A 22-year-old woman without any serious distincted symptoms was found to have hypertension on a health examination. On further examinations, involving echocardiography and chest enhanced CT, showed dilatation of the ascending aorta, aortic coarctation, well-developed intercostal arteries and other collateral arteries. She was only 137 cm tall and weighed 52 kg. Besides, she had not had menstruation for the past two years. Chromosomal studies revealed Turner syndrome. Left lateral thoracotomy was thought to have the risk of heavy bleeding from collateral arteries, therefore we chose ascending-to-descending aorta bypass grafting through median sternotomy. She had an uncomplicated postoperative course. Here we report about operation in a adult case of coarctation of the aorta and discuss the usefulness of extraanatomical bypass grafting.

We report a successfully treated case of infectious endocarditis with ventricular septal defect (VSD) and double-chambered right ventricle. A 41-year-old man complained of dyspnea. Echocardiography showed his tricuspid valve, aortic valve, and pulmonary valve had vegetation and severe regurgitation. He received treatment with antibiotics but it was not effective. He underwent TVR, AVR, pulmonary valve resection, VSD patch closure and RV abnormal muscle resection. Pathological findings of resected valves showed infectious endocarditis. He recovered uneventfully and resumed his original social activities.

A 41-year-old woman was given a diagnosis of Behçet's disease at age 25. When she was 31, a large aortic pseudoaneurysm developed near the left renal artery. Isolation of the aneurysm and anatomical grafting and ancillary bypass were performed. Ten years later, a graft duodenal fistula developed. Extra-anatomical reconstruction was done after complete resection of the original graft and the infectious lesion. It was found that the intra-abdominal organs were receiving blood supply only from the inferiol mesenteric artery. Moreover, severe ischemia of the intra-abdominal organs was a concern during surgery. Therefore, hepatic vein oxygen saturation was monitored continuously with a Swan-Ganz catheter for ischemia of the intra-abdominal organs. It proved to be a very effective indicator and we could perform this operation safely. Reoperation of grafting is often inevitable in patients with Behçet's disease. Also, two stumps of abdominal aorta were left in this patient because of the extra-anatomical reconstruction. Pseudoaneurysm may later occur at the site of the stumps, thus necessitating careful follow-up observations.

A 52-year-old man was referred to our institution because of a solitary aneurysm of the left common iliac artery. The presence of the aneurysm was demonstrated by CT and angiography. The aneurysm had a diameter of about 30mm. The walls of the bilateral external iliac arteries were irregular, like a string of beads. The aneurysm was resected and replaced with a vascular prosthesis. Histological examination of the aneurysm and a part of the left external iliac artery indicated a diagnosis compatible with fibromuscular dysplasia (FMD: medial fibroplasia). A solitary iliac aneurysm is rare, and is usually due to atherosclerosis. However, the aneurysm in this case was related to FMD. To the best of our knowledge, only one other case of a solitary common iliac aneurysm caused by FMD has so far been described.

Intravenous leiomyomatosis (IVL) is defined as the extension into the venous channels of histologically benign smooth muscle tumors originating either from a uterine myoma or from the walls of a uterine vessel. We report a case of IVL extending to the right atrium and right ventricle through the right internal iliac vein and the inferior vena cava. The patient was a 43-year-old woman. The tumor was extirpated by simultaneous median sternotomy and laparotomy with the use of cardiopulmonary bypass. It was necessary to use cardiopulmonary bypass in order to open the right atrium. However, it proved difficult to insert the venous cannulae into the inferior vena cava due to the presence of the tumors. In order to perform the cannulation, a trans-right atrial excision of this tumor was necessary. Nevertheless, hemodynamic deterioration tended to occur during the procedure because of unexpected bleeding. We believe that to safely carry out this operation, it would be better to ensure circulatory arrest before trans-right atrial excision of the tumor. We have been continuing preventive antiestrogen therapy because recurrence would be very likely if any tumorous tissue remained after surgery. Fortunately, no intravenous infiltration of the tumor has been detected by either pelvic computed tomography or ultrasonography during the 26-month follow-up period. Surgical excision of the tumors and postoperative medication are now believed to have been effective.

The case was a 27-year-old woman with a history of Turner syndrome. The patient underwent ascending-descending aorta bypass for aortic coarctation 6 years previously and underwent subsequent follow-up on an outpatient basis. She consulted our department because of fever, chest pain and headache as the main complaints. Age-indeterminate type A aortic dissection was found on computed tomography, and she was admitted to the hospital on the same day. Echocardiography also revealed an enlarged aortic root and bicuspid aortic valve. Aortic root replacement and total arch replacement were performed, and her postoperative course was favorable. It is reported that in cases of Turner syndrome with aortic coarctation, aortic aneurysm and aortic dissection are likely to occur due to the vulnerability of the aortic wall. We encountered a patient with Turner syndrome who underwent ascending-descending aorta bypass for adult aortic coarctation and subsequently developed type A aortic dissection, underwent aortic root and total arch replacement, and rehabilitated after surgery, as well as provide bibliographic considerations.

