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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.

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