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This article presents updates and an overview of pediatric infective endocarditis. It includes a discussion of presentation of illness, diagnosis of this disorder, differential diagnosis, treatment recommendation, and associated morbidity and mortality.

Glenn Ligon, Come Out Study #13, 2014, silkscreen on canvas on panel, 36 x 47⅞ in. (91.4 x 121.6 cm) (artwork © Glenn Ligon; photograph by Ronald Amstutz, provided by the artist, Hauser & Wirth, Ne...

There are no published data on left ventricular (LV) reverse remodeling after transcatheter aortic valve replacement (TAVR) in children. The aim of this study was to assess changes in LV echocardiographic parameters 6 months after TAVR in children.This single-center, retrospective study included all 22 patients (age21 years) who underwent TAVR. The median age was 14.7 years (interquartile range, 13.3-15.9 years), median weight was 57 kg (interquartile range, 46.0-66.3 kg), and 59% of patients were male. Demographics, type and duration of aortic valve dysfunction, symptom and treatment data, and preprocedural and 6-month follow-up echocardiographic data (LV volume, mass, end-diastolic dimension, end-systolic dimension, ejection fraction [EF], sphericity, and longitudinal strain) were collected. Failure to reverse remodel at 6 months was defined as meeting at least two of the following: Z score ≥ 2 that was unchanged or increased from baseline for LV volume, mass, end-diastolic dimension, or end-systolic dimension; abnormally high sphericity index that was unchanged or increased; and abnormally low EF or longitudinal strain. Median, interquartile range, and range are reported for continuous variables, and pre- and post-TAVR data were compared using the Wilcoxon signed rank test.Eight patients (36%) had isolated aortic stenosis, four (18%) had isolated regurgitation, and 10 had (46%) mixed disease. Twelve (55%) had symptoms and 20 (91%) had prior surgical or catheter valve interventions. The primary complication was left bundle branch block, occurring in four children (18%). At 6 months, LV volume, mass, end-diastolic dimension, end-systolic dimension, and sphericity index improved. EF and strain were normal at baseline and at follow-up. Of three patients who failed to reverse remodel, two had left bundle branch block. Of three patients with persistent symptoms, one had failure of reverse remodeling.Most pediatric patients had evidence of reverse LV remodeling 6 months after TAVR, suggesting a possible alternative to surgical aortic valve replacement in this population. Functional parameters (EF and strain) were normal at baseline and follow-up. Future studies are needed to determine optimal timing of TAVR and to explore the association of postprocedural left bundle branch block on failed reverse remodeling and outcomes in this population.

Objectives:To compare patients treated for incomplete Kawasaki disease whose practitioners followed versus did not follow American Heart Association criteria and to evaluate the association of cardiology consultation with adherence to these guidelines.Study design:Single centre retrospective cohort study of patients Results:Of the 357 patients treated for Kawasaki disease, 109 (31%) were classified as incomplete Kawasaki disease. The American Heart Association algorithm for identifying patients with incomplete Kawasaki disease was followed in 81/109 (74%). Coronary artery abnormalities were present in 46/109 (42%) of the patients who were treated for incomplete Kawasaki disease. Cardiology consultation was more frequent in those fulfilling American Heart Association criteria for the diagnosis of incomplete Kawasaki disease versus those who did not fulfill criteria (76% versus 48%, p = 0.005).Conclusions:Over 25% of patients treated for incomplete Kawasaki disease did not meet American Heart Association guidelines. Guidelines were more frequently followed when the paediatric cardiology team was consulted. Consulting physicians with experience and expertise in the evaluation and management of incomplete KD should be strongly considered in the care of these patients.

Neutron energy-dependent angular distributions were observed for individual $\ensuremath{\gamma}$ rays from the 0.74 eV $p$-wave resonance of $^{139}\mathrm{La}+n$ to several lower excited states of $^{140}\mathrm{La}$. The $\ensuremath{\gamma}$-ray signals were analyzed in a two-dimensional histogram of the $\ensuremath{\gamma}$-ray energy, measured with distributed germanium detectors, and neutron energy, determined with the time-of-flight of pulsed neutrons, to identify the neutron energy dependence of the angular distribution for each individual $\ensuremath{\gamma}$ rays. The angular distribution was also found for a photopeak accompanied with a faint $p$-wave resonance component. Our results can be interpreted as interference between $s$- and $p$-wave amplitudes, which may be used to study discrete symmetries of fundamental interactions.

1g model experimental studies were carried out in the laboratory to evaluate vertical performance with varying angles battered pile in dense sand. Relatively rigid piles with slenderness ratios 9 and 13.5 were undertaken in a sand-filled tank under strain-controlled lateral loading. The tank dimensions were designed so that boundary effects could be minimized and earth pressure measured using transducers at the front and bottom, and rear in each case. The relative density of 90% was achieved to simulate a more precise field condition in all test cases using the tamper compaction method. In order to determine the efficiency of the ultimate bearing capacity of screw pile configurations in lateral loading, the plate and pipe pile configurations were chosen for comparison. Totally 26 experimental cases were performed, which included vertical-batter combinations. The results indicated that the load-displacement characteristic was nonlinear under lateral loading. The case of 45 degree demonstrated higher ultimate lateral resistance.

The aim of this study was to compare the immediate and midterm echocardiographic performance of the Melody (Medtronic Inc) and Sapien (Edwards Lifesciences Inc) valves after transcatheter pulmonary valve replacement (TPVR) in native and conduit right ventricular outflow tracts (RVOTs).TPVR is now a common procedure, but limited data exist comparing postimplantation echocardiographic findings between Melody and Sapien valves.This was a single-institution retrospective cohort study of all patients who underwent successful TPVR from 2011 to 2020. Patient demographics, procedural details, and immediate and midterm echocardiographic findings were collected and compared between valve types using the Wilcoxon rank sum, chi-square, or Fisher exact test as appropriate. Subgroups were analyzed individually and were adjusted for multiple comparisons using the Bonferroni method.A total of 328 patients underwent successful TPVR (Melody: n = 202, Sapien: n = 126). The groups had a similar baseline age, weight, and diagnosis. The most common indications for TPVR were pulmonary stenosis (32.2%) or mixed disease (46%) in the Melody group and pulmonary insufficiency in the Sapien group (52.4%) (P 0.001). Sapien valves were more often placed in native RVOTs (43.7% vs 18.8%; P 0.001). The discharge and follow-up mean and peak Doppler gradients were similar between the Melody and Sapien groups. Valves implanted in native RVOTs had significantly lower postimplantation gradients at each follow-up period.Echocardiographic performance after TPVR was generally acceptable and similar when comparing Melody and Sapien valves despite differences in the indication and anatomy in each group. The peak and mean gradients were lower in transcatheter valves implanted in native RVOTs compared with those implanted in conduits or bioprosthetic valves.

