A 52-year old woman was referred to our hospital because of abdominal pain and restlessness. A chest contrast-enhanced CT showed huge pericardial effusion and intraluminal defects in the main pulmonary artery. We could not make a diagnosis based on the cytology of the pericardial effusion and histopathology of the mass with a sample taken by a catheter. Therefore, we undertook biopsies of the mass by median sternotomy, which led to the diagnosis of pulmonary intimal sarcoma. The tumor resection was performed to release the right ventricular outflow stenosis. We tried to resect the tumor as much as possible, and reconstructed the pulmonary artery and aortic root. She was discharged to home and survived 5 months after surgery.

BACKGROUND: The Japan Environment and Children's Study (JECS) is a nation-wide birth cohort study investigating environmental effects on children's health and development. In this study, the exposure characteristics of the JECS participating mothers were summarized using two questionnaires administered during pregnancy. METHODS: Women were recruited during the early period of their pregnancy. We intended to administer the questionnaire during the first trimester (MT1) and the second/third trimester (MT2). The total number of registered pregnancies was 103,099. RESULTS: The response rates of the MT1 and MT2 questionnaires were 96.8% and 95.1%, respectively. The mean gestational ages (SDs) at the time of the MT1 and MT2 questionnaire responses were 16.4 (8.0) and 27.9 (6.5) weeks, respectively. The frequency of participants who reported "lifting something weighing more than 20 kg" during pregnancy was 5.3% for MT1 and 3.9% for MT2. The Cohen kappa scores ranged from 0.07 to 0.54 (median 0.31) about the occupational chemical use between MT1 and MT2 questionnaires. Most of the participants (80%) lived in either wooden detached houses or steel-frame collective housing. More than half of the questionnaire respondents answered that they had "mold growing somewhere in the house". Insect repellents and insecticides were used widely in households: about 60% used "moth repellent for clothes in the closet," whereas 32% applied "spray insecticide indoors" or "mosquito coil or an electric mosquito repellent mat." CONCLUSIONS We summarized the exposure characteristics of the JECS participants using two maternal questionnaires during pregnancy.
Adult ; Child Health ; Cohort Studies ; Female ; Gestational Age ; Humans ; Japan ; Maternal Exposure ; statistics & numerical data ; Mothers ; statistics & numerical data ; Pregnancy ; Surveys and Questionnaires ; Young Adult

Background : Connective tissue disease (CTD) is an idiopathic autoimmune disorder which causes systemic chronic inflammation. Inflammation causes various cardiovascular diseases. Systemic steroid use, which is usually the sole treatment for CTD, also causes arteriosclerosis. Although cardiovascular surgery is often necessary in patients with CTD, preexisting multiple organ dysfunction related to CTD, in addition to systemic administration of steroids or other immunosuppressants, is thought to increase the risk of surgery. However, little is known about how the disease process of CTD influences early and late cardiovascular surgery outcomes. Methods : To better understand these issues, we reviewed 31 patients with CTD (study group) and compared their outcomes to those of other patients (control group) who underwent cardiovascular surgery at our institution between April 2008 and November 2013. Results : There were 26 women and 5 men, and the average age was 64.4±16.7 years. CTD types included rheumatoid arthritis in 7 patients, systemic lupus erhythematosus in 6, aortitis syndrome in 6, polymyalgia rheumatica in 3, scleroderma in 3, polymyositis in 3, and others. The procedures included 10 valve cases, 10 coronary artery bypass grafting (CABG) or CABG-valve combination cases, and 11 isolated or complicated thoracic aortic surgery cases. Prior to undergoing these procedures, 24 patients (77.4%) were treated with steroids and/or immunosuppressant, and 6 patients had been diagnosed with interstitial pneumonia in the study group. Moreover, the rate of peripheral artery disease and carotid artery stenosis in the study group was significantly higher than that in the control group. There were no perioperative deaths in the study group. There were no significant differences in terms of major complications such as ischemic events, infection, acute kidney injury, lung injury, and others between the groups. We conducted a follow-up survey for the study group with an average period of 27.8±16.0 months. During the follow-up period, there were 4 late deaths. In addition, 8 patients required readmission, 6 for cardiovascular events and 2 for poor wound healing. All the survivors in the study group showed improved cardiac function and were in the NYHA functional class I and II. Conclusion : Cardiovascular surgery for patients with CTD can provide acceptable early and mid-term results.

