A 38-year-old man underwent surgery for impending rupture of an inflammatory celiac artery aneurysm with a maximum diameter of about 50 mm. First, an extra-anatomical bypass was performed from the iliac arteries to the celiac artery, superior mesenteric artery and bilateral renal artery using ringed ePTFE grafts. Second, the celiac artery aneurysm at the distal site was directly closed and then a stent graft was placed in the abdominal aorta to cover the orifice of the celiac artery. An endovascular stent graft treatment combined with extra-anatomical bypass is useful for the treatment of inflammatory aneurysm to avoid the various surgical complications in Behçet syndrome.

A 46-year-old man was given a diagnosis of hypertension about 20 years previously. At age 41, aortitis syndrome was diagnosed, with descending thoracic aortic aneurysm and the coarctation of abdominal aorta by CT scan. He then underwent surgery to replace the descending thoracic aortic aneurysm and right axillo-bifemoral bypass. Recently, a thoraco-abdominal aortic aneurysm was pointed out at the distal site of the graft and, he was referred to our institute. We occluded the distal end of the aneurysm using an endoluminal occlusion stent graft. Today, in most cases of aortopathy associated with aortitis syndrome, surgical replacement of the aneurysms and extra-anatomical bypass is performed. An endovascular stent graft treatment combined with extra-anatomical bypass could be useful for various aortic disorders.

We report two cases of coronary artery fistulas (CAF) in children with successful surgical repair. The first case was a 14-year-old boy with a continuous heart murmur. A fistula was diagnosed between the right coronary artery (RCA) and the right atrium by echocardiography. He was asymptomatic, but the pulmonary to systemic flow ratio was shown to be high by cardiac catheterization. He underwent surgical closure of the fistula and coronary artery bypass grafting to the RCA with the right interthoracic artery. The second case was a 5-year-old boy with a continuous heart murmur and cardiomegaly on the chest X-ray film. The CAF from the left coronary artery to the right atrium was revealed by echocardiography. The pulmonary to systemic flow ratio was shown to be high by cardiac catheterization. He was asymptomatic, but he also underwent surgical closure of CAF. Surgical repair was effective and safe in these cases. Echocardiography was very useful in the diagonosis of CAF. Surgical repair in the young is recommended because CAF is much more likely to cause congestive heart failure, angina, and infective endocarditis when growing.

Preoperative hemodynamics in the lower limbs in 174 limbs of 103 patients were measured and investigated and the surgical results were evaluated. The subjects were divided into the C_4-6-group, which included 51 limbs with skin changes, and the C_{2, 3}-group, which consisted of 123 limbs without skin changes. Plethysmography showed that the maximum venous outflow was significantly higher, and the refilling time was shorter in the C_4-6-group compared to the C_{2, 3}-group. All C_4-6-group patients had reflux of the long saphenous vein, and the reflux velocity and volume was significantly greater than in the C_{2, 3}-group. Limbs with deep vein reflux accounted for 45% of the C_4-6-group. We performed angioscopic deep vein external valvuloplasty in 23 of those limbs. There was no case of reccurence of skin changes at a mean follow-up of 22 months. However chronic venous insufficiency often was associated with multisegmental reflux lesions and in such cases it might be necessary to perform multiple surgical procedures to eliminate the reflux.

We report a rare case of vasculo-Behçet's disease complicated by an intracardiac thrombus. A 48-year-old woman with a history of Behçet's disease and deep vein thrombosis in a lower limb, and who had been treated with colchicine and warfarin, was given a diagnosis of an appendectal tumor by colonoscopy. Preoperative examinations, including chest computed tomography, showed a pedunculated mobile tumor from her inferior vena cava (IVC) to the right atrium (RA). Urgent surgery was performed to prevent pulmonary embolism. We exposed the RA through a median sternotomy under cardiopulmonary bypass and extirpated the tumor that appeared macroscopically to be an organized thrombus attached to the IVC wall. The pathological diagnosis was organized thrombus. A month later, she underwent ileocecal resection and was given a pathological diagnosis of mucinous cystadenoma. Her postoperative course was uneventful. Intracardiac thrombus complicated by vasculo-Behçet's disease is rare, and it is important in the differential diagnosis of intracardiac tumor.