BackgroundElevated serum IgG4 levels are one of the characteristic findings in immunoglobulin G4 (IgG4)-related disease (IgG4-RD). Serum IgG4 levels have an impact to a certain extent on the diagnosis of IgG4-RD although there are some issues in their sensitivity and specificity. In the reports from Japan, China, USA, and Europe, elevated serum IgG4 levels were reported to be observed in 83-97% of patients with IgG4-RD [1-5]. In the past investigations of hospital patients, some studies reported that 10-15% of hospital patients with elevated serum IgG4 levels had IgG4-RD [6,7]. However, in general adults with no symptom, investigations of prevalence of elevated serum IgG4 levels and/or IgG4-RD have rarely been conducted.ObjectivesThis study aimed to investigate the frequency of serum IgG4 elevation in the general Japanese population and its associated factors using data from resident examinations.MethodsWe measured the serum IgG4 levels in 1,204 residents who underwent a general medical examination in Ishikawa prefecture, Japan. Logistic regression analysis was used to search for factors related to elevated serum IgG4 levels. Secondary examinations were conducted for participants in whom elevation was identified.ResultsThe mean serum IgG4 level was 44 mg/dL, and elevated serum IgG4 levels were observed in 42 patients (3.5%). Univariate logistic regression analyses showed that male sex, older age, lower estimated glomerular filtration rates based on cystatin C (eGFR-CysC), serum high-density lipoprotein cholesterol levels, and higher hemoglobin A1c (HbA1c) levels were associated with elevated serum IgG4 levels. Subgroup analyses in men showed that older age, lower eGFR-CysC levels, and higher serum HbA1c levels were associated with elevated serum IgG4 levels. In contrast, the analyses in women found no significant factors. One of the 10 residents who underwent secondary examinations was diagnosed with possible IgG4-related retroperitoneal fibrosis.ConclusionIn the general population, elevated serum IgG4 levels are more common in elderly men, which is similar to the epidemiological features of IgG4-RD.References[1]Inoue D, et al. IgG4-related disease: dataset of 235 consecutive patients. Medicine (Baltimore). 2015;94(15):e680.[2]Yamada K, et al. New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases. Arthritis Res Ther. 2017;19(1):262[3]Culver EL, et al. Elevated serum IgG4 levels in diagnosis, treatment response, organ involvement, and relapse in a prospective IgG4-related disease UK cohort. Am J Gastroenterol 2016;111:733–43.[4]Lin W, et al. Clinical characteristics of immunoglobulin G4-related disease: a prospective study of 118 Chinese patients. Rheumatology (Oxford). 2015;54(11):1982–90.[5]Carruthers MN, et al. The diagnostic utility of serum IgG4 concentrations in IgG4-related disease. Ann Rheum Dis 2015;74:14-18.[6]James Yun, et al. Poor positive predictive value of serum immunoglobulin G4 concentrations in the diagnosis of immunoglobulin G4-related sclerosing disease. Asia Pac Allergy. 2014 Jul;4(3):172-176.[7]Taiwo N Ngwa, et al. Sreum immunoglobulin G4 ｌevel is a poor predictor of immunoglobulin G4–related disease. Pancreas. 2014 Jul;43(5):704-7.Disclosure of InterestsNone declared

This article presents updates and an overview of pediatric infective endocarditis. It includes a discussion of presentation of illness, diagnosis of this disorder, differential diagnosis, treatment recommendation, and associated morbidity and mortality.

Background Coronary artery abnormalities in Kawasaki disease (KD) are assessed using echocardiographic z-scores. We hypothesized that changing the coronary artery (CA) z-score model would alter diagnosis and management of children with KD. Methods In this retrospective single-center study of children treated for KD (9/2007–1/2020), we collected echocardiographic measurements for the left anterior descending (LAD), right (RCA), and left main (LMCA) coronary arteries during 3 illness phases and calculated Boston and Pediatric Heart Network (PHN) z-scores. Agreement between Boston and PHN z-scores was assessed using Kappa (κ) and Lin’s Concordance Correlation Coefficients (CCC) and Bland–Altman analysis. Results For 904 echocardiograms from 357 children, the median Boston LAD z-score was lower than the PHN (0.3 [IQR − 0.6, 1.5] vs 1.6 [IQR 0.7, 2.8], CCC 0.94 [95% CI 0.93, 0.95], moderate agreement), aggregated across all illness phases. RCA and LMCA z-scores showed substantial agreement. With conversion from Boston to PHN models, the percentage of individual LAD z-scores ≥ 2.5 increased (14.6% to 32.1%). At least one CA z-score classification changed in 213 children (59.7%) across all phases, and 48 children (13.4%) had a change that altered recommended antithrombotic strategy. Agreement between models differed by age, sex, and race. Conclusions Conversion from Boston to PHN z-scores changed at least 1 CA z-score classification in over half of KD patients and changed recommended antithrombotic management in 13%, largely driven by LAD measurements. Since diagnosis and management of KD and KD-like diseases rely upon CA z-scores, the clinical and research implications of these findings merit further exploration.