A Task Force team consisting of members from pharmaceutical companies --a central player to develop and implement RMP (Risk Management Plan)-- as well as health care professionals and members from academia was established in JSPE. The Task Force developed guidance for scientific approach to practical and ICH-E2E-compliant Pharmacovigilance Plan (PVP) stated in Japanese Risk Management Plan issued in April 2012 by the Ministry of Health, Labour and Welfare. The guidance contains the following topics.
1．Introduction: JSPE's activities and this task force's objectives for pharmacovigilance activities
2．How to select Safety Specification (SS) and describe its characteristics
・Selection of SS
・Characterization of SS
・Association with Research Questions (RQ)
3．How to define and describe RQ
・What is RQ ?
・RQ interpretation in other relevant guidelines
・Methodology to develop RQ for PVP with examples
・Best approach to integrating PVP for whole aspects of safety concern
4．How to optimize PVP for specific RQ
・Routine PVP or additional PVP ?
・Additional PVP design (RQ and study design, RQ structured with PICO or GPP's research objectives, specific aims, and rationale)
・Checklist to help develop PVP
5．Epilogue:
・What can/should be “Drug use investigation” in the context of ICH-E2E-compliant PVP.
・Significance of background incidence rate and needs for comparator group
・Infrastructure for the future PVP activities
6．Appendix: Checklist to help develop PVP activities in RMP
The task force team is hoping that this guidance help develop and conduct SS and PVP in accordance with ICH E2E, as stated in Japanese Risk Management Plan Guideline.

BACKGROUND: Irisin is a myokine implicated in lipid and glucose metabolism. The objective of this study is to examine the effect of a body weight reduction on the serum irisin level and physical indicators in obese Japanese patients without diabetes. METHODS: The subjects were 22 patients (male/female, 5/17; age, 46.1±16.0 years; body mass index [BMI], 36.9±5.0 kg/m²) who completed a 6-month body weight reduction program at our clinic. The program included diet, exercise therapy and cognitive behavioral therapy. Blood parameters, body composition, exercise tolerance, homeostasis model assessment of insulin resistance (HOMA-IR), and serum irisin were determined before and after intervention, and relationships among changes in these data were examined. RESULTS: There were significant decreases in body weight and BMI after the intervention. Irisin before the intervention was significantly positively correlated with HOMA-IR (r=0.434, P<0.05). The mean irisin level showed no significant change after the intervention in all participants. However, improvements in % body fat, subcutaneous fat area, triglycerides, and fasting glucose were significantly greater in patients with an increase in irisin compared to those with a decrease in irisin after the intervention. Patients with an increase in irisin also had significantly lower fasting insulin (9.7±4.8 vs. 16.4±8.2, P<0.05) and HOMA-IR (2.2±1.1 vs. 3.7±1.6, P<0.05) after the intervention, compared to patients with a decrease in irisin. CONCLUSION: Body weight reduction did not alter irisin levels. However, irisin may play important roles in fat and glucose metabolism and insulin resistance, and the effects of body weight reduction on irisin kinetics may be a key for obesity treatment.
Adipose Tissue ; Asian Continental Ancestry Group ; Body Composition ; Body Mass Index ; Body Weight* ; Cognitive Therapy ; Diet ; Exercise Therapy ; Exercise Tolerance ; Fasting ; Glucose ; Homeostasis ; Humans ; Insulin ; Insulin Resistance ; Kinetics ; Metabolism ; Obesity ; Subcutaneous Fat ; Triglycerides

BACKGROUND AND OBJECTIVES: Reactive oxygen species (ROS) mediate various signaling pathways that underlie vascular inflammation in atherogenesis and cardiovascular diseases. Cardiac rehabilitation (CR) has a variety of multiple beneficial effects, including anti-inflammatory effects. The purpose of the present study was to investigate the effects of CR on ROS in patients with cardiovascular diseases. SUBJECTS AND METHODS: The serum level of derivatives of reactive oxidative metabolites, an index of oxidative stress, was measured in 100 patients with cardiovascular diseases before, and, subsequently, 3 and 6 months after, CR. A biological antioxidant potential (BAP) test was applied to assess the antioxidant power of the serum. RESULTS: The resting reactive oxidative metabolite levels decreased 3-6 months after CR {pre: 351+/-97 Carratelli unit (CARR U), 3 months: 329+/-77 CARR U, 6 months: 325+/-63 CARR U, all p<0.01} with the increase of the percentage of the predicted values of VO2 peak and the percentage of the predicted values of VO2 at the anaerobic threshold (VO2 AT) and the decrease of the B-type natriuretic peptide (BNP). The BAP test and antioxidative/oxidative stress ratio increased 6 months after CR. The % changes of the antioxidative/oxidative stress ratio was positively correlated with the % changes of VO2 AT, and negatively correlated with the % changes of the BNP. CONCLUSION: These results suggest that intensive supervised CR significantly improved exercise capacity, which may be attributable to an adaptive response involving more efficient oxidative metabolites or the increased capacity of endogenous anti-oxidative systems in patients with cardiovascular diseases.
Anaerobic Threshold ; Antioxidants ; Atherosclerosis ; Cardiovascular Diseases ; Exercise Therapy ; Humans ; Inflammation ; Natriuretic Peptide, Brain ; Oxidative Stress ; Oxygen Consumption ; Reactive Oxygen Species