The effect of Aspergillus luchuensis pectin methylesterase genes pmeA and pmeB on methanol production in sweet potato shochu.

To reduce the methanol content in sweet potato shochu, we studied the pectin methylesterase genes of the shochu-koji mold Aspergillus luchuensis. We found the following three homologs of pectin methyleseterase in the genome of A. luchuensis: pmeA, pmeB, and pmeC. Using pectin as a substrate, the methanol-producing activity of the recombinant of each gene expressed in A. luchuensis was examined and found to be present in recombinant PmeA and PmeB. Additionally, small-scale fermentation of sweet potato shochu using disruptions of pmeA and pmeA-pmeB in A. luchuensis (∆pmeA and ∆pmeApmeB) resulted in significant reduction of the methanol content. Taken together, we revealed that the A. luchuensis pmeA gene was mainly involved in methanol production in sweet potato shochu.

Clinical trial monitoring effectiveness: Remote risk-based monitoring versus on-site monitoring with 100% source data verification.

BACKGROUND/AIMS: Traditional on-site monitoring of clinical trials via frequent site visits and 100% source data verification is cost-consuming, and it still cannot guarantee data quality effectively. Depending on the types and designs of clinical trials, an alternative would be combining several monitoring methods, such as risk-based monitoring and remote monitoring. However, there is insufficient evidence of its effectiveness. This research compared the effectiveness of risk-based monitoring with a remote monitoring system with that of traditional on-site monitoring. METHODS: With a cloud-based remote monitoring system called beagle View®, we created a remote risk-based monitoring methodology that focused only on critical data and processes. We selected a randomized controlled trial conducted at Tohoku University Hospital and randomly sampled 11 subjects whose case report forms had already been reviewed by data managers. Critical data and processes were verified retrospectively by remote risk-based monitoring; later, all data and processes were confirmed by on-site monitoring. We compared the ability of remote risk-based monitoring to detect critical data and process errors with that of on-site monitoring with 100% source data verification, including an examination of clinical trial staff workload and potential cost savings. RESULTS: Of the total data points (n = 5617), 19.7% (n = 1105, 95% confidence interval = 18.7-20.7) were identified as critical. The error rates of critical data detected by on-site monitoring, remote risk-based monitoring, and data review by data managers were 7.6% (n = 84, 95% CI = 6.2-9.3), 7.6% (n = 84, 95% confidence interval = 6.2-9.3), and 3.9% (n = 43, 95% confidence interval = 2.9-5.2), respectively. The total number of critical process errors detected by on-site monitoring was 14. Of these 14, 92.9% (n = 13, 95% confidence interval = 68.5-98.7) and 42.9% (n = 6, 95% confidence interval = 21.4-67.4) of critical process errors were detected by remote risk-based monitoring and data review by data managers, respectively. The mean time clinical trial staff spent dealing with remote risk-based monitoring was 9.9 ± 5.3 (mean ± SD) min per visit per subject. Our calculations show that remote risk-based monitoring saved between 9 and 41 on-site monitoring visits, corresponding to a cost of between US$13,500 and US$61,500 per trial site. CONCLUSION: Remote risk-based monitoring was able to detect critical data and process errors as well as on-site monitoring with 100% source data verification, saving travel time and monitoring costs. Remote risk-based monitoring offers an effective alternative to traditional on-site monitoring of clinical trials.

Detection of Pediatric Pulmonary Arterial Hypertension by School Electrocardiography Mass Screening.

