Anesthetic Management of Total Arch Replacement in a Patient with Acute Aortic Dissection and Hemophilia A

Abstract

  A 61-year-old man with hemophilia A was diagnosed as having acute aortic dissection. After preoperative administration of 3,000 units of coagulation factor VIII (FVIII), coagulation laboratory data normalized (PT 86.3%, APTT 103%, FVIII 98.6%). FVIII (3.9 units/kg/hr) was continuously administered during surgery. Due to the heparin administered for cardiopulmonary bypass, PT, APTT, and FVIII values decreased to 57.9%, 10%, and 17.3%, respectively. After heparin reversal with protamine, however, hemostasis was difficult to achieve, and the PT and APTT values remained low. Hemostasis was eventually established, most likely due to the additional administration of FVIII (infusion rate up to 5 units/kg/hr and intermittent boluses of 500 to 1,500 units). FVIII was administered continuously until the 9th postoperative day (POD), and the FVIII level remained >100% after POD 2. The postoperative course was uneventful.