止血に難渋した後天性血友病寛解状態にある肝移植後患者に対する開心術

Profuse Hemorage During Open Heart Surgery in a Patient Late After Liver Transplantation and Remission Stage Acquired Hemophilia

後天性血友病が寛解状態にある場合は，一般的に手術に対して特別な対応は不要であるとされているが，C型肝硬変を合併した血友病患者に肝移植を行い血友病の寛解にいたった症例に対する開心術の報告はない．われわれは，術中の出血に難渋し，半減期延長型第Ⅷ因子製剤により容易に止血を得た開心術例を経験したため報告する．

A 72-year-old male presented with severe mitral regurgitation, moderate tricuspid regurgitation, and chronic atrial fibrillation. One month prior, he encountered difficulties with dialysis and was subsequently referred to our department for cardiac surgery. The patient’s medical history includes living-donor liver transplantation for type C cirrhosis associated with acquired hemophilia A. The preoperative liver function was categorized as Child-Pugh grade B, with a model for end-stage liver disease （MELD） score of 23. His factor Ⅷ activity was close to the lower limit of the normal range. The patient underwent mitral valve replacement, tricuspid valve repair, and left atrial appendage closure. Initially, he experienced intractable bleeding, but hemostasis was easily achieved after administrating a factor Ⅷ preparation. Upon admission to the intensive care unit, his factor Ⅷ clotting activity was slightly below the normal range. Therefore, in cases where a patient with remission stage hemophilia A resulting in liver transplantation undergoes open heart surgery, it is crucial to have coagulation factor medication readily available, regardless of normal preoperative factor Ⅷ levels.