Rationale: To detect pulmonary arterial hypertension (PAH) at any early stage is a promising approach to optimize the outcome. Objectives: To investigate the impact of school ECG-based screening on detecting idiopathic or heritable (I/H)-PAH in the general pediatric population. Methods: This was a nationwide survey of patients with I/H-PAH newly diagnosed at 3 months to 18 years of age in Japan during 2005-2012. Measurements and Main Results: Eighty-seven eligible patients (age range, 1-16 yr) were recruited. Among 68 (78%) patients diagnosed at greater than or equal to 6 years of age (the age of the first ECG-based screening), 28 (41%) were detected by the ECG-based screening (screening group) and 40 (59%) were recognized by their symptoms (n = 37) or coincidental occasions (n = 3; nonscreening group). In the screening group, the proportion of patients in World Health Organization functional class I/II at diagnosis was higher (96% vs. 60%; P < 0.001), plasma brain natriuretic peptide level was lower (149 ± 290 vs. 398 ± 559 pg/ml; P = 0.045), and 6-minute-walk distance was longer (420 ± 109 vs. 327 ± 104 m; P < 0.001) than the nonscreening group, despite similar values in mean pulmonary artery pressure (58 ± 17 vs. 61 ± 17 mm Hg; P = 0.42) and pulmonary vascular resistance index (18 ± 8 vs. 21 ± 11 Wood units ⋅ m2; P = 0.24) between groups. The proportion of patients on intravenous epoprostenol at the final visit was lower in the screening group than the nonscreening group (14% vs. 50; P = 0.004). Conclusions: These findings suggest that the ECG-based screening detects a unique subpopulation of pediatric patients with I/H-PAH that is associated with already established pulmonary hypertension but without obvious right heart failure and warrants investigating the prognostic significance of this system.

Extremely short setting of optimal sensed atrioventricular interval in patients after Fontan procedure with implanted dual-chamber pacemaker.

BACKGROUND: Atrioventricular interval optimisation is important in patients with dual-chamber pacing, especially with heart failure. In patients with CHD, especially in those with Fontan circulation, the systemic atrial contraction is supposed to be more important than in patients without structural heart disease. METHODS: We retrospectively evaluated two patients after Fontan procedure with dual-chamber pacemaker with a unique setting of optimal sensed atrioventricular interval. RESULTS: The optimal sensed atrioventricular interval determined by echocardiogram was extremely short sensed atrioventricular interval at 25 and 30 ms in both cases; however, the actual P wave and ventricular pacing interval showed 180 and 140 ms, respectively. In both cases, the atrial epicardial leads were implanted on the opposite site of the origin of their own atrial rhythm. The time differences between sensed atrioventricular interval and actual P wave and ventricular pacing interval occurred because of the site of the epicardial atrial pacing leads and the intra-atrial conduction delay. CONCLUSION: We need to consider the origin of the atrial rhythm, the site of the epicardial atrial lead, and the atrial conduction delay by using electrocardiogram and X-ray when we set the optimal sensed atrioventricular interval in complicated CHD.

Cellular fraction analysis of pericardial effusion helps the diagnosis of eosinophilic myocarditis.

Eosinophilic myocarditis is rare in children, and consequently, it is difficult to diagnose eosinophilic myocarditis rapidly. We report the clinical course of acute eosinophilic myocarditis with pericarditis in two adolescent boys and their associated electrocardiograms. The two patients, 13- and 14-year-old boys, developed cardiomegaly and chest pain with vomiting. On examination by two-dimensional echocardiography, thickening of the ventricular septum and a pericardial effusion were detected. The eosinophil count had increased by the pericardial effusion. Acute eosinophilic myocarditis often complicates a moderate to severe pericardial effusion owing to acute pericarditis. A cellular fraction analysis of the pericardial effusion is easy and useful for the diagnosis of eosinophilic myocarditis. Some serial changes in the electrocardiogram occur during each stage of acute eosinophilic myocarditis. They are induced by eosinophilic granules, which are capable of inducing tissue damage and dysfunction, and those changes in the electrocardiogram resemble the changes after an acute myocardial infarction. It is important to know the characteristics of eosinophilic myocarditis in order to prevent lethal complications.

Comparison of Clinical Profiles in Patients with Protein-Losing Enteropathy With and Without Fontan Circulation.