Total anomalous pulmonary venous connection (TAPVC) is rarely associated with remarkably small left heart structures. In these types of cases, the hemodynamics resembles that of hypoplastic left heart syndrome, and the treatment strategy is controversial. We present the case of a 1-day-old girl with infracardiac TAPVC, small left heart structures (hypoplastic left heart complex), bilateral superior vena cava, and aberrant origin of the right subclavian artery. We performed a semi-emergent first-stage open palliation for repair of TAPVC, because of pulmonary venous obstruction. We concomitantly performed atrial septal defect (ASD) enlargement and bilateral pulmonary artery banding (BPAB). The postoperative course was uneventful and the left heart structures did not grow, so we performed the Norwood procedure and placed a right ventricle-pulmonary artery shunt with a 5.0 mm artificial graft. Subsequently, the left heart structures were not suitable for biventricular repair, so we chose univentricular repair. The patient underwent a bilateral bidirectional Glenn operation and Fontan completion at 6 and 23 months of age, respectively. TAPVC repair, BPAB, and ASD enlargement are reasonable surgical options for a patient with borderline small left heart structures and TAPVC, as they enable us to wait for growth in the left heart structures and to determine whether univentricular or biventricular repair is suitable.

Objective : The aim of this study is to describe a series of patients undergoing reoperation due to hemolytic anemia after mitral valve surgery and assess the mechanisms and surgical outcomes. Methods : Between 2009 and 2014, we performed redo mitral valve surgery in 11 patients who had refractory hemolytic anemia after mitral valve surgery at Kyoto University Hospital. The mean age of the patients was 72.2±6.8 years old, and there were 5 men. Results : Preoperative echocardiography demonstrated that only 3 patients had ≥ grade 3 mitral regurgitation (MR), the rest of the patients had only mild to moderate MR. The mechanisms of severe hemolysis included paravalvular leakage (PVL) after mitral valve replacement (MVR) in 8 patients, structural valve deterioration (SVD) after MVR using a bioprosthesis in one, and residual/recurrent mitral regurgitation after mitral valve plasty (MVP) in two. All the patients except one (re-MVP) underwent MVR. The mean interval between previous operation and current operation was 14.1±9.4 years in post-MVR cases, and 2.0±1.9 years in post-MVP cases. There were three late deaths, one of which was due to cardiac death (exacerbation of heart failure due to pneumonia). There was one patient who required re-MVR for recurrent hemolysis due to PVL after MVR. Conclusion : Although hemolytic anemia after mitral valve surgery is rare, it often requires reoperation regardless of the degree of MR at late follow-up period. Thus, patients after mitral valve surgery should be carefully followed-up.