The Adult Congenital and Pediatric Cardiology (ACPC) Section of the American College of Cardiology sought to develop quality indicators/metrics for ambulatory pediatric cardiology practice. The objective of this study was to report the creation of metrics for patients with Kawasaki disease. Over a period of 5 months, 12 pediatric cardiologists developed 24 quality metrics based on the most relevant statements, guidelines, and research studies pertaining to Kawasaki disease. Of the 24 metrics, the 8 metrics deemed the most important, feasible, and valid were sent on to the ACPC for consideration. Seven of the 8 metrics were approved using the RAND method by an expert panel. All 7 metrics approved by the ACPC council were accepted by ACPC membership after an “open comments” process. They have been disseminated to the pediatric cardiology community for implementation by the ACPC Quality Network.

Large sources of parity violation have been observed in compound nuclear reactions. These enhancements are described by the compound nuclear model, which can be used to describe the angular correlation of $\gamma$-rays produced in $(\rm{n},\gamma)$ reactions in medium-heavy nuclei. It has also been suggested that these same reactions could lead to a similar enhancement in time reversal violating processes. Therefore, we are interested in verifying the compound nuclear model by studying the $(\rm{n},\gamma)$ reaction using polarized neutrons. In this paper, we discuss the installation and characterization of a neutron polarizer used in the $(\rm{n},\gamma)$ measurement with transversely polarized neutrons.

AIM To describe neurobehavioral patterns in neonates with congenital heart disease (CHD). METHOD A cohort study describing neurobehavioral performance of neonates with CHD requiring cardiac surgery. The neonates were evaluated preoperatively and postoperatively with the Neonatal Intensive Care Unit Network Neurobehavioral Scale (NNNS) and scores were compared with published normative values. Clinical factors were obtained by chart review to assess their association with behavior. The CHD NNNS score pattern was compared with previously reported profiles in other high-risk populations. RESULTS NNNS evaluations were completed on 67 neonates with CHD, resulting in 97 evaluations (50 preoperative, 47 postoperative). Compared with normative values, the cohort with CHD demonstrated decreased attention, regulation, asymmetry, stress, arousal, and excitability, along with increased non-optimal reflexes, lethargy, and need for handling (p

Objective The aim of the study was to evaluate outcomes after pediatric out-of-hospital cardiopulmonary interventions (CPIs) by emergency medical services (EMS). Methods Children (age, ≤18 years) who received CPI by EMS from 2001 to 2008 were identified from the Utah Department of Health. Cardiopulmonary intervention was defined as oxygenation, ventilation or CPR, and transport to a hospital by EMS. Univariate and multivariable regression analyses evaluated associations between potential predictors and outcomes (death and new neurologic dysfunction). Results A total of 464 patients (58% male) received EMS attention. For the 71% patients (327) who were alive on EMS arrival, 63% (205) received CPI without CPR. Of note, 6% (12) of these patients died after arrival to the hospital and new neurologic dysfunction was diagnosed in 6% (13). Among the 12 patients who died, 50% (6) were younger than 1 year.On multivariable regression analysis, factors associated with increased risk of death before and in-hospital are the following: age younger than 1 year (odds ratio [OR], 0.26; 95% confidence interval [CI], 0.17-0.39), shorter EMS transport time (OR, 0.94; 95% CI, 0.89-0.99), and longer EMS dispatch time (OR, 1.23; 95% CI, 1.08-1.40). Factors associated with increased risk of new neurologic dysfunction are the following: lack of pulse (OR, 0.14; 95% CI, 0.04-0.53), requiring CPR (OR, 6.15; 95% CI, 1.48-25.6), and CPR duration (OR, 1.20; 95% CI, 1.05-1.37). Conclusions Age younger than 1 year, shorter transport time, and longer dispatch time were associated with increased risk of death. Being pulseless upon discovery and receiving CPR were associated with new neurologic dysfunction. Maximizing EMS transport interventions for patients younger than 1 year requiring CPI may improve patient outcomes.

Disease progression of an isolated bicuspid aortic valve (BAV) in children is poorly understood and adult management guidelines may not be applicable. Thus, we sought to evaluate disease progression of pediatric isolated BAV and its relationship to current management practices.Children with a BAV and ≤mild aortic stenosis (AS) and/or aortic regurgitation (AR) at the time of initial evaluation were included in this retrospective cohort study (1/2005-12/2014). Outcomes included change in z-scores for aortic root and ascending aorta diameters, cardiac interventions, adverse outcomes, recommended follow-up interval, and frequency of cardiac imaging studies at each follow up evaluation, as well as AS/AR severity at final evaluation. Outcomes were analyzed using generalized mixed-effect models with subject and provider clustering.BAV disease progression was evaluated in 294 subjects over 4.1 ± 2.4 (range 0.2-9.5) years. Ascending aorta z-scores increased by 0.1/year (P .001) but aortic root diameter z-scores were unchanged. AS and/or AR progressed tomild in 9 (3%), 1 subject underwent cardiac intervention, and none had a major complication. Management was evaluated in 454 subjects (1343 encounters) with 27 different cardiologists. The average recommended follow-up interval was 1.5 ± 0.9 years. Younger age at diagnosis, greater aortic root or ascending aorta z-score at diagnosis, ≥mild AS/AR at follow-up, and earlier diagnosis era were associated with shorter recommended follow-up interval (P.001 for all). Imaging was obtained at 87% of follow-up encounters and was associated with age at encounter with children ≥12 years most frequently imaged (P.001). Provider accounted for 14% of variability in recommended follow-up interval and 24% of imaging variability (P.001 for both).We found little to no evidence of disease progression in children with an isolated BAV. Given the low risk, close follow-up and frequent cardiac imaging for BAV surveillance may not be warranted for children.