Protein-losing enteropathy (PLE) is a life-threatening complication in patients following the Fontan operation. However, PLE also develops in some patients with congenital heart disease (CHD) after biventricular repair (BVR). This study compared clinical profiles of PLE patients following the Fontan operation with those after BVR. We retrospectively reviewed clinical charts of postoperative CHD patients with PLE. The study population comprised 42 PLE patients (14BVR, 28Fontan). Postoperative follow-up period until onset was significantly shorter in the Fontan group than in the BVR group (14 ± 2 vs. 8 ± 1 years, p = 0.02), while there was no difference in PLE onset age between groups. Furthermore, there were no differences in prevalence of clinically relevant arrhythmias, cardiac output, or central venous pressure between the two groups at PLE onset. Percentage of structural lesions (valve regurgitation and/or stenotic lesions) responsible for development of PLE and ventricular end-diastolic pressure were higher in the BVR group than in the Fontan group (93 vs. 50%, p < 0.01), (13.4 ± 6.3 vs. 7.5 ± 4.1, p < 0.0001). Catheter intervention was applied in 2Fontan and 6BVR patients, while surgical intervention was required in 8BVR and 7Fontan patients. Of these, catheter intervention was effective in 2 (25%, 1Fontan, 1BVR) and surgical intervention was effective in 4 (26.7%, 1Fontan, 3BVR). Only one patient (5.3%) improved without intervention. Complete PLE remission rate was higher in the BVR group than in the Fontan group (38 vs. 7%, p = 0.02). During follow-up, death of 2 BVR and 8 Fontan patients occurred. There were no group differences in 5- to 10-year survival rates after PLE onset (81 vs. 81%, BVR, 81 vs. 66%, Fontan). Although BVR patients may have greater chance of PLE remission when compared with those exhibiting Fontan pathophysiology, mortality in PLE-CHD patients was significantly high regardless of postoperative hemodynamics.

Hemodynamic determinants of mortality after Fontan operation.

BACKGROUND: Elevated central venous pressure (CVP), low cardiac output, and mild hypoxia are common early and late after Fontan operations. However, the association of these characteristics with late mortality is unclear. We aimed to elucidate the hemodynamic determinants of mortality after Fontan operation. METHOD: We evaluated early (group early; 0.5-5years postoperatively, n=387) and late (group late; ≥15years postoperatively, n=161) Fontan hemodynamics that included CVP (mm Hg), cardiac index (CI; L/min per m2), systemic ventricular end-diastolic volume index (mL/m2), ejection fraction (EF; %), and arterial blood oxygen saturation (%). We examined the effect of these variables on 5-year all-cause mortality. RESULTS: Mortality was higher in group late than in group early (17 vs 11, P<.0001). In both groups, higher CVP (hazard ratio [HR]1.46 and 1.38, respectively; P<.001-.0001) and lower arterial blood oxygen saturation (HR 1.12, P<.001 for both) were associated with increased mortality. Greater end-diastolic volume index (HR per 20: 1.73) and lower EF (HR per 10%: 3.38) were associated with increased mortality only in group early (P<.0001 for both). In contrast, only in group late was higher CI associated with increased mortality (HR 2.50, 95% CI 1.30-4.55, P<.01). Seven patients in group late with both high CVP (≥14) and CI (≥3.0) had the highest mortality (HR 18.1, 5.55-52.4, P<.0001). CONCLUSIONS: Elevated CVP and low arterial blood oxygen saturation correlate with mortality in both early and late Fontan survivors. End-diastolic volume index and EF are associated with mortality only in the earlier cohort, whereas interestingly, elevated cardiac output is associated with increased mortality in the later cohort.

The in vivo Inhibition of Oral Biofilm Accumulation and Streptococcus mutans by Ceramic Water.