Background : There has been no ideal valve prosthesis for children from the point of view of thromboembolism and size mismatch, and the surgical repair of native mitral valve has always been our first priority in children. However, valve replacement becomes the inevitable surgical option if repair is impossible. The purpose of this study was to assess the long-term results of mitral valvuloplasty (MVP) and mitral valve replacement (MVR) for isolated mitral valve diseases in children under 15 years old. Patients and Methods : From 1981 to 2010, 30 patients underwent a total of MVPs (P group) and 26 consecutive patients underwent a total of MVRs (R group). The median age was 4.6 years (4 months to 16 years) in group P and 6.2 years (4 months to 13.7 years) in group R, and the median body weight was 13.4 kg (6 to 35.5 kg) in group P and 16.4 kg (4.8 to 50.7 kg) in group R. The etiology was congenital in 55 (98%) patients, and due to endocarditis in 1 (2%) patient. Isolated mitral regurgitation was present in 41 (73%) patients (group iMR), and isolated mitral stenosis was present in 15 (27%) patients (group iMS). Mechanical valves (bileaflet disc, n=26) were used in all initial MVR patients. Results : Overall hospital mortality was 0%. The median follow-up time was 9.3±7.8 years (4 months to 27.7 years). There were 6 reoperations in P group and 5 explants due to size mismatch in R group with patient growth, and no explant due to structural valve deterioration. Survival rates at 10 years were 100% in the P group and 88.0% in the R group, and there was a significant difference (p=0.043). Freedom from reoperation at 10 years was 77.6% in P group and 77.0% in R group. Freedom rate from cerebral events at 10 years were 100% in both groups, respectively, and there were no significant differences. Survival rates at 10 years were 100% in the iMR group and 53.3% in the iMS group, respectively, and there was a significant difference between the groups (p<0.001). Freedom rates from reoperation at 10 years were 77.1% in the iMR group and 64.3% in the iMS group, respectively, there being no significant difference. Conclusions : The long-term results of pediatric mitral surgery were acceptable. Mitral valvuloplasty for patients with isolated mitral regurgitation were excellent. Mitral valve replacement can be performed with low initial mortality but should be reserved for medical and reconstruction failure because reoperation and late mortality are high, particularly for patients with isolated mitral stenosis.

In Japanese traditional medicine, “ganmenshin” (facial diagnosis), which means assessment of a patient's health based on the color of his/her face, is frequently used in a clinical setting. However, little scientific evidence is available to substantiate such diagnoses. In the present study, we investigated the relationship between skin color and patient's health status to ascertain the clinical usefulness of the “ganmenshin” technique. We evaluated the color of the cheeks and forearms of 30 healthy study participants and also administered questionnaires to determine their health. A correlation was observed between skin color and work efficiency and the inkyo (yin-fluid deficiency) scores in all subjects. In male subjects, skin color was correlated with their bodymass index (BMI), self-rated health status, work efficiency, and suitai (fluid stagnation) scores. In female subjects, skin color was correlated with the patient's age, work efficiency, and inkyo scores. These results suggest that “ganmenshin” may be clinically useful because of the correlation between skin color and the health of patients.

Introduction : We report the effect of adaptive servo ventilation (ASV) treatment on heart failure in a home care setting.
Methods : For eight home care patients aged ≥75 with heart failure, we diagnosed sleep-disordered breathing (SDB) by using a simplified monitor and implemented ASV treatment. We assessed its effect on heart failure by using NT-proBNP. We also assessed its effects on SDB by using apnea hypopnea index (AHI).
Results : Three patients dropped out because they felt fear of putting on the mask. Among the other five patients, we observed a significant decrease rate of NT-proBNP (mean : -0.43, 95% confidence interval (CI) : -0.64, -0.22) after the ASV treatment. With regard to AHI, we observed a decreasing tendency (mean : -20.4 /hour, 95% CI : -41.2/hour, 0.4/hour).
Conclusion : This study suggests that ASV treatment is effective for a treatment of heart failure in a home care setting.

