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This study was intended to determine the impact of extracardiac anomalies on outcomes in patients with functional single ventricle who underwent staged palliation. We reviewed medical records of patients who underwent first-stage palliation at our center between 2001 and 2020. The prevalence and type of extracardiac anomalies were evaluated, and their impact on outcomes during staged palliation was analyzed. Among 602 patients who underwent first-stage palliation, 81 (14%) patients had associated with extracardiac anomalies. They were more frequently associated with prematurity (P =.03) and low birth weight below 2.5 kg (P <.01). Mortality between first-stage palliation and stage II was similar in patients with and without extracardiac anomalies (24.7% vs 17.1%, P =.10). However, mortality between stage II and stage III was significantly higher in patients with extracardiac anomalies compared with those without (22.2% vs 12.5%, P =.02). Mortality after stage III was also higher in patients with extracardiac anomalies compared with those without (4.9% vs 1.5%, P =.04). In the subgroup analysis of 81 patients with extracardiac anomalies, renal anomalies were identified as a significant risk factor for mortality (P =.03, hazard ratio 2.44). The incidence of extracardiac anomalies in this study was 14%, and patients with extracardiac anomalies were highly associated with prematurity and low birth weight. Presence of extracardiac anomalies was associated with higher mortality between stage II and stage III palliation and after stage III phase, but not before stage II. Among extracardiac anomalies, renal anomalies were identified as a risk factor for mortality. [ABSTRACT FROM AUTHOR]

Total anomalous pulmonary venous connection (TAPVC) with a functional single ventricle is a risk factor for mortality during staged palliation. This study aimed to assess TAPVC's impact on staged palliation outcomes. In a total of 602 patients with a functional single ventricle who underwent stage 1 palliation (S1P) at our center between 2001 and 2020, 39 (6.5%) patients were associated with TAPVC. Median age at S1P was 12.0 (interquartile range, 7-21) days with a body weight of 3.1 (interquartile range, 2.8-3.6) kg. Outcomes during staged palliation were compared with the remaining 563 patients without TAPVC. Risk factors for mortality were identified using a Cox proportional hazards regression model. Primary diagnosis in functional single-ventricle patients with TAPVC included hypoplastic left heart syndromes (n = 13), unbalanced atrioventricular septal defects (n = 12) tricuspid atresias (n = 2), double inlet left ventricle (n = 1), and others (n = 11). Types of TAPVC were supracardiac (n = 21), cardiac (n = 10), infracardiac (n = 6), and mixed (n = 2). Pulmonary venous obstruction (PVO) was associated in 19 (49%) patients. S1Ps included Norwood (n = 13), aortopulmonary shunt (n = 21), and pulmonary artery banding (n = 5). Thirty-day mortality after S1P was significantly increased in patients with TAPVC vs without TAPVC (43.6% vs 16.3%; P <.001). After bidirectional cavopulmonary shunt and total cavopulmonary connection procedures, mortality was low in both groups, and no statistically significant differences were found. Correction of TAPVC at the time of S1P was not found to be a significant risk factor in univariable Cox regression analysis. In univariate and multivariate analysis, PVO was identified as an independent risk factor for mortality in patients with TAPVC (P <.001). Overall survival is lower in TAPVC single-ventricle patients than in non-TAPVC patients. Most deaths after S1P were associated with TAPVC, but not after S2P. PVO is a mortality risk factor in TAPVC patients. [ABSTRACT FROM AUTHOR]