Combustion-synthesized titanium carbide ceramics uniformly disperse silver, producing silver ions and hydroxyl radicals in water. This generates antimicrobial activity against various bacteria. One such bacterium is Streptococcus mutans, a gram-positive anaerobic bacterium known as a major pathogen of dental caries. In this study, we analyzed the inhibition of oral biofilms and S. mutans by ceramic water in in vitro and human studies. S. mutans strains showed significantly lower antimicrobial and sucrose-dependent adhesion activity in the presence of ceramic powder compared with untreated culture medium. Confocal microscopy revealed that S. mutans biofilm structures with ceramic powder were thin and coarse. Twenty-seven volunteers (13 males, 14 females; 18-37 years old, mean 25.2 years) were enrolled for subsequent studies. After each meal, one group was asked to rinse with ceramic water while the other rinsed with untreated water for 1 week. After 1 week, the rinsing contents were switched between the groups and the same protocol was followed for an additional week. After rinsing with ceramic water, the average plaque score was 43.0 ± 3.7, which was significantly lower than the baseline value (74.1 ± 5.7, p < 0.001). However, no significant difference was observed when rinsing with untreated water. In addition, the total number of S. mutans in saliva was significantly reduced after rinsing with ceramic water compared with untreated water (p < 0.05). These results suggest that ceramic water possesses antimicrobial activity against S. mutans and inhibits biofilm formation. Rinsing with ceramic water can also inhibit dental plaque formation and S. mutans colonization in humans.

Determinants of Aortic Size and Stiffness and the Impact on Exercise Physiology in Patients After the Fontan Operation.

The pathophysiology of congenital heart disease includes aortic dilation and increased stiffness. However, the clinical determinants and significance remain unclear in patients after the Fontan operation.Size and stiffness index (SI) of the ascending and descending aorta (aAO and dAO, respectively) were assessed using angiography in 130 consecutive Fontan patients and 30 age-matched controls. Compared with controls, Fontan patients showed a dilated aAO and smaller dAO (P < 0.0001) with greater SI (3.2 ± 0.7 versus 2.2 ± 0.3 for aAO and 2.7 ± 0.6 versus 2.2 ± 0.3 for dAO, P < 0.0001 for both). aAO was stiffer than dAO (P < 0.0001) and the greater aAO size was independently determined by the presence of pulmonary atresia, older age at Fontan operation, and low arterial oxygen saturation (P < 0.05-0.01). High plasma levels of brain natriuretic peptide (BNP) and glucose were independently associated with aAO SI (P < 0.05-0.01) and the SI ratio of aAO to dAO SI, whereas body mass index, plasma levels of highsensitivity C-reactive protein, and dAO size were independently associated with dAO SI (P < 0.05-0.01). A greater aAO and aAO/dAO ratio predicted an impaired exercise blood pressure response (P < 0.0001). Furthermore, in addition to age at Fontan operation and BNP level, the aAO SI independently predicted a lower peak oxygen uptake (P < 0.05).Fontan patients have a stiffer dilated aAO with rapidly tapering smaller dAO that predicts exercise pathophysiology. In addition to intrinsic aortic structural abnormalities, heart failure severities as well as traditional cardiovascular risk factors are also involved in the aortic structural and functional abnormalities.

[Problems in Emergency Shelters and Laboratory Testing].

During disasters, medical services are occasionally provided by temporary medical offices organized within emergency shelters. The types of disease dealt with by these offices change with time after the event, and their conditions also change depending on the time needed for normal activities to resume. As usable devic- es and test methods are limited due to various causes, such as interrupted life lines, it is necessary for medi- cal service providers to flexibly manage the situation, and for supporters to provide them with resourceful support. [Review].

Diverse multi-organ histopathologic changes in a failed Fontan patient.

We report multi-organ histopathological changes in a patient with protein-losing enteropathy (PLE) over 12 years after Fontan operation. A 14-year-old boy with right isomerism heart and single ventricle had undergone Fontan procedure at 19 months of age, and PLE was diagnosed at 28 months. He had several episodes of intestinal bleeding and pre-renal failure with elevated creatine, and eventually died of pneumonia. The intrapulmonary small arteries showed medial and intimal thickening resembling pulmonary hypertension. No major ulcerative lesions were found in the small or large intestines. Dilated lymph ducts, one of the characteristic features of PLE, were not seen in mucosal and submucosal areas. Liver cirrhosis was obvious despite little increase in liver enzymes. Histological changes in bilateral kidneys were subtle despite repeated episodes of renal failure. Thus, there may be significant discrepancies between clinical manifestations and multi-organ histological changes in failed Fontan patients.

