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Ventricular-assist device therapy for small patients with congenital heart disease is challenging due to its complex anatomy and hemodynamics. We describe a 3-year-old patient with heart failure with truncus arteriosus in the palliative stage. The patient underwent palliative right ventricular outflow tract reconstruction following bilateral pulmonary artery banding. At 6 months of age, the patient developed severe truncal valve regurgitation and left ventricular dysfunction. Emergent truncal valve replacement with a mechanical valve was performed, but left ventricular dysfunction persisted. At 3 years of age, the patient developed acute progression of heart failure triggered by influenza infection. The patient was intubated and transferred to our center to determine the indication for heart transplantation. On the second day after admission, signs of multiorgan failure appeared. Emergent ventricular-assist device implantation for both ventricles was performed with truncal valve closure, ventricular septal defect closure, atrial septal defect closure, and re-right ventricular outflow tract reconstruction. The right ventricular-assist device was successfully removed on the seventh postoperative day. Due to the small pulmonary arteries, severe pulmonary stenosis persisted after ventricular-assist device implantation, but it gradually improved with multiple pulmonary angioplasties. The patient was registered in the Japanese organ transplant network and is awaiting a donor organ in a stable condition., (© 2024. The Author(s).)

The effectiveness of ultrasound-guided peripheral arterial cannulation (UGPAC) in children has been increasingly been reported. However, to the best of our knowledge, there have been no reports of UGPAC in neonates, including very low birth weight infants (VLBWIs). In this study, we aimed to retrospectively review the results of UGPAC in neonates, including VLBWIs, and assess its effectiveness. This case series was conducted in a tertiary neonatal intensive care unit (NICU) in Japan. We included neonates aged below 28 days who underwent UGPAC in our NICU between April 2021 and October 2022. We extracted the following data from medical records and analysed it retrospectively: patient age (days), postconceptional age, patient weight at the time of cannulation, number of punctures using the conventional technique before ultrasound guidance was performed and number of punctures with the ultrasound-guided technique until successful cannulation. A total of 27 UGPACs were performed in 19 neonates, including 14 cannulations in 10 VLBWIs. In infants weighing > 1500 g and VLBWIs, the success rate within the first three punctures was 100% (13/13) and 79% (11/14), respectively. Overall, 41% (11/27) of UGPACs were performed following failed punctures using conventional methods, with a 100% success rate within the first three attempts. In all cases, no apparent adverse events, such as hypothermia, were noted.  Conclusions: Our results suggest that UGPAC had a high success rate in neonates, including VLBWIs. Further studies are required to compare the effectiveness of UGPAC with conventional methods in neonates. What is Known: • The use of ultrasound guidance for arterial cannulation is recommended in children. • Ultrasound-guided peripheral arterial cannulation (UGPAC) in neonates, including very low birth weight infants (VLBWIs), has not been reported. What is New: • UGPAC in neonates, including VLBWIs, was performed with a high success rate; approximately 40% of UGPACs were performed after the failure of the conventional methods. • This study suggested the effectiveness of UGPAC in neonates, including VLBWIs., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

This single-centre prospective feasibility study (UMIN000030232) evaluated whether zinc supplementation was safe and effective for improving outcomes among patients with acute myocardial infarction (AMI). Within 24 h after successful primary percutaneous coronary intervention, consenting patients with AMI were randomly assigned 1:1 to receive conventional treatment (conventional treatment group) or conventional treatment plus zinc acetate supplementation (zinc supplementation group). The two groups were compared in terms of major adverse cardiovascular events (MACE), and scar size, which was evaluated using cardiac magnetic resonance imaging (CMR) at 4 weeks after discharge. A total of 56 patients underwent randomization (with 26 assigned to the zinc supplementation group and 27 to the conventional treatment group). The two groups had generally similar laboratory findings and clinical characteristics. The two groups also had similar lengths of hospital stay and rates of MACE. Forty of the 53 patients underwent CMR and it revealed that % core zone was numerically lower in the zinc supplementation group than in the conventional treatment group (9.3 ± 6.9% vs. 14.2 ± 9.1%, P = 0.07). This small single-centre study failed to detect a significant reduction in mid-term MACE after AMI among patients who received zinc supplementation., (© 2023. Springer Nature Japan KK, part of Springer Nature.)