The study objective was to compare the results after Norwood procedure between modified Blalock-Taussig shunt (MBTS) and right ventricle-to-pulmonary artery conduit (RVPAC) according to Sano in patients with hypoplastic left heart syndrome (HLHS) and aortic atresia (AA). A total of 146 neonates with HLHS and AA who underwent the Norwood procedure at our institution between 2001 and 2020 were divided into 2 groups according to shunt type (MBTS or RVPAC). Survival after the Norwood procedure was compared between the groups. Longitudinal right ventricular and tricuspid valve function in each group were evaluated using cubic splines method. RVPAC was performed in 103 patients and MBTS in 43 according to surgeon preference. There were no differences in the 30-day mortality rates (16.5% vs 16.3%, P  = 0.973). Survival at 0.5, 1 and 3 years was 79.6%, 74.6%, and 68.9% in RVPAC and 66.8%, 64.3%, and 58.5% in MBTS (P  =  0.293). Among 23 patients undergoing tricuspid valve procedure, different mechanisms of tricuspid regurgitation were observed between the groups. Longitudinal analysis revealed greater prevalence of late right ventricular dysfunction in RVPAC patients. In 77 patients who completed Fontan procedure, the postoperative N-terminal pro B-type natriuretic peptide value was significantly higher in RVPAC vs MBTS (554 vs 276 ng/L, P  =  0.007). No survival advantage of RVPAC over MBTS was observed in neonates with HLHS and AA undergoing the Norwood procedure. Longitudinal analysis demonstrated a greater prevalence of right ventricular dysfunction and higher N-terminal pro B-type natriuretic peptide values during late follow-up in patients with RVPAC. [ABSTRACT FROM AUTHOR]

This study sought to determine long-term outcomes of a primary Damus-Kaye-Stansel (DKS) procedure in patients with a functional single ventricle and to compare the results with those of our historical control subjects who underwent pulmonary artery banding before the DKS procedure. The study reviewed the medical records of all patients who underwent the DKS procedure at the German Heart Center of Munich, Germany between December 1994 and December 2019. The DKS procedure was performed as initial palliation in 52 patients (primary DKS group) and as staged palliation after pulmonary artery banding in 24 patients (staged DKS group). The median follow-up period after the DKS procedure was 8.9 years in the primary DKS group and 8.0 years in the staged DKS group. The survival rates at 10 years after the DKS procedure were 89% in the primary DKS group and 68% in the staged DKS group (log-rank P = 0.04). Before total cavopulmonary connection, the pressure gradient through the systemic ventricular outflow tract was significantly lower in the primary DKS group than in the staged DKS group (P <.001). At last follow-up echocardiography, reduced ventricular function was observed in 1 patient in the primary DKS group and in 7 patients in the staged DKS group (P <.001). The degree of neoaortic regurgitation was significantly higher in the staged DKS group than in the primary DKS group (P <.001). A primary DKS procedure in patients with a functional single ventricle and potential systemic ventricular outflow tract obstruction is recommended to obtain favorable long-term survival with preserved ventricular function and competent semilunar valve function. [ABSTRACT FROM AUTHOR]

Thrombus formation is a feared complication following bidirectional cavopulmonary shunt (BCPS). We aimed to investigate the effect of thrombus formation on outcome. BCPS was performed in 525 patients at our center between 1998 and 2018. The impacts of thrombus formation on survival and probability of Fontan completion were analyzed, and risk factors for thrombus formation were examined. Thrombus formation occurred in 30 patients (5.7%). Compared with the remaining 495 patients, there was no significant difference in the median age at BCPS (4.9 vs 4.7 months; P = 0.587). However, unbalanced atrioventricular septal defects (17 vs 5%; P = 0.008) and preoperative ventricular dysfunction (23.3 vs 8%; P = 0.004) were more frequent in patients who developed a thrombus. Thrombolytic therapy was performed in all patients and surgical thrombus removal was required in 13 patients. In-hospital mortality was higher in patients with thrombus (30.0 vs 2.2%; P < 0.001). Of 505 hospital survivors, an estimated survival at 1 year after hospital discharge following BCPS was 84.4% (95% CI, 76.1-92.7%) in patients with thrombus and 96.8% (95% CI, 96.0-97.6%) in those without (P < 0.001). Cumulative incidence of Fontan completion at 3 years after BCPS was 52.8% (95% CI, 30.3-75.2%) in patients with thrombus and 90.1% (95% CI, 87.2-92.9%) in those without (P = 0.004). Higher left atrial pressure (OR = 1.165; P = 0.029) and longer cardiopulmonary bypass time (OR = 1.013, P = 0.001) at BCPS were independent risk factors for thrombus formation after BCPS. Thrombus formation after BCPS poses a significant risk for survival and Fontan completion. Preoperative higher left atrial pressure and longer cardiopulmonary bypass time are significant risk factors. [ABSTRACT FROM AUTHOR]