Usefulness of Percutaneous Transluminal Coronary Balloon Angioplasty for the Left Coronary Artery Stenosis 10 Years More Than After Arterial Switch Operation.

Proximal stenosis adjacent to the orifice of one or both coronary arteries may occur after the arterial switch operation (ASO) for d-transposition of the great arteries (d-TGA). Coronary artery stenosis (CAS) often progresses within the first 6 months postoperatively and may result in myocardial ischemia and infarction. Although percutaneous transluminal coronary balloon angioplasty (PCBA) for CAS within 15 months after ASO for d-TGA has been reported, there is no report of PCBA for CAS in the late period after ASO. We present the results of PCBA for CAS of the left coronary artery performed more than 10 years after ASO in an 11-year-old boy and a 14-year-old boy without complication. The stenosis degree improved in both patients from 81 to 45 and 80 to 54 %, respectively. Restenosis did not occur, and the stenosis degree improved to about 25 % late after PCBA. Although the initial effect of PCBA may not be dramatic, it can improve late after PCBA. It was considered that the optimal balloon-reference vessel ratio was about 1.0, to obtain the minimal effective lumen diameter. PCBA for CAS even if performed many years after ASO is feasible without complication. PCBA can also provide delayed improvement late after the procedure.

Chronological changes in stenosis of translocated coronary arteries on angiography after the arterial switch operation in children with transposition of the great arteries: comparison of myocardial scintigraphy and angiographic findings.

BACKGROUND: The peri-operative mortality of the arterial switch operation in neonates with transposition of the great arteries is considerably low; however, long-term outcomes of translocated coronary arteries still remain one of the most crucial issues. Methods and results A total of 110 neonates with transposition of the great arteries after arterial switch operation were evaluated; three (2.7%) late deaths occurred. The remaining 107 patients except for one underwent follow-up angiography. Angiography showed coronary artery stenosis in nine (8.4%), with right coronary artery lesions in two and left main trunk lesions in seven. In two patients, right coronary artery stenosis regressed during follow-up. In left main trunk lesions, the severity of stenosis improved in four, did not change in one, and progressed to total occlusion in two patients. In children with coronary artery stenosis, myocardial scintigraphy showed perfusion defects in five out of six (83%) with left main trunk with ⩾75% stenosis and in four out of four with left main trunk stenosis ⩾90%. In contrast, patients whose coronary artery stenosis disappeared during follow-up had no perfusion defects on scintigraphy. CONCLUSIONS: Regression of ostial stenosis of the transplanted coronary artery on angiogram was observed. The stenosis regressed over time in six patients; two coronary arteries with 99% stenosis and delayed angiographic enhancement of the distal coronary artery resulted in total occlusion within 1 year after the arterial switch operation. Combination of angiography and myocardial scintigraphy could be useful to differentiate deceptive stenosis from progressive stenosis.

Transcriptomic analysis of temperature responses of Aspergillus kawachii during barley koji production.

The koji mold Aspergillus kawachii is used for making the Japanese distilled spirit shochu. During shochu production, A. kawachii is grown in solid-state culture (koji) on steamed grains, such as rice or barley, to convert the grain starch to glucose and produce citric acid. During this process, the cultivation temperature of A. kawachii is gradually increased to 40 °C and is then lowered to 30 °C. This temperature modulation is important for stimulating amylase activity and the accumulation of citric acid. However, the effects of temperature on A. kawachii at the gene expression level have not been elucidated. In this study, we investigated the effect of solid-state cultivation temperature on gene expression for A. kawachii grown on barley. The results of DNA microarray and gene ontology analyses showed that the expression of genes involved in the glycerol, trehalose, and pentose phosphate metabolic pathways, which function downstream of glycolysis, was downregulated by shifting the cultivation temperature from 40 to 30 °C. In addition, significantly reduced expression of genes related to heat shock responses and increased expression of genes related with amino acid transport were also observed. These results suggest that solid-state cultivation at 40 °C is stressful for A. kawachii and that heat adaptation leads to reduced citric acid accumulation through activation of pathways branching from glycolysis. The gene expression profile of A. kawachii elucidated in this study is expected to contribute to the understanding of gene regulation during koji production and optimization of the industrially desirable characteristics of A. kawachii.

