Aortic root repair in a patient with acquired hemophilia A: case report

Abstract Background Patients with acquired hemophilia A (AHA) who require open heart surgery have a life-threatening risk of hemorrhage. Limited data exist to guide perioperative management of these patients. Case presentation A 53-year-old female with rheumatoid arthritis, concomitant aortic valve...

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Bibliographic Details
Main Authors: Emre Gok, Mehmet H. Akay, Ismael Salas de Armas, Kimberly Klein, Hlaing Tint, Paul M. Allison, Alice J. Chen, Bindu Akkanti, Biswajit Kar, Igor D. Gregoric
Format: Article
Language:English
Published: Japan Surgical Society 2021-08-01
Series:Surgical Case Reports
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Online Access:https://doi.org/10.1186/s40792-021-01256-x
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Summary:Abstract Background Patients with acquired hemophilia A (AHA) who require open heart surgery have a life-threatening risk of hemorrhage. Limited data exist to guide perioperative management of these patients. Case presentation A 53-year-old female with rheumatoid arthritis, concomitant aortic valve endocarditis, and severe aortic regurgitation presented to our hospital. Bleeding and abnormal coagulation tests were noted during the initial workup, and she was diagnosed with AHA. The perioperative management plan included the use of pharmaceuticals, porcine recombinant factor VIII, and blood products. Extensive preoperative coagulation data were obtained, and factor VIII levels were continuously monitored to mitigate bleeding complications. The aortic valve replacement and root repair were uneventful. Conclusion Cardiac surgery in patients with AHA is possible as long as complex perioperative hemostatic and hematology management is used.
ISSN:2198-7793