A 63-year-old Japanese woman with multiple cysts in both lungs on chest computed tomography (CT) was referred to our hospital after a thorough examination, including a transbronchial lung biopsy (TBLB), failed to provide a diagnosis. Based on the findings on chest CT and pathological examination of the bronchoalveolar lavage fluid and transbronchial lung cryobiopsy (TBLC) specimen, the patient was diagnosed with pulmonary Langerhans cell histiocytosis (PLCH). TBLC may replace TBLB as the main diagnostic technique for PLCH, although further studies are required to determine the usefulness of TBLC for the diagnosis of PLCH. [ABSTRACT FROM AUTHOR]

Background This study aimed to identify the prognostic value of the preoperative stroke volume ratio (right ventricular stroke volume/left ventricular stroke volume) for redilatation of the right ventricle after pulmonary valve replacement in patients with repaired tetralogy of Fallot. Methods From April 2004 to November 2013, 20 patients with repaired tetralogy of Fallot underwent pulmonary valve replacement for pulmonary valve regurgitation and right ventricular dilatation. Serial changes in ventricular volume were examined by cardiac magnetic resonance or computed tomography imaging. The redilatation ratio was calculated for right ventricular end-diastolic and end-systolic volume indices by dividing the increment in right ventricular volume from the first (median, 1.1 years) to the second (median, 3.2 years) evaluations after pulmonary valve replacement by the first evaluation. The relationships between the stroke volume ratio and redilatation ratio were assessed. The degree of right ventricular myocardial fibrosis was examined in 13 patients and compared with the stroke volume ratio. Results Right ventricular volume (redilatation) significantly increased from a median of 1.1 to 3.2 years after pulmonary valve replacement. Significant positive correlations were detected between the stroke volume ratio and redilatation ratio of the right ventricular end-diastolic ( r = 0.50. p = 0.02) and end-systolic volume indices ( r = 0.49, p = 0.03). The stroke volume ratio also showed a significant positive correlation with the degree of right ventricular myocardial fibrosis ( r = 0.73, p = 0.005). Conclusions The preoperative stroke volume ratio can predict redilatation of the right ventricle after pulmonary valve replacement and the degree of right ventricular myocardial fibrosis. [ABSTRACT FROM AUTHOR]

We sought to identify the impact of pulmonary artery size on outcomes after nonfenestrated total cavopulmonary connection. In a subgroup of patients with right-sided bidirectional cavopulmonary shunt, the impact of each branch pulmonary artery size was individually determined. The medical records of all patients who underwent nonfenestrated total cavopulmonary connection between 2009 and 2021 were reviewed. The pulmonary artery index was calculated using angiography before the operation. A total of 247 patients were included in this study. A right-sided bidirectional cavopulmonary shunt was performed in 217 patients (88%). Median pulmonary artery index was 162 (133-207) mm2/m2 before total cavopulmonary connection. Chylothorax occurred in 55 patients (22%). Pulmonary artery index was an independent factor for chylothorax (odds ratio, 0.98, 95% confidence interval, 0.97-0.99, P <.001) with a cutoff value of 170 mm2/m2. In a subgroup of patients with right-sided bidirectional cavopulmonary shunt, the left pulmonary artery index was identified as an independent risk factor for longer stay in the intensive care unit (coefficient B –0.02, 95% confidence interval, –0.04 to –0.002, P =.034) and for adverse events (hazard ratio, 0.98, 95% confidence interval, 0.96-0.99, P =.011) with a cutoff value of 56 mm2/m2. The pulmonary artery index is significantly associated with the occurrence of chylothorax after nonfenestrated total cavopulmonary connection with a cutoff value of 170 mm2/m2. In patients with right-sided bidirectional cavopulmonary shunt, left pulmonary artery index has a significant predictive value for longer stay in the intensive care unit and adverse events with a cutoff value of 56 mm2/m2. [Display omitted] [ABSTRACT FROM AUTHOR]

