Perioperative management of acute type A aortic dissection using extended half-life factor IX (Alprolix) in a patient with severe hemophilia B: a case report.

BACKGROUND: Emergency surgery for acute type A aortic dissection (ATAAD) in patients with severe hemophilia B is extremely rare, and perioperative coagulation management is a major challenge.

CASE PRESENTATION: A 63-year-old man with severe hemophilia B (factor IX activity < 1%) was diagnosed with ATAAD and underwent emergency surgery. Replacement therapy with an extended half-life factor IX product (eftrenonacog alfa; Alprolix) was administered targeting a factor IX activity of 80-100%, and total arch replacement was performed. Cardiopulmonary bypass was performed under standard heparinization conditions. Since rapid measurement of factor IX activity was not possible, postoperative replacement therapy was based primarily on the clinical assessment of hemostasis, with serial activated partial thromboplastin time measurements providing supportive information, whereas intraoperative anticoagulation was managed using standard activated clotting time monitoring. Intra- and postoperative bleeding volumes were within ranges comparable to those observed in standard complex aortic surgery, and the postoperative course was uneventful.

CONCLUSIONS: The use of an extended half-life factor IX product may be a practical option for perioperative coagulation management during emergency aortic surgery in patients with severe hemophilia B.