A 34-year-old woman had received a diagnosis of X-linked hypophosphatemic osteomalacia when she was born. As an adult she complained of general fatigue, palpitations dyspnea exertional and leg edema. Transthoracic echocardiography showed aortic stenosis and regurgitation with severe aortic valve calcification. Chest computed tomography revealed her ascending aorta to be circumferentially calcified. Surgery was performed through a median sternotomy via cardiopulmonary bypass, with perfusion from the right subclavian artery and the right femoral artery and drainage from the superior and the inferior venae cavae. A left ventricular venting cannula was inserted from the right superior pulmonary vein. When her bladder temperature had dropped to 28°C by central cooling, we stopped the perfusion from the right femoral artery and performed aortotomy. We examined the lumen and clamped the aorta at the usual site, while flushing with blood by femoral perfusion. Aortic valve calcification was observed to extend from the sinus of valsalva to the outflow tract of the left ventricle on the non-coronary cusp side. He was necessary to repair the aortic annulus due to a defect resulting from careful removal of the calcified valve. We then performed aortic valve replacement using a 19-mm Carpentier-Edwards perimount Magna. Her postoperative course was uneventful without any sign of neurological complications, and she was discharged 13 days after surgery. On pathological examination, localized ossification with calcification was observed in her aortic valve which seemed to be characteristic of X-linked hypophosphatemic osteomalacia.

A 65-year-old man was admitted with subacute myocardial infarction. During medical treatment, the patient lost consciousness as a result of an atrioventricular block and underwent an operation for an emergency percutaneous coronary intervention in the right coronary artery. In a follow-up examination, transthoracic echocardiography and computed tomography showed a left ventricular pseudo-false aneurysm, and therefore another operation was carried out. The operative findings showed that the heart markedly adhered to the pericardium and the aneurysm at the apex. The patient then underwent a double-patch closure of the ruptured point using an equine pericardial patch and a Dacron patch. No perioperative complication was observed. Left ventricular pseudo-false aneurysm is a rare complication following myocardial infarction. Here, we report a successful case of a double-patch closure of a pseudo-false aneurysm.

To investigate the effect of nafamostat mesilate (FUT) for disseminated intravascular coagulation (DIC) after surgery using cardiopulmonary bypass, we studied DIC scores and parameters of coagulation and fibrinolysis in the DIC cases. Although 12 patients developed DIC, the platelet counts improved by administration of FUT apart from one complicated by sepsis. The DIC scores decreased as a result of the increase of platelets and fibrinogen and improvement of FDP. Thrombin-antithrombin III complex, D-dimer and plasmin-α₂ plasmin inhibitor complex showed an even higher value at the endpoint of FUT administration. These results indicate that patients with DIC after cardiopulmonary bypass may have severe fibrinolytic acceleration and that administration of FUT can be useful in those cases.

This is a case report of a rare combination of a single coronary artery and a coronary artery fistula. An 8-year-old girl was asymptomatic with a grade II continuous murmur in the third left intercostal space. Selective coronary angio-graphy revealed that a single coronary artery arising from the left aortic sinus was dilated and ended as a fistula to the outflow tract of the right ventricle. At operation, the fistula could not be exposed on the surface of the heart. On cardiopulmonary bypass, the aorta was clamped, and the fistula was closed by direct suture in the right ventricle. The postoperative course was uneventful with no complications.

The activated clotting time (ACT) is used to assess adequacy of anticoagulation during cardiopulmonary bypass (CPB). However, ACT values during CPB do not correlate with heparin concentration and are affected by variations of such factors as hypothermia and hemodilution. ACT is also used to estimate protamine doses, because excess protamine may result in hypotension and an increase in bleeding after CPB. This study was designed to evaluate the effect of heparin and protamine administration that were administered based on whole blood heparin concentration using Hepcon/HMS (HC group) on the incidence of bleeding and blood transfusion after CPB. We treated 32 of adult cases and 36 pediatric cases. For the control group (NC group), an initial fixed dose of 300U/kg heparin was administered and if the ACT was less than 400s an additional fixed dose of 100U/kg heparin was administered. Heparin was neutralized with an initial fixed dose of protamine. For the HC group, the initial dose of heparin and the additional dose of heparin were based on an automated heparin dose response assay. The initial dose of protamine was based on the residual heparin concentration. The patients in the HC group received greater doses of heparin and lower doses of protamine than the patients in the NC group. In the pediatric HC group, the amount of TAT, FTC and D-dimer post CPB were smaller than those in the NC group. Operative time and closure time were similar the two groups. Operative bleeding, mediastinal chest tube drainage in the postoperative period were similar in the two groups. The volume of total blood transfusion was also comparable in the two groups. In conclusion, the monitoring of heparin concentration during CPB in children was effective for the maintenance of coagulation factors.