Abstract: Objective: Exposure to particulate matter air pollution may be an independent risk factor for cardiovascular morbidity and mortality; however, the biological mechanisms are unclear. We hypothesize that exposure to diesel exhaust (DE), an important source of traffic-related particulate air pollution, promotes changes of atherosclerotic plaque component that may lead to plaque vulnerability. Methods and results: 30-week old ApoE knockout mice fed with regular chow inhaled DE (at 200μg/m3 of particulate) or filtered-air (control) for 7 weeks (6h/day, 5 days/week) (12mice/group). Total number of alveolar macrophages (p <0.01) and alveolar macrophages positive for particles (p <0.0001) were more than 8-fold higher after DE inhalation than the control. DE inhalation caused 1.5 to 3-fold increases in plaque lipid content (p <0.02), cellularity (p <0.02), foam cell formation (p <0.04), and smooth muscle cell content (p <0.05). The expression of oxidative stress markers, iNOS, CD36, and nitrotyrosine was significantly increased by 1.5 to 2-fold in plaques, with enhanced systemic lipid and DNA oxidation (p <0.02). Increased foam cells and the expression of iNOS (R 2 =0.72, p =0.0081) and CD36 (R 2 =0.49, p =0.015) in plaques were positively correlated with the magnitude of DE exposure. Conclusions: Exposure to DE promotes changes in atherosclerotic plaques characteristic of unstable vulnerable plaques. Increased systemic and plaque oxidative stress markers suggest that these changes in plaques could be due to DE-induced oxidative stress. [Copyright &y& Elsevier]

In this study, we sought to identify independent risk factors for mortality and reintervention after early surgical correction of truncus arteriosus using a novel statistical method. Patients undergoing neonatal/infant truncus arteriosus repair between January 1984 and December 2018 were reviewed retrospectively. An innovative statistical strategy was applied integrating competing risks analysis with modulated renewal for time-to-event modeling. A total of 204 patients were included in the study. Mortality occurred in 32 patients (15%). Smaller right ventricle to pulmonary artery conduit size and truncal valve insufficiency at birth were significantly associated with overall mortality (right ventricle to pulmonary artery conduit size: hazard ratio, 1.34; 95% confidence interval, 1.08-1.66, P =.008; truncal valve insufficiency: hazard ratio, 2.5; 95% confidence interval, 1.13-5.53, P =.024). truncal valve insufficiency at birth, truncal valve intervention at index repair, and number of cusps (4 vs 3) were associated with truncal valve reoperations (truncal valve insufficiency: hazard ratio, 2.38; 95%, confidence interval, 1.13-5.01, P =.02; cusp number: hazard ratio, 6.62; 95% confidence interval, 2.54-17.3, P <.001). Right ventricle to pulmonary artery conduit size 11 mm or less was associated with a higher risk of early catheter-based reintervention (hazard ratio, 1.54; 95% confidence interval, 1.04-2.28, P =.03) and reoperation (hazard ratio, 1.96; 95% confidence interval, 1.33-2.89, P =.001) on the right ventricle to pulmonary artery conduit. Smaller right ventricle to pulmonary artery conduit size and truncal valve insufficiency at birth were associated with overall mortality after truncus arteriosus repair. Quadricuspid truncal valve, the presence of truncal valve insufficiency at the time of diagnosis, and truncal valve intervention at index repair were associated with an increased risk of reoperation. The size of the right ventricle to pulmonary artery conduit at index surgery is the single most important factor for early reoperation and catheter-based reintervention on the conduit. A novel analysis for risks of mortality and reintervention after truncus arteriosus repair. [Display omitted] [ABSTRACT FROM AUTHOR]

