Japanese Journal of Cardiovascular Surgery Vol46,No6

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

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