Coagulation management in endovascular aortic repair in a patient with congenital prekallikrein deficiency: a case report.

Prekallikrein deficiency is a rare autosomal recessive coagulation disorder that prolongs the activated clotting time. Guidelines for safe intraoperative coagulation control in patients with prekallikrein deficiency undergoing endovascular aortic repair are limited. We present a case of a 70-year-old man with prekallikrein deficiency who underwent endovascular aortic repair and required intraoperative anticoagulation control. Intraoperative anticoagulation with heparin and protamine was successfully managed using the activated clotting time measurements after preoperative administration of two units of fresh frozen plasma for prekallikrein replenishment. Preoperative prekallikrein replenishment by fresh frozen plasma administration contributed to safe intraoperative anticoagulation management.