Characteristics and Fate of Systemic Artery Aneurysm after Kawasaki Disease.

OBJECTIVE: To determine the long-term outcome of systemic artery aneurysms (SAAs) after Kawasaki disease (KD). STUDY DESIGN: We investigated the characteristics and the fate of SAAs in 20 patients using medical records and angiograms. The age of onset of KD ranged from 1 month to 20 months. The interval from the onset of KD to the latest angiogram ranged from 16 months to 24 years. The regression rate of peripheral artery aneurysm and the frequency of stenotic lesions were analyzed by the Kaplan-Meier method in 11 patients who had undergone initial angiography within 4 months. RESULTS: The mean duration of fever was 24 ± 12 days. All 20 patients had at least 1 symmetric pair of aneurysms in bilateral peripheral arteries, and 16 patients had multiple SAAs. The distributions of SAAs was as follows: brachial artery, 30; common iliac artery, 20; internal iliac artery, 21; abdominal aortic aneurysm, 7; and others, 29. The frequencies of regression of SAA and of the occurrence of stenotic lesions at 20 years after the onset of KD were 51% and 25%, respectively (n = 42). The diameter of all SAAs in the acute phase leading to stenotic lesions in the late period was >10 mm. CONCLUSION: SAAs occurred symmetrically and were multiple in younger infants and those with severe acute vasculitis. The fate of SAAs resembles that of coronary artery aneurysms, and depends on the diameter during the acute phase. Larger SAAs can lead to stenotic lesions in the late period.

Cardiac resynchronization therapy for various systemic ventricular morphologies in patients with congenital heart disease.

BACKGROUND: Cardiac resynchronization therapy (CRT) can result in functional improvement and reduced mortality in patients with medically refractory heart failure. Although CRT is reportedly effective in patients with congenital heart disease (CHD), it is still controversial in patients who have systemic right ventricle. METHODS AND RESULTS: Twenty CHD patients treated with CRT since 2006 were divided into 3 groups based on systemic ventricular (sysV) morphology (7 with left ventricle [sLV], 7 with right ventricle [sRV], and 6 with unbalanced 2 ventricles as a single-ventricular physiology [sBV]). The acute effects of CRT on hemodynamics and sysV function before device implantation was retrospectively evaluated and the chronic (≥6 months) effects of CRT on late outcomes was assessed. In our CHD populations, sysV volume index was reduced from 139±41 to 118±33 ml/m(2)(P=0.04) after CRT, and there was significant improvement in B-type natriuretic peptide levels (from 341±384 to 160±152 pg/ml, P=0.01) and New York Heart Association (NYHA) functional class (from 2.1±0.6 to 1.8±0.7, P=0.02) on a late outcome. The sRV group did not show a late sysV volume reduction despite significant QRS shortening, and an increase of sysV peak dP/dt in the acute study differed from that of other groups. CONCLUSIONS: CRT improves late hemodynamic and functional status in sLV and sBV CHD patients with a dyssynchronized sysV. However, an acute CRT effect cannot guarantee long-term benefit in sRV patients.

The clinical characteristics of sudden cardiac arrest in asymptomatic patients with congenital heart disease.