This study aimed to determine the optimal cut-off value of the early drop in systolic blood pressure (SBP) for worsening renal function (WRF) in hospitalized patients with heart failure (HF) and analyze predictors of WRF and the early drop in SBP at that threshold. We retrospectively enrolled 396 patients with acute decompensated HF. The early drop in SBP was defined as the difference between baseline and SBP measured 24 h after hospitalization. We performed receiver operating characteristic (ROC) analysis to determine the optimal cut-off value of the early drop in SBP for WRF and evaluated the effect of the early drop in SBP on in-hospital mortality by multivariate logistic regression analyses. The mean age of the patients was 73.4 ± 14.7 years, and 61.2% were men. A 14.0% drop in SBP was identified as the optimal cut-off value for WRF from the ROC curve analysis. An early drop in SBP ≥ 14.0% was associated with WRF in multivariate logistic regression analysis (odds ratio 7.84; 95% confidence interval 4.06-15.14; P < 0.0001). The dose of intravenous furosemide within 24 h of admission was one of the predictors of the early drop in SBP ≥ 14.0%, while no early drop in SBP was a predictor of in-hospital mortality in multivariate logistic regression models. In conclusion, the optimal cut-off value for WRF in patients with HF was a 14.0% drop in SBP within 24 h of admission. The early drop in SBP ≥ 14.0% was one of the predictors of WRF in patients with HF. However, no early drop in SBP was associated with in-hospital mortality. This study was registered with the University Hospital Medical Information Network in Japan (UMIN000035989)., (© 2022. Springer Japan KK, part of Springer Nature.)

Computed tomography (CT)-derived three-dimensional maximum principal strain (MP-strain) can provide incremental value to coronary CT angiography for cardiac dysfunction assessment with high diagnostic performance in patients with myocardial infarction. Global longitudinal strain (GLS) measured using two-dimensional speckle tracking echocardiography (2D-STE) is more sensitive than left ventricular ejection fraction (LVEF) for detecting early myocardial dysfunction. We aimed to compare CT-derived MP-strain with each of 2D-STE-derived strains (i.e., longitudinal, circumferential, and radial strains), and identify the major determinants of CT-derived MP-strain among 2D-STE-derived strains. We studied 51 patients who underwent cardiac CT and echocardiography. CT images were reconstructed at every 5% (0-95%) of the RR interval. A dedicated workstation was used to analyze CT-derived MP-strain on the 16-segment model. We calculated CT-derived global MP-strain with all the 16 segments on a per patient basis. Pearson's test was used to assess correlations between CT-derived MP-strain and STE-strain at global and segmental levels. The intra-class correlation coefficient for interobserver agreement for CT-derived global MP-strain was 0.98 (95% confidence interval 0.96-0.99). The low-CT-derived global MP-strain group (≤ 0.43) had more patients with LV dysfunction than the high-CT-derived global MP-strain group (> 0.43). CT-derived global MP-strain was associated with STE-GLS (r = 0.738, P < 0.001), global circumferential strain (r = 0.646, P < 0.001), and global radial strain (r = 0.432, P = 0.001). In multivariate analysis, STE-GLS had the strongest association to CT-derived global MP-strain among three directional STE-strains and LVEF by echocardiography (standardized coefficient =  - 0.527, P < 0.001). STE-GLS is a major determinant of CT-derived global MP-strain. CT-derived MP-strain may enhance the value of coronary CT angiography by adding functional information to CT-derived LVEF., (© 2021. Springer Japan KK, part of Springer Nature.)

