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

Emre Gok,Paul M Allison,Biswajit Kar,Hlaing Tint,Mehmet H Akay,Kimberly Klein,Ismael Salas De Armas,Bindu Akkanti,Alice J Chen,Igor D Gregoric

doi:10.1186/s40792-021-01256-x

Emre Gok, Paul M Allison + Show 8 more

Open Access

https://doi.org/10.1186/s40792-021-01256-x

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Abstract

BackgroundPatients 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 presentationA 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.ConclusionCardiac surgery in patients with AHA is possible as long as complex perioperative hemostatic and hematology management is used.

Highlights

Patients with acquired hemophilia A (AHA) who require open heart surgery have a life-threatening risk of hemorrhage
Cardiac surgery is often performed under CPB for which systemic anticoagulation with heparin is necessary; coagulopathy after CPB is complex and affects the perioperative management of AHA patients as they already suffer bleeding manifestations
We believe that porcine recombinant FVIII (rFVIII) is more effective than human-based versions for acquired hemophilia A because the porcine factor VIII’s protein sequence differs from human factor VIII and may be less inactivated by factor VIII inhibitors

Summary

Introduction

Patients with acquired hemophilia A (AHA) who require open heart surgery have a life-threatening risk of hemorrhage. Conclusion: Cardiac surgery in patients with AHA is possible as long as complex perioperative hemostatic and hematology management is used. Limited data are available to guide perioperative management of patients with AHA.

Results

Conclusion