Management guidelines for refractory Kawasaki disease (KD) are vague. We sought to assess practice variation and identify factors associated with large/complex coronary artery aneurysms (LCAA) and resource utilization in refractory KD. This retrospective cohort study identified patients aged ≤18 years with KD (2004 to 2014) using the Pediatric Health Information System. Refractory KD was defined as receiving1 dose of intravenous immunoglobulin. Demographics, medications, concomitant infections, length of stay (LOS), and charges were collected. Antithrombotic therapy was a surrogate for LCAA. LOS and hospital charges assessed resource utilization. Multivariate regression identified factors associated with LOS, charges, and LCAA. Of 14,194 patients with KD, 2,974 (21%) had refractory KD and 203 of those 2,974 (7%) had LCAA. Additional intravenous immunoglobulin was the sole medication in 77%. Other medications added were steroids (18%), infliximab (2%), and both (3%). Warfarin, low-molecular-weight heparin, tissue plasminogen activator, and clopidogrel were prescribed with equal frequency (2%). Male gender (adjusted relative risk 1.52, 95% confidence interval [CI] 1.08 to 2.16, p0.01), admission to an intensive care unit (4.79, 95% CI 3.40 to 6.74, p0.001), arrhythmia (3.00, 95% CI 1.94 to 4.65, p0.001), and concomitant viral infection (2.29, 95% CI 1.49 to 3.52, p0.001) were associated with LCAA. Severe illness, race, region, and payer were independently associated with increased charges (p0.05 for all). In conclusion, treatment for refractory KD varies widely. Concomitant viral infection was associated with a greater risk of LCAA in refractory KD. Better understanding of optimal management may improve outcomes and decrease both variability in management and resource utilization for refractory KD.

Inflammatory heart diseases include rheumatic fever, Kawasaki disease, and myocarditis. These conditions all stem from an inflammatory process that may lead to significant cardiac morbidity and mortality. These patients often present initially to the emergency department, and prompt recognition and treatment of these conditions are essential. Rheumatic fever is caused by group A streptococcal (GAS) infections and is diagnosed using the Jones criteria. Rheumatic fever may lead to mitral and/or aortic valve dysfunction. Kawasaki disease is a systemic vasculitis that affects medium-sized arteries. Diagnostic criteria include fever, conjunctivitis, mucositis, extremity changes, rash, and lymphadenopathy. Patients may present with shock related to myocarditis and can develop aneurysms in the coronary arteries with the potential for ischemic heart disease. Treatment consists of immunomodulators and anticoagulation. Myocarditis has numerous causes but is most commonly secondary to a viral illness. Clinical presentation varies widely, but patients may present acutely in cardiogenic shock. Treatment consists of immunomodulators and heart failure medications.

The aim of this study was to evaluate the applicability of genotyping of the ABCG2 gene using MALDI-TOF MS and to estimate the allele frequency in the Japanese population.Jr (a-) phenotype has a prevalence of approximately 0·05% among Japanese blood donors; DNA-based genotyping was conducted to investigate the molecular basis of the Jr (a-) phenotype along with serological typing. To detect all SNPs of the ABCG2 gene, a high-throughput SNP genotyping platform is needed.Overall, 1004 Jr (a-) blood samples were collected from blood donors in Japan and pre-genotyped. To detect the SNPs of the ABCG2 gene using MALDI-TOF MS, polymerase chain reaction and unextend primer were designed. In total, 205 Jr (a-) samples were genotyped using MALDI-TOF MS analysis.The SNPs of 1004 Jr (a-) samples were identified using the HRM analysis and DNA sequencing, and 799 of 1004 (80%) Jr (a-) samples had the homozygous for c.376 T. The designed primers for MALDI-TOF MS perfectly detected the SNPs of the ABCG2 gene. A total of 205 Jr (a-) samples were genotyped using MALDI-TOF MS. Calling failures occurred in only two samples with the mutations c.736CT to c.376C and c.421C to c.421CA. The concordance rate between the pre-genotyped and MALDI-TOF MS-based genotyping results was very high (99·02%) for all ABCG2 alleles.Jr (a-) Japanese donors had almost the homozygous for c.376 T. However, detections of more than 20 SNPs of the ABCG2 gene for the JR blood group genotyping are needed. MALDI-TOF MS-based genotyping was highly concordant with the pre-genotyped results for all ABCG2 alleles.

AHA guidelines (2004 and 2017) provided similar algorithms for diagnosing incomplete Kawasaki disease (KD). Our aims were to describe differences between subjects treated for incomplete KD where the algorithm was or was not adhered to and evaluate the influence of cardiology consultation on

Background— Despite medical advances, children with dilated cardiomyopathy (DCM) remain at high risk of death or need for cardiac transplantation. We sought to identify predictors of disease progression in pediatric DCM. Methods and Results— The Pediatric Heart Network evaluated chronic DCM patients with prospective echocardiographic and clinical data collection during an 18-month follow-up. Inclusion criteria were age 2 months. Patients requiring intravenous inotropic/mechanical support or listed status 1A/1B for transplant were excluded. Disease progression was defined as an increase in transplant listing status, hospitalization for heart failure, intravenous inotropes, mechanical support, or death. Predictors of disease progression were identified using Cox proportional hazards modeling and classification and regression tree analysis. Of the 127 patients, 28 (22%) had disease progression during the 18-month follow-up. Multivariable analysis identified older age at diagnosis (hazard ratio=1.14 per year; P z -score (hazard ratio=1.49; P z -score (hazard ratio=0.81; P =0.01) as independent predictors of disease progression. Classification and regression tree analysis stratified patients at risk of disease progression with 89% sensitivity and 94% specificity based on LV end-diastolic M-mode dimension z -score ≥7.7, LV ejection fraction z -score Conclusions— In children with chronic stable DCM, a combination of diagnosis after late infancy and echocardiographic parameters of larger LV size and systolic and diastolic function predicted disease progression. Clinical Trial Registration— URL: http://www.clinicaltrials.gov . Unique identifier: NCT00123071.

Echocardiography is the mainstay of preoperative arch imaging in infants with coarctation of the aorta. In simple coarctation, repair by way of sternotomy or thoracotomy is often determined by echocardiographic transverse arch measurements. The degree of arch hypoplasia that is prohibitive to repair by way of thoracotomy is unknown. Clinical predictors of recoarctation are also unknown. Demographic, echocardiographic (transverse arch and aortic measurements), operative, and postoperative data of infants90 days old with simple coarctation repaired by way of thoracotomy between February 2005 and November 2011 were evaluated. Recoarctation was defined as surgical or catheter reintervention after hospital discharge. Eighty-four infants underwent coarctation repair at median age of 12 (range 1-85) days with median follow-up of 12.3 (range 0.5-71.9) months. The seven (8 %) infants with recoarctation underwent balloon angioplasty. In multivariable analysis, only greater postoperative Doppler peak velocity [1.13, confidence interval (CI) 1.04-1.23] and greater sinotubular junction z-score (hazard ratio 4.19, CI 1.47-11.95) independently predicted coarctation. Doppler peak velocity2.12 m/s had sensitivity of 63 % and specificity of 83 % of predicting recoarctation, and ST junction z-score-0.93 had sensitivity of 100 % and specificity of 58 %. No transverse arch dimensions were independently associated with recoarctation. Infants with transverse arch z-score as low as -2.8 underwent successful repair by way of thoracotomy. No clinical predictors were significant.