Berlin Heart EXCOR® (BHE) ventricular assist device (VAD) (Berlin Heart, Berlin Heart AG, Berlin, Germany) implantation is prevalent in patients with severe heart failure. However, clinical outcomes of pediatric patients on long-term BHE support remain mainly unknown. This study aimed to report our clinical experience with long-term support of pediatric patients with severe heart failure supported by BHE VAD. Clinical outcomes of 11 patients (median age 8.4 months; two male), who underwent LVAD implantation of the Berlin Heart EXCO® (BHE) VAD (Berlin Heart, Berlin Heart AG, Berlin, Germany) between 2013 and 2017 at our institution were reviewed. The median support period was 312 (range 45-661) days and five patients were supported for more than 1 year. The longest support duration was 661 days. No mortality occurred, and six patients were successfully bridged to heart transplantation, while three patients were successfully weaned off the device. Two patients are currently on BHE support while they await heart transplantation. Four patients had cerebral bleeding or infarction, but only one case of persistent neurological deficit occurred. No fatal device-related infection occurred during LVAD support. BHE VAD can provide long-term support for pediatric patients with severe heart failure with acceptable mortality and morbidity rates with long-term support.

The purpose of the study is to analyze the impact of hybrid stage 1 palliation on right ventricular myocardial pathology in hypoplastic left heart syndrome. Sufficient amount of right ventricular biopsies could be obtained from 16 of 32 patients who underwent Norwood operation between 2007 and 2013. Histopathological findings of right ventricle in patients who underwent primary Norwood operation (primary group, n = 5), patients with aortic atresia (HS1P AA group, n = 6) or aortic stenosis (HS1P AS group, n = 5) who underwent staged Norwood palliation following hybrid stage 1 palliation were compared. To eliminate the influence of right ventricular pressure afterload, right ventricular biopsies were obtained from patients with truncus arteriosus communis (TAC group, n = 6) at total correction. The percentage of myocardial fibrosis was significantly higher in both HS1P groups than in TAC group; moreover, it was significantly higher in HS1P AA group than in primary group. Capillary vascular density was significantly lower in all hypoplastic left heart syndrome groups than in TAC group. At the sub-endocardial layer, collagen type I/III ratios were higher in HS1P AA group than in other hypoplastic left heart syndrome groups. The proportions of N-cadherin immunolocalized to myocyte termini were lower in all hypoplastic left heart syndrome groups than in TAC group. Right ventricle in hypoplastic left heart syndrome showed more significant ischemic change and myocardial immaturity than that in truncus arteriosus communis. Hybrid stage 1 palliation for aortic atresia would be a risk factor for further right ventricular myocardial ischemia.

To assess a two-phase contrast injection protocol for contrast enhancement during cardiac computed tomography (CT) in children with congenital heart disease. Forty-three children (20 boys, 23 girls) of median age 13 months (range 3 days-8.3 years) and weighing ≤ 20 kg who underwent cardiac CT using a two-phase contrast injection protocol at our institution were retrospectively identified. High-pitch spiral third-generation dual-source cardiac CT (tube voltage 70 kV) was performed with a fixed delay of 60 s after contrast injection in the order of 10 mgI/kg/s (30 s), 15 mgI/kg/s (20 s), and a saline chaser (10 s). Attenuation in the inferior vena cava (IVC), superior vena cava (SVC), right atrium (RA), right ventricle (RV), pulmonary artery (PA), left atrium (LA), left ventricle (LV), and descending aorta (AO) was compared using the Steel-Dwass and Fisher's exact tests. The median (interquartile range) attenuation in the IVC, SVC, RA, RV, PA, LA, LV, and AO was 285 (264-347) Hounsfield units (HU), 416 (370-445) HU, 368 (320-388) HU, 373 (322-417) HU, 397 (330-432) HU, 425 (373-469) HU, 435 (385-468) HU, and 437 (392-491) HU, respectively (p < 0.05, IVC vs. the other anatomic sites). There was no significant difference in diagnostic success rate for attenuation > 250 HU between the IVC (41 children, 95.3%) and the other sites (43 children, 100%). A two-phase contrast injection protocol is useful for effective contrast enhancement in pediatric cardiac CT.

