We have studied potential for pulmonary circulational assist by the dynamic Fontan model with a skeletal muscle ventricle (SMV) constructed using the latissimus dorsi muscles of 5 dogs. After 2 weeks of vascular delay, the SMV was electrically preconditioned for 8 weeks. Under cardiopulmonary bypass (CPB), the right heart (RV) bypass model was established with the SMV anastomosed between the right atrium and pulmonary trunk. The SMV was paced at a burst frequency of 25Hz, 60/min, with an asynchronization ratio. The aortic pressure (AoP), pulmonary arterial pressure (PAP), central venous pressure (CVP), and pulmonary flow (PAF) were measured. Just after on-SMV, PAP and PAF increased, CVP decreased. CVP decreased from 17±1.4mmHg to 13.5±0.7mmHg (p<0.05). PAP increased from 20±2.8/19±1.6mmHg (non-pulsatile flow) to 37.5±4.9/18±2.1mmHg (pulsatile flow). After CPB, pulmonary vascular resistance (Rp) showed 5.9±1.5 Wood units corresponding to a high risk factor for the Fontan procedure. On this Rp, under off-SMV the CVP was 18mmHg and severe RV failure was recognized as PAF was 35% of the preoperative value. Under on-SMV, CVP decreased and PAF was almost the same as the preoperative value. On physiological CVP, an RV bypass model with intrathoracic SMV maintained PAF at the preoperative value under high Rp. We concluded that this model may be a viable surgical option for univentricular heart with high Rp, which may not be Fontan candidates.

Preoperative evaluation of cardiac anatomy is essential to determine the correct surgical procedure for congenital heart disease. Multi-detector row CT (MDCT) is a useful alternative imaging modality to cardiac catheterization and echocardiography. Sixteen patients (12 with total anomalous pulmonary venous return (TAPVR) and 4 with aortic arch anomalies) underwent 16-slice multi-detector row CT scanning. Three-dimensional reconstruction by MDCT was useful to determine the type of TAPVR and the presence of pulmonary venous obstruction (PVO) in TAPVR patients, as well as to detect postoperative PVO in patients who underwent intracardiac repair. In 2 patients who had asplenia associated with TAPVR III and I a, MDCT enabled an intra-atrial approach for TAPVR repair by precise preoperative determination of the relationship between the common PV chamber and single atrium. In patients with aortic arch anomalies, MDCT was useful to determine the type of anomaly, the presence of arch hypoplasia, and any associated rare vascular anomalies (including isolated subclavian artery, and the right-sided descending aorta with left aortic arch). In conclusion, MDCT provides reliable preoperative evaluation of pulmonary venous return and aortic arch anatomy, and therefore is extremely useful for surgical management of congenital heart disease.

A 9-month-old boy who had been given a diagnosis of double outlet right ventricle (DORY), partial anomalous pulmonary venous return (PAPVR), ventricular septal defect (VSD), pulmonary hypertension (PH) and polysplenia with azygos connection, underwent pulmonary artery banding at the age of 6 months. At 2 months after surgery, a chest computed tomogram revealed a main pulmonary artery aneurysm and a main pulmonary artery-right pulmonary artery fistula caused by bacterial endocarditis due to a methicillin-resistant Staphylococcus epidermidis. We performed pulmonary arterioplasty and re-pulmonary artery banding for acute aggravation of cardiac insufficiency and obtained good results. This is an extremely rare case that was treated infectious pulmonary artery aneurysm and fistula after pulmonary artery banding.

Background: In Japan, although clinicians have been extremely interested in conducting clinical research, the shortage of clinical researchers is a serious problem. Therefore, it is important to explore barriers to conducting clinical research.1) We mailed a cross-sectional survey to hospital managers asking about their interest in and barriers to conducting clinical research and training clinical researchers at their hospitals.2) Of 810 eligible hospital managers, 301 completed questionnaires (response rate: 37.2%).3) The managers of university hospitals and national medical centers were more interested in conducting clinical research than were managers of other hospitals.4) Furthermore, 60.6% of managers of university hospital and 18.8% of managers of other hospitals reported the need to employ physicians who specialized in clinical research. However, given public research grants, about 50% of hospital managers were willing to employ research residents.5) Our results suggest there are still barriers to conducting clinical research, such as a lack of time set aside for clinicians and specialists to teach clinical research. A substantial strategy is needed to address the shortage of clinical researchers in Japan.

Chronic disseminated intravascular coagulation (DIC) complicates 5.7% of thoracic aortic aneurysm. DIC with thoracic aortic aneurysm is characterized by hyperfibrinolysis, but usually shows a stable condition in a state of compensated non-overt DIC with limited hemorrhagic symptoms. However, in some cases, hemorrhage caused by external factors may induce uncompensated overt DIC and lead to serious hemorrhagic tendencies. In the present study, we report a patient with a thoracic aortic aneurysm complicated by DIC who exhibited marked hemorrhagic tendencies. DIC remarkably improved following administration of recombinant human soluble thrombomodulin.

There has been significant progress in the understanding of the pathology and molecular biology of rare ovarian cancers, which has helped both diagnosis and treatment. This paper provides an update on recent advances in the knowledge and treatment of rare ovarian cancers and identifies gaps that need to be addressed by further clinical research. The topics covered include: low-grade serous, mucinous, and clear cell carcinomas of the ovary. Given the molecular heterogeneity and the histopathological rarity of these ovarian cancers, the importance of designing adequately powered trials or finding statistically innovative ways to approach the treatment of these rare tumors has been emphasized. This paper is based on the Rare Ovarian Tumors Conference for Young Investigators which was presented in Tokyo 2015 prior to the 5th Ovarian Cancer Consensus Conference of the Gynecologic Cancer InterGroup (GCIG).
Consensus ; Diagnosis ; Female ; Humans ; Molecular Biology ; Mucins ; Ovarian Neoplasms* ; Ovary ; Pathology ; Population Characteristics ; Rare Diseases ; Research Personnel*

Purpose: To examine the feasibility of the Integrated Palliative care Outcome Scale (IPOS), a comprehensive palliative care scale, for non-cancer patients. Methods: Twenty non-cancer patients and 20 healthcare providers who provide care to the participating patients were asked to complete the IPOS questionnaire, and cognitive interviews were conducted with them. The interviews were analysed using content analysis, a qualitative analysis method. Results: About half to 90% of both patients and healthcare providers answered that they did not find it difficult to answer or understand all items of the IPOS. Therefore, we confirmed its surface validity, and the content validity of the IPOS was confirmed. Conclusion: The surface validity and content validity of the IPOS for non-cancer patients were confirmed, and it became clear that the IPOS can be used as a comprehensive assessment tool for the palliative care of non-cancer patients.