Aims We sought to determine whether velocity vector imaging (VVI)-derived left ventricular (LV) myocardial deformation indices could detect subtle myocardial abnormalities in acute Kawasaki disease (KD). Methods and results The study cohort of children with KD was divided by coronary artery dilation (CAD, Z -score >2.5) and/or uncomplicated vs. treatment-resistant (persistent/recrudescent fever) cases and compared with age-matched controls. Peak systolic LV myocardial strain ( ɛ ) and strain rate (SR) were obtained using VVI on pre-treatment echocardiograms. Comparisons were made between controls and (i) the entire KD group, (ii) KD group subdivided by CAD, and (iii) KD group subdivided by treatment resistance. The KD group consisted of 32 children (66% male, 24 ± 20 months). Of these, 17 had CAD and 14 had resistant KD. The control group consisted of 22 children (55% male, 20 ± 17 months). Routine echo indices of LV systolic function were normal for both groups. Compared with controls, KD patients had lower global longitudinal ɛ (−15.29 vs. −12.94, P = 0.04) and SR (−1.12 vs. −0.87, P = 0.003). On subgroup analysis compared with controls, KD patients with CAD ( n = 17) had lower longitudinal ɛ (−15.29 vs. −11.87, P = 0.02) and SR (−1.12 vs. −0.86, P = 0.005). Subdivided by treatment resistance, compared with controls, those with resistant KD had lower longitudinal ɛ (−15.29 vs. −11.8, P = 0.01) and SR (−1.12 vs. −0.82, P = 0.003). Conclusion Despite normal LV systolic function by routine echocardiographic measurements, KD patients have reduced longitudinal LV ɛ and SR, which may be more sensitive indicators of myocardial inflammation and may provide supportive criteria to avoid delayed diagnosis of KD.

Elevated ventricular filling pressure is a marker of diastolic dysfunction and a known risk factor for failure of single-ventricle surgical palliation. Doppler echocardiography has been shown valuable in identifying patients with elevated ventricular end-diastolic pressure (VEDP) in other settings, but its utility in evaluating pediatric patients with single ventricle is unclear. The aim of this study was to compare Doppler parameters to simultaneous catheter measurements of VEDP in children with single ventricle.All consecutive patients (age18 years) with single ventricle who underwent simultaneous echocardiography and catheterization in 2009 and 2010 were included in this prospective study. Data regarding diagnosis, morphology of the "single" ventricle, prior surgeries, Doppler tissue imaging (DTI), atrioventricular valve inflow and pulmonary vein Doppler, and myocardial performance index (MPI) were collected. Ventricular Doppler echocardiography was performed from the dominant ventricle. Simultaneous Doppler and catheter measurements of systolic and diastolic function and VEDP were obtained. Correlation of continuous variables was examined using linear regression analysis. Receiver operating characteristic curves, two-sample Student's t tests, χ(2) analyses, and Fisher's exact tests were used as appropriate.A total of 32 patients (15 male; mean age, 30.2 ± 22 months) were studied (nine post-Fontan, 15 post-Glenn, and eight pre-Glenn). Mean systemic arterial saturation was 81 ± 10%, mean VEDP was 11 ± 3 mm Hg, and mean echocardiographically estimated ejection fraction was 55 ± 7%. VEDP was correlated positively with E/E' ratio (r = 0.44, P.01), pulmonary vein atrial reversal duration (r = 0.77, P.001), and E' (r = 0.49, P.01). Receiver operating characteristic curve analysis using an E/E' cutoff of 12 showed sensitivity of 90% (95% confidence interval, 54.1%-99.5%) and specificity of 75.0% (95% confidence interval, 47.4%-91.7%) for identifying VEDP10 mm Hg. Single left ventricles had higher DTI S' and E' velocities and lower E/E' ratios and MPIs compared with single right ventricles. S' velocity correlated positively with ejection fraction (r = 0.77, P.001) and negatively with single left ventricular MPI (r = -0.46, P.01).In patients with single-ventricle physiology, DTI and pulmonary vein Doppler echocardiographic parameters correlated modestly with direct measurement of VEDP and may be helpful in identifying patients with elevated filling pressures. In addition, single left ventricles had better systolic and diastolic performance than single right ventricles. DTI systolic velocities and left ventricular MPI correlated well with ventricular ejection fraction.

Bilateral patent ductus arteriosus and isolated right pulmonary artery (RPA) is a rare anomaly often interpreted as congenital absence of the RPA. Recognized early, continuity between the main pulmonary artery and distal RPA can be established and long-term sequelae avoided. We report 2 patients who underwent neonatal repair using a nonvalved pulmonary artery allograft conduit placed anterior to the aorta to establish continuity between the main pulmonary artery and RPA. Both patients continue to do well 10 and 15 years postoperatively with good growth of the distal RPA and good exercise tolerance. This approach offers good long-term palliation.

