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[A successful surgical case of atrial septal defect with congenital factor XI deficiency]
T Ichihara1, H Masumoto, M Sasaki
1Department of Cardiovascular Surgery, Okazaki Municipal Hospital, Aichi, Japan.
Insights
This case study highlights a successful open heart surgery on a 16-year-old boy with atrial septal defect and congenital factor XI deficiency, a rare bleeding disorder. The surgery proceeded without complications or the need for blood product transfusions.
Area of Science:
- Cardiovascular Surgery
- Hematology
- Congenital Disorders
Background:
- Congenital factor XI deficiency is an inherited coagulation disorder affecting the intrinsic pathway.
- It typically presents with mild to moderate bleeding tendencies, but can cause severe hemorrhage during surgery.
- Open heart surgery in patients with factor XI deficiency is exceptionally rare due to bleeding risks.
Abstract:
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.