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 (89.5 s 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.

A 61-year-old woman had a history of deep vein thrombosis of the right leg at the age of 36 years. Primary antiphospholipid syndrome (APS) had been diagnosed at the age of 38 years, and rapidly progressive glomerulonephritis had developed at 54 year. She started hemodialysis one month before presentation due to deterioration of renal function. This time, she presented to the emergency department with paroxysmal nocturnal dyspnea. Echocardiography showed severe combined aortic stenosis and regurgitation (ASR). It was considered that the combination of ASR and construction of an arteriovenous fistula for dialysis had led to congestive heart failure. The patient had also experienced headache and agraphia for several days. Therefore, brain MRI was performed and multiple cerebral infarcts were detected. Early surgery should be considered for ASR, but we planned delayed surgery owing to the complication of acute cerebral infarction. During follow-up observation, a new asymptomatic cerebral infarct was detected. Eventually, aortic valve replacement (AVR) with a biological valve was performed on day 38 of hospitalization. Because she had highly active primary APS, surgery was performed with oral administration of aspirin, followed by continuous systemic heparinization from the early postoperative period. No perioperative thrombosis or bleeding was noted, and the patient was discharged uneventfully on postoperative day 34.