital factor XI deficiency suggest in this case.