This study reviewed early clinical outcomes of right ventricular outflow tract reconstruction with Contegra ® valved conduits in pediatric patients. Between April 2013 and July 2014, thirteen pediatric patients underwent right ventricular outflow tract reconstruction with Contegra valved conduits. The size of the implanted conduits were 12 mm in 5 patients, 14 mm in 3, 16 mm in 3, and then 2 patients were implanted with bicuspidized conduits for downsizing the conduit to 9 and 10 mm in each. Follow-ups were completed in all patients. One conduit was explanted 7 days after a neonatal biventricular repair for Ebstein's anomaly and pulmonary atresia, timed to be at the point of conversion to a single ventricular palliation. Among the 5 patients who developed significant pulmonary insufficiency and/or conduit stenosis, 3 patients exhibited persistent pulmonary hypertension. Both the bicuspidized conduits resulted in early pulmonary insufficiency. One patient implanted with a ring-supported conduit developed coronary artery stenosis, caused by suppression between the ring of the implanted conduit and the annulus of the mechanical vale. Although the small caliber Contegra valved conduit might be an alternative to RVOT reconstruction, the indication should be carefully considered. More than mild pulmonary hypertension, and low body weight at operation of less than 3.0 kg caused early conduit dysfunction.

Half-life of urinary cadmium level (U-Cd) was estimated in inhabitants whose initial U-Cd was ≥5 μg/L (131 men and 177 women) or ≥5 μg/gcr (195 men and 246 women), using a linear mixed model adjusted for the baseline age. To clarify the effect of initial U-Cd, the target participants were divided into higher or lower initial U-Cd group. In the higher groups, the half-lives were 15.4 and 13.1 years for unadjusted U-Cd and 19.0 and 23.0 years for creatinine-adjusted U-Cd, in men and women, respectively. In the lower groups, the half-lives were 38.0 and 26.0 years for unadjusted U-Cd in men and women. For creatinine-adjusted U-Cd, it was 42.9 years in men. For attenuation of U-Cd, there were an early fast component shown in the higher group and late slow component shown in the lower group. The attenuation of U-Cd is slower in the longer time compared to that previously reported.

Background: Asthma is characterized by airflow limitation with chronic airway inflammation, hyperresponsiveness and mucus hypersecretion. NO is generated by three nitric oxide synthase (i/n/eNOSs) isoforms, but conflicting results have been reported using asthmatic mice treated with NOSs inhibitors and NOS-knockout mice. To elucidate the authentic role of NO/NOSs in asthma, we used asthmatic mice lacking all NOSs (n/i/eNOS(-/-))., Methods: Wild-type and n/i/eNOS(-/-) mice were sensitized and challenged with ovalbumin. Pathological findings and expressions of interferon (IFN)-γ, interleukin (IL)-4, -5, -10, -13 and chemokines in the lung were evaluated., Results: Decreased eosinophilic inflammation, bronchial thickening and mucus secretion, IL-4, -5 and -13, monocyte chemoattractant protein-1, eotaxin-1 and thymus and activation-regulated chemokine expressions were observed in n/i/eNOS(-/-) mice compared to wild-type, but expressions of IFN-γ and IL-10 were similar., Conclusion: Using asthmatic n/i/eNOS(-/-) mice, NO plays important roles in accelerating bronchial eosinophilic inflammation and mucus hypersecretion in the pathophysiology of asthma.

