Unruptured sinus of Valsalva aneurysm is a rare anomaly which may be acquired or congenital. We describe a case of a 64 year old man with aneurysms (left : 31 mm, non : 21 mm) of the coronary sinus. The Bentall operation was successfully performed and his postoperative course was uneventful. The histopathology of the aortic wall showed Takayasu's arteritis. Bentall surgery is a good choice for multiple aneurysms of the Valsalva sinus with aortitis.

Between January 1991 and December 1998, we performed two successful procedures to repair abdominal aortic aneurysm with primary aortoenteric fistula. We had 197 surgical repair proceduers of aortic aneurysm during the same period. Incidence of primary aortoenteric fistula in abdominal aortic aneurysm was 1% in our institute. We performed primary closure of the fistula and removal of the possibily infected aneurysmal wall followed by anatomical grafting. We utilized omental wrapping for prophylaxis of potential graft infection. We achieved excellent surgical results in both patients by this approach.

A 27-year-old woman was given a diagnosis of infectious endocarditis with severe tricuspid regurgitation. Despite adequate antibiotics therapy, her general condition did not improve, and moreover multiple pulmonary abscesses were detected by computed tomography. Therefore surgery was indicated. Surgery consisted of removal of vegetation and tricuspid valve plasty with autologous pericardial patch augmentation of the anterior leaflet. Tricuspid valve plasty was carried out without prosthetic materials. Her postoperative course was uneventful with only mild tricuspid regurgitation. One year after surgery, neither recurrence of infection nor worsening of tricuspid regurgitation was noted. This method could be a useful technique for young patients with severe infection.

We report 2 patients with unilateral pulmonary edema after heart surgery who were successfully treated using venovenous extracorporeal membrane oxygenation (VV ECMO). Case 1 : A 35-year-old woman presented with dyspnea. Echocardiography showed severe mitral regurgitation (MR) and tricuspid regurgitation (TR) and therefore, mitral valve plasty (MVP) and tricuspid annular plasty (TAP) were performed via right thoracotomy. After weaning from cardiopulmonary bypass, respiratory failure occurred with expectoration of foamy sputum and it was difficult to maintain oxygenation. Therefore, we performed VV ECMO to maintain oxygenation. A chest X-ray film after surgery showed ipsilateral pulmonary edema. After weaning from VV ECMO, deep venous thrombosis occurred and therefore we inserted an IVC filter. Case 2 : A 67-year-old man, who had previously received aortic valve replacement experienced dyspnea and visited our hospital. Echocardiography showed an aortic root abscess, and therefore Bentall operation was performed. After weaning from cardiopulmonary bypass, oxygenation was difficult to maintain, and therefore we performed VV ECMO. A chest X-ray film post operatively showed right ipsilateral pulmonary edema. The patient was weaned from VV ECMO 5 days post operatively and was discharged 60 days post operatively. We believe that VV ECMO can be beneficial for patients with respiratory failure after heart surgery, but complications related to this approach such as DVT should also be considered.

We report a patient who underwent an operation for an infectious abdominal aortic aneurysm 10 months after intravesical bacillus Calmette-Guerin therapy. A 68-year-old man had previous gastrectomy for early gastric cancer and intravesical BCG therapy for early stage urinary bladder cancer. His follow up CT scan revealed an abdominal aorta pseudoaneurysm. We performed aneurysmectomy, omentopexy and bilateral axillo-femoral bypass. The culture of an abscess in the aneurysm identified Mycobacterium bovis. The patients improved clinically with antituberculosis agents after operation. Intravesical bacillus Calmette-Guerin therapy is effective in the treatment of early stage urinary bladder cancer. Although this treatment is generally considered safe, serious complications including vascular complications have been reported.

A 47-year-old man had suffered from high grade fever and dyspnea for 10 days. He was transferred to our hospital in a condition of shock. Echocardiography showed severe diffuse hypokinesis of left ventricle (EF 21%), and multiple mobile thrombi in the left ventricle. Under a diagnosis of LV thrombi due to acute myocarditis, transatrial removal of LV thrombi was performed using video-assisted cardioscopy. He was weaned from cardiopulmonary bypass under IABP support. Postoperatively, he suffered from thromboembolism of the cerebral and right brachial artery. Thrombectomy of the right brachial artery and anticoagulation therapy was performed. IABP was removed on POD 3, and he no longer needed respiratory control on POD 4. Echocardiography on POD 6 showed marked improvement of the LV contraction (EF 52%). After rehabilitation, he was discharged on POD 23 on foot. Video-assisted cardioscopy allowed transatrial removal of LV thrombi, and preserved left ventricular function by avoiding ventriculotomy. Perioperative thromboembolism must be taken care of for a patient with multiple LV thrombi.

Introduction: We report a case of sudden loss of consciousness due to hypoglycemia in the terminal stage of cancer. Case: A 73-year-old man underwent craniotomy in 2016 and was diagnosed with solitary fibrous tumor. In 2022, his brain tumor recurred and he was admitted to our hospice. On the morning of the 120th day after transfer, the patient suddenly developed impaired consciousness. Blood tests showed hypoglycemia (33 mg/dL), and intravenous glucose injection improved his loss of consciousness, leading to a diagnosis of impaired consciousness due to hypoglycemia. After various examinations, hypoglycemia due to extrapancreatic tumor (non-islet cell tumor hypoglycemia: NICTH) was strongly suspected. Discussion: NICTH is considered to be caused by excessive secretion of an insulin-like substance (high molecular weight insulin-like growth factor (IGF-)II) from the tumor. In patients who are exposed to hypoglycemia for a long period of time, central nervous system symptoms such as impaired consciousness may suddenly develop without sympathetic symptoms. NICTH should be considered as a differential diagnosis in patients with delirium, impaired consciousness, or convulsive seizures and a large tumor, although it is not easy to prove the excessive secretion of high-molecular-weight IGF-II, especially in the terminal stage of cancer.

Autoimmune enteropathy (AIE) is a rare disease, characterized by intractable diarrhea, villous atrophy of the small intestine, and the presence of circulating anti-enterocyte autoantibodies. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome, and mutations in FOXP3, which is a master gene of regulatory T cells (Tregs), are major causes of AIE. Recent studies have demonstrated that mutations in other Treg-associated genes, such as CD25 and CTLA4, show an IPEX-like phenotype. We present the case of a 13-year-old girl with CTLA4 haploinsufficiency, suffering from recurrent immune thrombocytopenic purpura and intractable diarrhea. We detected an autoantibody to the AIE-related 75 kDa antigen (AIE-75), a hallmark of the IPEX syndrome, in her serum. She responded well to a medium dose of prednisolone and a controlled dose of 6-mercaptopurine (6-MP), even after the cessation of prednisolone administration. Serum levels of the soluble interleukin-2 receptor and immunoglobulin G (IgG) were useful in monitoring disease activity during 6-MP therapy. In conclusion, autoimmune-mediated mechanisms, similar to the IPEX syndrome, may be involved in the development of enteropathy in CTLA4 haploinsufficiency. Treatment with 6-MP and monitoring of disease activity using serum levels of soluble interleukin-2 receptor and IgG is suggested for such cases.