OBJECTIVE: Neonates undergoing congenital heart surgery require highly specialized, resource-intensive care. Location of care and degree of specialization can vary between and within institutions. Using a multi-institutional cohort, we sought to determine if location of admission is associated with an increase in health care costs, resource use and mortality. METHODS: We retrospectively analyzed admissions for neonates (

Standard therapy, consisting of intravenous immunoglobulin and aspirin, reduces, but does not eliminate, coronary artery aneurysms (CAAs) in patients with Kawasaki disease. Large CAAs can persist or undergo varying degrees of regression. The treatment of large CAAs using abciximab has been associated with short-term regression; however, longer term data are unavailable. We sought to obtain longer term follow-up data regarding the changes in the diameters of large CAAs in patients receiving both abciximab and standard therapy and to compare these changes to those of a similar group receiving standard therapy alone. All patients with Kawasaki disease and large CAAs (diameter5 mm or Z score10) treated from 1986 to 2007 were identified and divided into 2 groups. The abciximab group received abciximab plus standard therapy and the no-abciximab group received standard therapy alone. The maximum diameters of the proximal right and left anterior descending CAAs were obtained from echocardiograms. The Z scores were calculated for 3 points: the acute/subacute phase (8 weeks) and at 1 and 3 to 5 years of follow-up. The patients in the abciximab (n = 11) and no-abciximab (n = 7) groups were similar in age, interval to treatment, gender, and largest CAA Z score at diagnosis (19.6 +/- 6.2 vs 25.8 +/- 9.5, p = 0.11). The change in CAA Z score was similar between the 2 groups at 1 year (p = 0.99). At 3 to 5 years of follow-up, compared to baseline, the abciximab group had a greater decrease in the CAA Z score than did the no-abciximab group (-14.0 +/- 4.0 vs -8.2 +/- 5.9, p = 0.04). In conclusion, abciximab treatment might be associated with vascular remodeling in patients with large CAAs.

Echocardiography is used to measure the therapeutic effectiveness of heart failure therapy in adults and children. The purposes of this study were (1) to assess baseline echocardiographic predictors of clinical outcome, (2) to investigate changes in echocardiographic parameters, and (3) to compare these echocardiographic changes with changes in plasma levels of b-type natriuretic peptide (BNP) in a population of children with systemic ventricular dysfunction and symptomatic heart failure treated with carvedilol or placebo. All available baseline and 6-month echocardiograms from Pediatric Carvedilol Trial (PCT) participants (carvedilol n = 161; placebo n = 55) were reviewed. Systolic and diastolic sphericity index (SI; n = 110), TEI index (n = 145), and systemic ventricular dP/dt (n = 70) were measured. The PCT composite definition of clinical outcome (i.e., worsened, improved, or unchanged) was used. For all patients, baseline TEI index was a predictor of worsened outcome. Only children treated with carvedilol showed a significant decrease in systolic SI (P B 0.0001), diastolic SI (P B 0.0001), and TEI index (P = 0.02). An inverse correlation between changes in BNP and changes in dP/dt (r = -0.45, P = 0.04) was found only in the carvedilol group. In conclusion, TEI index predicted outcome in children with systemic ventricular dysfunction and heart failure. Carvedilol may have a beneficial effect on reversal of left ventricular remodeling and global ventricular function in pediatric heart failure.

SCRAM-jet engine is considered to be one of the useful system propulsion for super/hypersonic transportation vehicle and various researches were made to develop the engine. However, there are a lot of problems to be solved to develop it and one of them is the problem of supersonic mixing. In the SCRAM-jet engine combustor, main airflow is supersonic and residence time of the air is very short (about 1 ms). Hence rapid mixing of air and fuel is necessary. However, usually it is quite difficult to mix fuel with air in very short distance. Also total pressure loss occurs by flow interaction the air and fuel. Total pressure loss is not preferable because it causes the thrust loss. Therefore, supersonic mixing with very rapid mixing and lower total pressure loss ratio is highly requested. In order to develop the supersonic mixing, it is very important to understand the effect of injection angle. In present study, we investigate the effect of injection angle with circular sonic nozzle by changing the injection angle. Experimental and computational studies on supersonic mixing phenomena of two-dimensional slot injector with various injection angles were conducted. Supersonic wind tunnel was used for the experiments. The free stream Mach number is 3.8, total pressure is 1.1 MPa and total temperature is 287 K on average. As a secondary gas, helium gas was injected at sonic speed from the circular nozzle. The injection angle is 30°, 90° and 150°. Its total pressure is 0.4 MPa and total temperature is 287 K on average. The same flow field was also simulated by solving three-dimensional full Navier–Stokes equation with AUSM-DV scheme [Y. Wada, M.S. Liou, A flux splitting scheme with high-resolution and robustness for discontinuities, AIAA Paper 94–0083, 1994] for convective terms and full implicit LU-ADI factorization method [S. Obayashi, K. Matsushima, K. Fujii, K. Kuwahara, Improvements in efficiency and reliability for Navier–Stokes computations using the LU-ADI factorization algorithm, AIAA Paper 86–0338, 1986] for time integration. Central difference was used for viscous terms and k – ω two-equation turbulence model [D.C. Wilcox, Reassessment of the scale determining equation for advanced turbulent models, J. AIAA 26(11) (1988) 1299–1310; D.C. Wilcox, A two-equation turbulence model for wall-bounded and free-share flows, AIAA Paper 93–2905, 1993] was also employed. In the experiments, stream line on the wall surface was revealed by the oil flow picture and the flow field was visualized by the Shlieren photograph. The wall static pressure profile along the flow was obtained by the wall pressure measurements. Also volume fraction distribution measurements were conducted. Each one of those showed good agreement with computational ones. From the thorough investigation of the flow field by experiments and computations, various characteristics of the supersonic mixing with circular nozzle have been revealed.

Objective Pulmonary regurgitation (PR) is common after repair of congenital heart disease involving the right ventricular outflow tract. Because PR results in chronic right ventricular volume overload and associated morbidity and mortality, accurate assessment of its severity is important. The aim of this study was to compare echocardiography with the gold standard of PR quantitation by magnetic resonance imaging (MRI) in a young population with repaired congenital heart disease. Design/methods Patients with congenital heart disease who had undergone right ventricular outflow tract reconstruction and/or pulmonary valve replacement and had an MRI within 3 months of an echocardiogram formed the study group. Echocardiographic indices were compared with MRI-determined pulmonary regurgitant fraction (PRF) to determine the most accurate measurements to quantitate PR. Results Of the 69 MRI/echocardiography pairs in 64 patients, 53 data sets were complete and used in the analysis. For the prediction of MRI PRF > or =20%, PR jet width/annulus ratio > or =0.5 demonstrated excellent sensitivity (94%), specificity (100%), positive predictive value (PPV 100%), and negative predictive value (NPV 82%). For the prediction of MRI PRF > or =40%, jet width/annulus ratio > or =0.7 and diastolic flow reversal in the branch pulmonary arteries showed useful sensitivity (92%), specificity (68%), PPV (76%), and NPV (88%). Conclusion Pulmonary regurgitation jet width/annulus ratio combined with diastolic flow reversal is the most valuable echocardiographic measure for assessing PR severity after right ventricular outflow tract reconstruction or pulmonary valve replacement; however, this surrogate measure does not replace the importance of MRI evaluation.