Background: IgG4-related disease (IgG4RD) is a recently recognized disease entity. Differentiating IgG4RD from plasma cell type Castleman's disease (PCD) is important but also difficult using only pathological findings. In addition, little is known about the association between these two diseases with diffuse parenchymal lung involvement., Methods: We analyzed the serum IgG4 levels and the ratio of IgG4/IgG-positive plasmacytes in the lung and lymph node specimens of eight patients previously pathologically diagnosed of PCD with diffuse parenchymal lung involvement (DL-PCD). We also compared the clinical and laboratory findings observed in these patients., Results: Six of the eight patients exhibited abundant IgG4-positive plasmacytes in the lung and lymph node tissues and elevated serum IgG4 levels, thereby fulfilling the diagnostic criteria of IgG4RD with DL (DL-IgG4RD) in addition to having obstructive phlebitis and massive lymphoplasmacytic infiltration with fibrosis. However, three of these six patients exhibited higher levels of serum interleukin-6 and were still diagnosed with DL-PCD. Accordingly, three of these eight patients were considered as IgG4RD with DL (DL-IgG4RD), and the other five patients were ultimately given a diagnosis of DL-PCD. These two diseases have different characteristics in terms of age, symptoms, serum levels of C-reactive protein, and IgA, complicating allergic disorders, response to corticosteroids, and prognosis., Conclusions: This is the first report to show a high prevalence of DL-IgG4RD in DL-PCD patients, although additional large investigations are necessary. Clinical and laboratory findings are important for distinguishing between these two diseases in other organs, as previously described.

Aims/hypothesis: Little is known about the relationship between the HOMA of insulin resistance (HOMA-IR) and the risk of cardiovascular events in Asian populations, which have lower levels of HOMA-IR than Western populations. Accordingly, we determined the predictive value of HOMA-IR for cardiovascular risk in a Japanese population that was apparently free of diabetes, addressing whether insulin resistance itself increases cardiovascular risk independently of other relevant metabolic disorders., Methods: We followed 2,548 non-diabetic men aged 35 to 59 years for 11 years. The hazard ratios for the incidence of cardiovascular events due to increased HOMA-IR were estimated using a Cox proportional hazards model that was adjusted for potential confounding factors., Results: The multivariate-adjusted hazard ratio for cardiovascular events compared with the first quartile of HOMA-IR (or=1.52). The hazard ratio associated with a one SD (0.61) increment in log-transformed HOMA-IR was 1.51 (1.13-2.02). A similar positive relationship was observed for coronary events and stroke. In addition, the relationship between HOMA-IR and cardiovascular risk was broadly similar in participants with and without hypertension, dyslipidaemia (elevated triacylglycerol and/or reduced HDL-cholesterol), abdominal obesity and current smoking., Conclusions/interpretation: Increased HOMA-IR predicted subsequent cardiovascular events in non-diabetic Japanese men. The association was independent of traditional cardiovascular risk factors and other relevant metabolic disorders.

The purpose of this study was to clarify the change and relationship of urinary cadmium (Cd) and beta(2)-microglobulin (beta(2)-MG) concentrations of inhabitants in Cd-polluted areas after soil restoration. The urinary Cd and beta(2)-MG concentrations of 25 males and 28 females did not show a significant change, 22 years after the Cd-polluted soil was restored. Once exposed to Cd, it was found to remain in the body, 22 years after the Cd -polluted soil was restored. However, this did not influence renal tubular dysfunction in most of the younger generation compared with elders heavily exposed to Cd.

We performed this study to determine whether both eating cadmium (Cd)-polluted rice and drinking and/or cooking with Jinzu River water are associated with renal tubular dysfunction. A multiple logistic regression analysis of retrospective data indicated that both factors may contribute to this condition. Estimated threshold values of rice Cd concentration in men were 0.13-0.27 ppm and 0.09-0.18 ppm in women, without adjustment for use of Jinzu River water. The additional influence of drinking and/or cooking with Jinzu River water was estimated to be about 0.008 ppm.

An 86-year-old woman was admitted to our hospital because of vomiting and anorexia. Although serum cardiac markers, an electrocardiogram, and echocardiography suggested acute myocardial infarction, emergency cardiac catheterization revealed akinesis of the left ventricular apex without significant coronary artery stenosis. She was diagnosed as having takotsubo cardiomyopathy. The left ventricular dysfunction was considered transient and reversible but did not improve at all, contrary to our expectations. She died of worsening heart failure on day 14. We discuss this serious clinical course of a very elderly patient with takotsubo cardiomyopathy.