Off-Pump Coronary Artery Bypass Graft in a Patient with Congenital Factor V Deficiency and Hereditary Spherocytosis Complicated with Stage 4 Diabetic Nephropathy

Saori Nagura＊	Kazuaki Fukahara＊	Mari Sakai＊
Toshio Doi＊	Shigeki Yokoyama＊	Kimimasa Sakata＊
Hayato Obi＊	Naoki Yoshimura＊

（First Department of Surgery, University of Toyama＊, Toyama, Japan）

A 64-year-old man with congenital factor V deficiency and hereditary spherocytosis was attending our hospital for type II diabetes and stage 4 diabetic nephropathy. Coronary angiography performed to assess chest pain revealed severe triple-vessel disease, including total occlusion of the right coronary artery. The patient required surgical coronary revascularization. In the preoperative examination, the activated partial thromboplastin time（APTT）and prothrombin time-international normalized ratio（PT-INR）were high（89.5s and 1.95）and factor V activity was low（6%;normal range, 70-135%). Hemodialysis was performed on the day of the operation, and 6 units of fresh frozen plasma（FFP）were administered, which reduced immediately the preoperative PT-INR to 1.33. We performed off-pump coronary artery bypass grafting（OPCAB）and perioperatively administered 6 units of FFP with 4 units of red blood cells（RBC）transfusion. The postoperative course of the patient was uneventful, and he was discharged on postoperative day 22. Here we report the case of a patient with a very rare disease of congenital factor V deficiency and hereditary spherocytosis complicated with stage 4 diabetic nephropathy who required OPCAB.

 

Jpn. J. Cardiovasc. Surg. 46:296-300（2017）

Keywords：factor V deficiency;hereditary spherocytosis;diabetic nephropathy;off pump coronary artery bypass grafting