Background The purpose of this statement is to update the recommendations by the American Heart Association (AHA) for the prevention of infective endocarditis, which were last published in 1997. Methods and Results A writing group appointed by the AHA for their expertise in prevention and treatment of infective endocarditis (IE) with liaison members representing the American Dental Association, the Infectious Diseases Society of America and the American Academy of Pediatrics. The writing group reviewed input from national and international experts on IE. The recommendations in this document reflect analyses of relevant literature regarding procedure-related bacteremia and IE; in vitro susceptibility data of the most common microorganisms, which cause IE; results of prophylactic studies in animal models of experimental endocarditis; and retrospective and prospective studies of prevention of IE. MEDLINE database searches from 1950 through 2006 were done for English language articles using the following search terms: endocarditis, infective endocarditis, prophylaxis, prevention, antibiotic, antimicrobial, pathogens, organisms, dental, gastrointestinal, genitourinary, streptococcus, enterococcus, staphylococcus, respiratory, dental surgery, pathogenesis, vaccine, immunization and bacteremia. The reference lists of the identified articles were also searched. The writing group also searched the AHA online library. The American College of Cardiology/AHA classification of recommendations and levels of evidence for practice guidelines were used. The article subsequently was reviewed by outside experts not affiliated with the writing group and by the AHA Science Advisory and Coordinating Committee. Conclusions The major changes in the updated recommendations include the following. (1) The committee concluded that only an extremely small number of cases of IE might be prevented by antibiotic prophylaxis for dental procedures even if such prophylactic therapy were 100 percent effective. (2) IE prophylaxis for dental procedures should be recommended only for patients with underlying cardiac conditions associated with the highest risk of adverse outcome from IE. (3) For patients with these underlying cardiac conditions, prophylaxis is recommended for all dental procedures that involve manipulation of gingival tissue or the periapical region of teeth or perforation of the oral mucosa. (4) Prophylaxis is not recommended based solely on an increased lifetime risk of acquisition of IE. (5) Administration of antibiotics solely to prevent endocarditis is not recommended for patients who undergo a genitourinary or gastrointestinal tract procedure. These changes are intended to define more clearly when IE prophylaxis is or is not recommended and to provide more uniform and consistent global recommendations.

To describe Sydenham chorea among children in a cohort of patients with rheumatic fever (RF).An existing database was used to identify demographic characteristics, clinical manifestations, and therapy in persons with RF identified in Salt Lake City, Utah, from 1985 through January 2002.Of 584 cases in the database, 537 (91%) were new-onset RF (median age of 10 years) and 177 (33%) had chorea. Patients with chorea were more often female (OR = 0.37, 95% CI = 0.25-0.55, P.0001) and were less likely to have carditis or arthritis. Prednisone treatment may lead to a shortened course of chorea (4.0 weeks in prednisone-treated [n = 32] vs 9.0 weeks in untreated [n = 14]; P.0001). Among 33 patients seen at a median of 10.3 years (range 6.3-14.9 years) after their initial bout of chorea, 20% reported residual tremor or mood swings. Ten of the 33 (30%) had one or more recurrences of chorea.Chorea affected one-third of the children with RF. Patients with chorea were less likely to have severe cardiac or rheumatologic complications of RF. Therapy with prednisone shortened the duration of rheumatic chorea; some reported recurrences of chorea and had minor neurologic sequelae.

Cryopreserved valved allografts are routinely oversized to account for somatic growth in children requiring right ventricle-to-pulmonary artery (RV-PA) continuity. The objective of this study is to determine the effect of oversizing on conduit longevity.We reviewed the records of all patients undergoing RV-PA cryopreserved valved allograft placement from 1988 to 2006 for diagnosis, age, allograft type, time to valved conduit explant, and indication for surgery. Conduit size at the time of insertion was compared with pulmonary valve size normalized for body surface area (z score). Multivariate Cox regression models with cluster analysis were constructed to assess risk of allograft oversizing for conduit failure. Kaplan-Meier analysis was used to obtain median freedom from explantation time.A total of 140 cryopreserved valved allografts (z score, 1.8 +/- 1.3; range, -1.5 to 4.9) were implanted in 99 patients (median age, 5.6 years). Reoperation was required in 66 (67%) of 99 patients during the study period. Modeling z scores as a dichotomous variable revealed that risk of allograft explantation increases 113% when allografts with z scores of 2.7 or higher are used compared with those with z scores of less than 2.7 (p0.01). Median adjusted freedom from explantation for this same grouping was 4.9 years versus 9.4 years. The presence of branch pulmonary artery stenosis shortens the conduit life (p0.001), whereas insertion of a pulmonary allograft may extend the conduit life (p = 0.13).Cryopreserved valved allograft oversizing (z scoreor = 2.7) in the pulmonary position results in decreased longevity in children. Presence of branch pulmonary artery stenosis is also associated with earlier conduit explantation.