Sudden cardiac arrest (SCA) is a major cause of death in patients with congenital heart disease (CHD). Systemic ventricular dysfunction is a reported risk factor for SCA. We retrospectively analyzed the medical records of 46 patients (age >6 years) who experienced SCA. The following underlying cardiac defects were observed: biventricular repair with affected subpulmonary right ventricle (n = 18, 39 %), biventricular repair with systemic right ventricle and Eisenmenger syndrome (n = 6 each, 13 %), Fontan circulation and unrepaired CHD (n = 5 each, 11 %), and others (n = 6, 13 %). Twenty-one patients (46 %) had no history of arrhythmias, and 21 of 43 (49 %) showed systemic ventricular ejection fraction >55 %. According to the New York Heart Association classification, 18 patients (39 %) were class I and 28 (61 %) were class II/III. SCA occurred at a younger age in class I (16 ± 5 years) than in the other classes (23 ± 10 years; P = 0.004). QRS duration was similar between the groups (136 ± 38 vs. 141 ± 50 ms; P not significant). Seven patients in class I (15 % of all SCAs) had no history of arrhythmias or features of hemodynamic abnormalities. The proportion of patients with biventricular repair and affected subpulmonary right ventricle was higher than that of patients with other defects, and the majority of SCA patients had more complicated defects than a simple repaired ventricular septal defect or an atrial septal defect. No symptoms of heart failure, history of arrhythmias, or features of hemodynamic abnormalities were observed in 15 % of the patients who experienced SCA. Prolonged QRS duration might be a predictor of SCA even in asymptomatic CHD patients. Prevention of SCA in CHD patients may require more detailed evaluation than is typically considered necessary.

Heterogeneity of Ventricular Sympathetic Nervous Activity is Associated with Clinically Relevant Ventricular Arrhythmia in Postoperative Patients with Tetralogy of Fallot.

This study aimed to clarify whether there is an association between ventricular sympathetic nervous activity (SNA) and clinically relevant ventricular arrhythmia (a run of ≥ 3 consecutive ventricular beats, RVA) in postoperative patients with tetralogy of Fallot (TOF). We performed a retrospective study in a national referral center of pediatric cardiology in Japan. Twenty-four postoperative TOF patients (13 males, median age 17 years) undergoing myocardial (123)I metaiodobenzylguanidine (MIBG) scintigraphy were included. We measured the heart-to-mediastinum ratio (HMR) and washout ratio (WR) from planar MIBG myocardial scintigraphy. Tomographic images and polar maps were generated with 20 segments. The standard deviation of percentage uptake of 20 segments (SD-uptake) as an index of heterogeneous MIBG uptake to the ventricular myocardium was calculated. We compared these MIBG-derived variables with the patients' clinical profiles, including ECG findings and hemodynamics. Eight of 24 patients had RVA (RVA group), and the other 16 did not have RVA (non-RVA group). There were no significant differences in the HMR (1.9 ± 0.5 vs. 2.2 ± 0.4) and WR (50 ± 5 vs. 42 ± 10) between the two groups. SD-uptake was significantly higher in the RVA group than in the non-RVA group (15 ± 3 vs. 12 ± 3, p = 0.03). QT dispersion (ms) was also higher in the RVA group than in the non-RVA group (53 ± 23 vs. 44 ± 18, p = 0.04). Multivariate logistic regression showed that SD-uptake and QT dispersion were independent predictors in the RVA group (p = 0.02, p = 0.03). In addition to greater QT dispersion, heterogeneous SNA is associated with RVA in TOF patients postoperatively.

[Management of POCT Devices and Reagents].

In order to ensure the accuracy of POCT devices and reagents, it is necessary to appropriately manage and store them. There are various points to be considered for these items, such as management before and environments when using them; it is more complex than when using conventional analysis apparatuses in clinical laboratories. In addition, staff using such devices should be provided with opportunities to obtain sufficient knowledge and skills. These approaches are indispensable to ensure POCT accuracy and provide reliable data, and, in this respect, support for staff with expertise in clinical examination is crucial.

[One-stage resection of intravenous leiomyomatosis extending into the right atrium].

Intravenous leiomyomatosis (IVL) is a benign tumor that originates from a uterine myoma and rarely extends to the heart through the inferior vena cava (IVC). Echocardiography revealed an abnormal mass in the right atrium in a 63-year-old asymptomatic woman. Preoperative examination revealed a tumor extending from a myoma through the right internal iliac vein to the right atrium, and the patient was diagnosed with IVL. She underwent sternotomy combined with laparotomy, and the intravenous and intracardiac tumor was removed under normothermic cardiopulmonary bypass without cardiac arrest. Hysterectomy and bilateral adnexectomy were also performed. No additional therapy was required after surgery.