A 60-year-old Japanese man with a history of heavy smoking came to our hospital for a detailed examination, suspecting interstitial pneumonia because of gradually increasing dyspnea on exertion over a period of one year. Chest high-resolution computed tomography revealed ground-glass shadows with emphysematous changes. Pathological analysis of samples obtained using transbronchial lung cryobiopsy revealed an accumulation of alveolar macrophages with abundant eosinophilic cytoplasm in the alveolar space. Following a multidisciplinary discussion, the patient was diagnosed with desquamative interstitial pneumonia. To our knowledge, this is the first detailed report of desquamative interstitial pneumonia diagnosed using transbronchial lung cryobiopsy. [ABSTRACT FROM AUTHOR]

Inhaled liposomal antimicrobials are known to cause hypersensitivity pneumonitis. Amikacin liposome inhalation suspension (ALIS) is a promising novel antimicrobial agent against refractory Mycobacterium avium complex infections. The frequency of drug-induced lung injury caused by ALIS is relatively high. To date, no reports of ALIS-induced organizing pneumonia diagnosed by bronchoscopy are available. We report a case of a 74-year-old female patient presenting with non-tuberculous mycobacterial pulmonary disease (NTM-PD). She was treated with ALIS for refractory NTM-PD. Fifty-nine days after starting ALIS, the patient developed a cough, and her chest radiographs indicated deterioration. She was diagnosed with organizing pneumonia based on pathological findings of the lung tissues obtained by bronchoscopy. After switching from ALIS to amikacin infusion, her organizing pneumonia improved. It is difficult to distinguish between organizing pneumonia and an exacerbation of NTM-PD based on chest radiography alone. Therefore, it is essential to perform an active bronchoscopy for diagnosis. [ABSTRACT FROM AUTHOR]

Systemic corticosteroid therapy is occasionally used as an additive therapy, especially for patients with severe pneumonia. However, its recommendation for use in patients with pneumonia varies worldwide, and its efficacy is unclear. Adult Japanese patients hospitalized with community-onset pneumonia between January and December 2012 were analyzed using the Diagnostic Procedure Combination database. The patients were classified into mild-to-moderate and severe groups using the A-DROP (age, dehydration, respiration, orientation, and blood pressure) system. The 90-day survival rate was evaluated between the presence or absence of corticosteroid treatment using the Kaplan-Meier method in the overall, mild-to-moderate and severe groups, respectively. The patients' clinical characteristics were adjusted between the two groups using the inverse probability of treatment weighting method. Among 123,811, 110,534 patients were classified as mild-to-moderate grade (corticosteroid group: 8,465, non-corticosteroid group: 102,069) and 13,277 patients were classified as severe grade (corticosteroid group: 1,338, non-corticosteroid group: 11,939). The 90-day survival rate was higher in the non-corticosteroid group than in the corticosteroid group in patients with pneumonia of overall grade (weighted hazard ratio [HR]: 1.36; P < 0.001) and those with mild-to-moderate grade (weighted HR: 1.46; P < 0.001). However, there were no significant differences in the outcomes between the two groups in those with severe grade (weighted HR: 1.08; P = 0.38). Additive systemic corticosteroid therapy may be related to poor 90-day prognosis in patients with mild-to-moderate grade community-onset pneumonia, although it may not be positively associated with its prognosis in those with severe grade. [ABSTRACT FROM AUTHOR]

