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.
- 1Department of Cardiovascular Surgery, Sasebo Chuo Hospital, 15 Yamato, Sasebo City, Nagasaki, 857-1195, Japan. [email protected].
- 2Department of Cardiovascular Surgery, Sasebo Chuo Hospital, 15 Yamato, Sasebo City, Nagasaki, 857-1195, Japan.
Abstract
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.
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BibTeX
@article{kitamura2026perioperative,
title = {{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.}},
author = {Tessho Kitamura and Yutaro Ryu and Shun Nakaji and Shinichiro Taniguchi},
journal = {General thoracic and cardiovascular surgery cases},
year = {2026},
month = oct,
doi = {10.1186/s44215-026-00282-1},
url = {https://europepmc.org/article/MED/42902440},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - 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.
AU - Tessho Kitamura
AU - Yutaro Ryu
AU - Shun Nakaji
AU - Shinichiro Taniguchi
PY - 2026
DA - 2026/10/20
JO - General thoracic and cardiovascular surgery cases
DO - 10.1186/s44215-026-00282-1
UR - https://europepmc.org/article/MED/42902440
AB - 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.
SN - 2731-6203
ER -