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.

This study reports a 38-year-old man who suffered traumatic thoracic aorta injury due to a fall accident during mountain climbing, and was saved by swift helicopter transport and emergency surgery. Approximately 2 h after the accident, the subject was brought to the hospital by the emergency helicopter transport service. Contrast-enhanced CT of the thorax and abdomen revealed leakage of the contrast medium from the aortic isthmus and a hematoma in the surrounding area. Thus, traumatic thoracic aorta injury was diagnosed and emergency surgery was performed. The patient went into a state of shock after being given anesthesia. Further rupture was diagnosed and a left fourth intercostal thoracotomy was performed immediately in order to control the hemorrhage. Concurrently, a partial cardio-pulmonary bypass was used. A lacerated foramen was observed across 1/3 of the lesser curvature of the aortic isthmus ; the affected site was replaced by a prosthetic graft. The postoperative recovery was generally uneventful, and the patient was discharged from the hospital 30 days after the operation.

Background : Several studies have shown that Fontan circulation may lead to liver congestion and possible structural liver alteration. The aim of this study is to analyze the relationships between biochemical fibrosis markers and hemodynamic parameters in the long term after the Fontan operation.
Methods : The study enrolled 51 patients who underwent total cavopulmonary connection between March 1994 and July 2010. We analyzed the relationships between the 5 liver fibrosis markers (hyaluronic acid, retinol-binding protein, procollagen type III peptide, type IV collagen 7S, type IV collagen) and the 6 hemodynamic parameters (pulmonary artery pressure, pulmonary artery index, pulmonary vascular resistance, ejection fraction, atrioventricular valve regurgitation, cardiac index).
Results : Hyaluronic acid and type IV collagen 7S positively correlated with pulmonary artery pressure. Hyaluronic acid negatively correlated with ejection fraction, and type IV collagen 7S positively correlated with atrioventricular valve regurgitation in patients followed up for more than 8 years after Fontan completion. Pulmonary artery pressure was significantly higher in patients in whom type IV collagen 7S was elevated. Hyaluronic acid correlated with pulmonary vascular resistance (p=0.0035) and ejection fraction (p=0.014), as well as type IV collagen 7S with pulmonary artery pressure (p=0.0001) by multiple regression analysis.
Conclusion : Hyaluronic acid and type IV collagen 7S reflected the degree of hepatic congestion, and cardiac function, in the long term after the Fontan operation.

We report a case of syphilitic aortitis (SA) associated with severe right coronary ostial stenosis, aortic regurgitation (AR), and annuloaortic ectasia (AAE). A 48-year-old man presented to a regional hospital with easy fatigability and nocturnal dyspnea. Echocardiography revealed Seller's grade 3 AR. A computed tomography scan showed AAE, dilatation of the ascending aorta, and calcification of both coronary ostia. Coronary angiography demonstrated that the left coronary artery was intact ; however, the right coronary artery was obscure. Active syphilis was detected on routine blood tests on admission. Therefore, the patient was started on a course of ampicillin/sulbactam (ABPC/SBT). Subsequently, he underwent the Bentall procedure and coronary artery bypass grafting with the right internal thoracic artery. The intraoperative findings showed degeneration of the aorta and severe right coronary ostial stenosis. The pathological findings of the aortic wall and aortic valve were consistent with SA. The postoperative course was uneventful. The patient continued receiving ABPC/SBT for 3 weeks postoperatively, and was then switched to oral amoxicillin.

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.