Donation after circulatory death (DCD) offers an additional source of cardiac allografts, potentially allowing expansion of the donor pool, but is limited owing to the effects of ischemia. In this study, we investigated the efficacy of mitochondrial transplantation to enhance myocardial function of DCD hearts. Circulatory death was induced in Yorkshire pigs (40–50 kg, n = 29) by a cessation of mechanical ventilation. After 20 minutes of warm ischemia, cardioplegia was administered. The hearts were then reperfused on an ex-situ blood perfusion system. After 15 minutes of reperfusion, hearts received either vehicle alone (vehicle [VEH], 10 ml; n = 8) or vehicle containing autologous mitochondria (vehicle with mitochondria as a single injection [MT], 5 × 109 in 10 ml, n = 8). Another group of hearts (serial injection of mitochondria [MT S ]; n = 6) received a second injection of mitochondria (5 × 109 in 10 ml) after 2 hours of ex-situ heart perfusion and reperfused for an additional 2 hours. A Sham group (sham hearts; n = 6) did not undergo any warm ischemia. At the end of 4 hours of reperfusion, MT and MT S groups showed a significantly increased left ventricle/ventricular peak developed pressure (p = 0.002), maximal left ventricle/ventricular pressure rise (p < 0.001), fractional shortening (p < 0.001), and myocardial oxygen consumption (p = 0.004) compared with VEH. Infarct size was significantly decreased in MT and MT S groups compared with VEH (p < 0.001). No differences were found in arterial lactate levels among or within groups throughout reperfusion. Mitochondrial transplantation significantly preserves myocardial function and oxygen consumption in DCD hearts, thus providing a possible option for expanding the heart donor pool. [ABSTRACT FROM AUTHOR]

Background We previously reported a decrease in the vaccine serotypes of a 7-valent pneumococcal conjugate vaccine (PCV7) and pneumococcal polysaccharide vaccine (PPSV) 23 in adult pneumonia patients after starting PCV7 vaccination in children in Japan between 2011 and 2013, suggesting that the vaccination of children had an indirect effect on adults. PCV7 was replaced by PCV13 in 2013 and was authorized for individuals ≥65 in 2014; vaccination with PPSV23 has been routinely implemented since the same year. We continuously evaluated the pneumococcal serotype changes. Methods This retrospective epidemiological study was performed at the University of Occupational and Environmental Health, Japan, from January 2014 to December 2015, while also referring to the data from January 2011 to December 2013. The pneumococcal serotypes that were isolated from pneumonia patients and clinical information were evaluated. Results The proportions of the PCV7 and PCV13 vaccine serotypes significantly decreased each year (from 2011 to 2015) from 46.4% to 8.3% (p < 0.05) and 71.4% to 33.3% (p < 0.05), respectively. The PPSV23 serotypes without PCV13 showed a continuous, mild increase, while the mortality rates tended to decrease in patients with pneumococcal pneumonia. Discussion The present study showed that the vaccine serotypes of PCV7 and PCV13 have been decreasing since the introduction of PCV7 in October 2009 and since PCV13 was introduced to replace PCV7 from November 2013, and that the mortality rates of patients have tended to decrease. These results indicate that a continuous analysis of the pneumococcal serotype data is necessary for the appropriate administration of vaccines. [ABSTRACT FROM AUTHOR]

Background Pneumonia is now the third leading cause of death in Japan, and the number of patients with pneumonia is expected to increase with the progression of aging of the country. Higher dosage of antibiotics has recently been used, and high-dose doripenem (DRPM; 3 g daily) was approved for use in Japan in April 2011. However, there is a lack of data regarding the efficacy and safety of high-dose DRPM in Japan. This study prospectively assessed the clinical efficacy and safety of high-dose DRPM in Japanese patients with refractory pneumonia. Methods This study was performed at University of Occupational and Environmental Health, Japan and affiliated hospitals. The efficacy and safety of DRPM in patients with pneumonia treated with 3 g daily of DRPM, including those in whom DRPM dosage was increased from 1.5 g to 3 g daily, were evaluated. Results The safety evaluations included 56 patients, and the efficacy was evaluated in 51 patients. DRPM (3 g daily) treatment showed the response rate of 92.2% (47/51). Adverse effects of DRPM (3 g daily) were observed in 11 of 56 patients (19.7%), and all of these patients improved after DRPM cessation. Two patients had to stop DRPM administration due to elevated aminotransferase levels. The efficacy of DRPM in patients with pneumonia in whom DRPM was increased from 1.5 g daily to 3 g daily was 84.6% (11/13). Conclusions High-dose DRPM (3 g daily) treatment is effective and relatively safe in Japanese patients with pneumonia. [ABSTRACT FROM AUTHOR]