Factor XI deficiency and aortic valve replacement: Perioperative management

Abstract

Severe factor XI deficiency (hemophilia C) is a rare coagulation disorder. A 73-year-old woman, a homozygote for factor XI deficiency, required aortic valve replacement. An initial dose of 15 U kg of factor XI concentrate was administered preoperatively and on postoperative day 3. During surgery, concentrated red cells, fresh frozen plasma, platelets, tranexamic acid, and fibrinogen were transfused. Intraoperative bleeding and total chest drainage were minimal. Postoperatively, there was no need for further transfusions and no bleeding or thrombotic complications occurred. The patient was well 16 months after surgery.

Perioperative anesthetic management in a patient with factor XI deficiency undergoing coronary artery bypass graft surgery

“…The use of FFP, factor concentrates, and antifibrinolytic agents are among the recommended treatment options to achieve this 4 . Thrombosis may occur in 10% of patients receiving factor concentrate due to the procoagulant and antifibrinolytic effects of FXI 5 . In 1984, Brunken et al, 6 were the first to report the successful management of perioperative bleeding during CABG surgery using FFP in two patients with FXI deficiency.…”

“…They noted that the bleeding was controlled only after FFP replacement. Petroulaki et al 5 used the preoperative infusion of an FXI concentrate, intraoperative FFP replacement, and tranexamic acid infusion to manage bleeding during an aortic valve replacement. Teruya et al 12 reported a female patient with severe FXI deficiency undergoing aortic valve replacement, who also underwent exchange transfusion and FFP infusion due to the presence of an inhibitor, however, massive bleeding and tamponade were observed in the postoperative period.…”

“…The majority of the patients with FXI deficiency are asymptomatic and are usually diagnosed after they are admitted to the hospital with excessive and unexpected bleeding following a surgical procedure or trauma. Therefore, FXI deficiency should be considered in the presence of a prolonged aPTT 5 …”

Perioperative management of rare coagulation factor deficiency states in cardiac surgery

“…A repeat dose (10-15 IU/kg) can be given every 72 h to maintain 30-40% FXI activity. 122 Normalization of FXI above 70% is not recommended because of the risk of thrombosis. 123 In one patient with severe FXI deficiency (2.9%) and aPTT 59.4 s undergoing aortic valve surgery, 15 IU/kg of FXI concentrate (Hemoleven) was given before surgery, normalizing aPTT and FXI level (36 s, and 54.2%, respectively; Table 5).…”

Management of severe factor XI deficiency in cardiac surgery: A case report and review of the literature

“…7 Two case reports described the management of FXI deficiency in the setting of aortic valve replacement. 8,9 One patient was managed successfully with FFP and tranexamic acid and the other with FXI concentrates without excessive bleeding. A paediatric case described a 4.5-year-old male with FXI deficiency underwent a mitral valvuloplasty that was supported with nine serial doses of rFVIIa at 90 mcg/kg IV without excessive perioperative bleeding 13 .…”