Background : The improvement in surgical results for congenital heart disease has resulted in an increase in the number of adult congenital heart disease (ACHD) cases. Some ACHD patients are known to develop thoracic aortic aneurysm (TAA) at a young age, so we examined TAA in ACHD patients presenting at our institute over a 10-year interval. Methods : From 2002 to 2011, we performed 32 cases of surgery for TAA in ACHD patients. We excluded 5 cases of adult bicuspid aortic valve, 2 of TAA with untreated congenital heart disease (CHD), 1 of Marfan syndrome with CHD, and 9 of coarctation of the aorta (CoA) repair for the same site ; 15 patients were included. Results : The male/female ratio was 13/2, and the age of reoperation was 33.3±10.8 years. The 15 ACHD patients included 5 cases of the tetralogy of Fallot (TOF), 4 of congenital aortic stenosis (AS), 3 of ventricular septal defect (VSD), and 1 of each CoA complex, polysplenia/double outlet right ventricle (DORV), and polysplenia/corrected transposition of the great arteries (cTGA). Twelve cases of root dilatation and 2 of ascending aortic aneurysm were observed and 10 cases were concomitant with moderate to severe aortic regurgitation. Thirteen cases underwent elective surgery and the other two cases were emergency surgeries : a Bentall procedure for type 2 acute aortic dissection of polysplenia/DORV, and a Bentall and right ventricular outflow reconstruction (RVOTR) for ascending aorta/right ventricle rupture due to Konno patch detachment in congenital AS. The 13 elective cases included 11 cases of Bentall procedure, 1 of ascending aorta/hemi arch replacement, and 1 of ascending aorta replacement. Concomitant procedures were 1 case of aortic valve replacement, 1 of mitral valve replacement, 1 of subaortic stenosis release, and 2 of RVOTR. Operation time was 572.8+/-101.4 min, cardiopulmonary bypass time was 295.8+/-100.2 min, and aorta clamp time was 188.1+/-58.8 min. One hospital death was observed in 1 emergency case due to methicillin-resistant Staphylococcus aureus (MRSA) sepsis, but no 30-day mortality was observed. Intensive care unit (ICU) stay was 9.4+/-10.1 days and hospital stay was 34.4+/-18.2 days. Conclusion : The most common ACHD found during TAA surgery in our institute was Tetralogy of Fallot. ACHD had various complications and restrictions for surgery but TAA surgery in ACHD patients was safe and feasible.

Risk analysis models are becoming more important in various aspects of the clinical setting. We have used the logistic EuroSCORE as a risk analysis model, but there is divergence between the model and actual clinical reality in our country. The Japan Score is a risk model based on the Japan Adult Cardiovascular Surgery Database and it is considered to be better reflect from Japanese clinical results. We compared the logistic EuroScore (ES) and Japan Score (JS) and their predictive accuracy, using our clinical results. Between October 2006 and June 2011, 733 operations suitable for evaluation by the Japan Score were performed at our institute. Isolated coronary artery bypass grafting (CABG) was performed in 151 cases, valve surgery (Valve) in 346 cases and aortic surgery (Aorta) in 236 cases. In these cases we calculated 30-day mortality using the EuroSCORE and JapanSCORE and compared the results and prediction accuracy, by calculating the receiver operating characteristic curve (ROC curve) and the area under the ROC curve (AUC). We also calculated 30-day mortality and morbidity by the JapanSCORE and analyzed it by the same method. In the entire group, logistic 30-day mortality by ES and JS was 7.28 and 4.05% respectively. The AUC was 0.740 and 0.806, while 30-day mortality and morbidity calculated by JS was 17.72% and the AUC was 0.646. In the CABG group the 30-day mortality by ES and JS was 5.7 and 3.18% respectively, the AUC was 0.636 and 0.770, the 30-day mortality and morbidity was 13.37% and the AUC was 0.631. In the Valve group 30-day mortality by ES and JS was 6.00 and 3.79% respectively. The AUC was 0.715 and 0.794, 30-day mortality and morbidity was 17.54% and the AUC was 0.606. In the Aorta group 30-day mortality was 10.17 and 4.99% respectively. The AUC was 0.720 and 0.827. The 30-day mortality and morbidity was 20.83% and the AUC was 0.640. The 30-day mortality calculated by JS was significantly lower than that of ES (p<0.001). The prediction accuracy of both of the ES and the JS was satisfactory but the prediction accuracy of JS was better than that of the ES. The prediction accuracy of the logistic 30-day mortality and morbidity were not as accurate as 30-day mortality. JS was a good risk analysis model not only for prediction of surgical results but also for improving surgical outcome.