Background. The purpose of this statement is to update the recommendations by the American Heart Association (AHA) for the prevention of infective endocarditis, which were last published in 1997. Methods and Results. A writing group appointed by the AHA for their expertise in prevention and treatment of infective endocarditis (IE) with liaison members representing the American Dental Association, the Infectious Diseases Society of America and the American Academy of Pediatrics. The writing group reviewed input from national and international experts on IE. The recommendations in this document reflect analyses of relevant literature regarding procedure-related bacteremia and IE; in vitro susceptibility data of the most common microorganisms, which cause IE; results of prophylactic studies in animal models of experimental endocarditis; and retrospective and prospective studies of prevention of IE. MEDLINE database searches from 1950 through 2006 were done for English language articles using the following search terms: endocarditis, infective endocarditis, prophylaxis, prevention, antibiotic, antimicrobial, pathogens, organisms, dental, gastrointestinal, genitourinary, streptococcus, enterococcus, staphylococcus, respiratory, dental surgery, pathogenesis, vaccine, immunization and bacteremia. The reference lists of the identified articles were also searched. The writing group also searched the AHA online library. The American College of Cardiology/AHA classification of recommendations and levels of evidence for practice guidelines were used. The article subsequently was reviewed by outside experts not affiliated with the writing group and by the AHA Science Advisory and Coordinating Committee. Conclusions. The major changes in the updated recommendations include the following. (1) The committee concluded that only an extremely small number of cases of IE might be prevented by antibiotic prophylaxis for dental procedures even if such prophylactic therapy were 100 percent effective. (2) IE prophylaxis for dental procedures should be recommended only for patients with underlying cardiac conditions associated with the highest risk of adverse outcome from IE. (3) For patients with these underlying cardiac conditions, prophylaxis is recommended for all dental procedures that involve manipulation of gingival tissue or the periapical region of teeth or perforation of the oral mucosa. (4) Prophylaxis is not recommended based solely on an increased lifetime risk of acquisition of IE. (5) Administration of antibiotics solely to prevent endocarditis is not recommended for patients who undergo a genitourinary or gastrointestinal tract procedure. These changes are intended to define more clearly when IE prophylaxis is or is not recommended and to provide more uniform and consistent global recommendations.

Objective Repair of aortic valve insufficiency is difficult, and durability is relatively unknown in children. This study evaluates the intermediate-term results of repair of the systemic semilunar valve, including the native aortic valve, neoaortic valve (anatomic pulmonary), and truncal valve. Methods We reviewed the records of 54 children (aged 2 days to 18 years) who underwent repair of the functional aortic valve for moderate or greater insufficiency from 1991 to 2005. Valve anatomy was tricuspid aortic in 26 patients, bicuspid aortic in 11 patients, tricuspid neoaortic in 9 patients, bicuspid neoaortic in 1 patient, and truncal valve in 7 patients. Multiple surgical techniques were used in most of the 54 patients, including leaflet plication in 17, leaflet repair in 15, commissuroplasty in 32, pericardial cusp augmentation in 8, and sinus of Valsalva reduction in 3. Results There was 1 early death and no late deaths. Actuarial freedom from reoperation was 68% at 5 years and 58% at 10 years. Freedom from aortic valve replacement was 82% at 5 years and 73% at 10 years. Duration of cardiopulmonary bypass was the most significant risk factor for reoperation with multivariate analysis. Of the 40 patients who have not undergone reoperation, 37 have had follow-up echocardiograms with the latest study (4.5 ± 4.2 years) demonstrating trace to 1+ insufficiency in 23 patients, 1 to 2+ in 12 patients, 2 to 3+ in 1 patient, and 3 to 4+ in 1 patient. Conclusion Repair of the insufficient systemic semilunar valve offers acceptable 10-year freedom from reoperation and functional results, and should be considered for most children.

Background Alzheimer's disease (AD) is an age-related neurodegenerative disorder that is characterized by a progressive loss of higher cognitive functions. The brain of an individual with AD exhibits extracellular senile plaques (SPs) of aggregated amyloid-beta peptide (Aβ) and intracellular neurofibrillary tangles (NFTs). Given the critical role of neuronal transport of both proteins and organelles, it is not surprising that perturbation of microtubule-based transport may play a major role in the pathogenesis of AD. Materials and methods We used the cDNA subtraction methodology and in vitro neural cell culture analyses to study the meaning of the brain site-specific gene expression pattern in cerebral tissue obtained from AD patients and also from control subjects at autopsy. Results We observed that cytoskeleton-associated proteins were down-regulated in AD subjects. We also noted an altered expression of the microtubule-associated protein 1B (MAP1B), the heat-shock protein (HSP)-90 (a key chaperone molecule), the tripartite motif-containing proteins (TRIM)-32/37 (an anti apoptotic enzyme with ubiquitin-protein ligase activity) and the Reticulon-3 (a modulator of the amyloid-precursor-protein (APP) cleavage) in AD brains. Additional molecular- and cell-biological studies revealed that small interfering RNA (siRNA)-mediated down-regulation of MAP1B expression leads to neuronal cell death in vitro. Conclusion Altered expression of MAP1B, HSP90, TRIM32/37 and Reticulon-3 provides new clues by which the ubiquitin-proteasome-, the protein-chaperon- and the APP-processing systems are disturbed in AD, thus, leading to neuritic amyloid plaques and neurofibrillary tangles.

Background: Monoarticular arthritis in children is most often suppurative septic arthritis (SA) of bacterial origin. We recently cared for 3 patients with monoarticular arthritis who developed carditis while receiving antibiotics for SA. Distinguishing SA from rheumatic fever (RF) is critical to avoid lifelong cardiac complications associated with RF. Methods: We compared the 3 cases of RF presenting with monoarticular arthritis with 12 cases of culture-confirmed SA to assess the clinical and laboratory differences between the 2 groups. Results: Erythrocyte sedimentation rate, C-reactive protein and mean synovial fluid white blood cell counts were elevated in both groups. Mean antistreptolysin O (ASO) and anti-DNase B titers were elevated in patients with RF. Conclusions: The clinical and laboratory features of RF and SA demonstrate substantial overlap. Patients with monoarticular arthritis and sterile synovial fluid cultures should have RF included in their differential diagnoses. Patients with an elevated ASO and/or anti-DNase B titer should have a careful cardiac examination looking for clinical evidence of carditis. Echocardiogram should be considered if clinical carditis associated with RF is suspected.

This paper discusses an accurate magnetic field analysis that addresses stress distribution in the magnetic core by using the finite-element method (FEM) to estimate motor characteristics. This analysis method consists of the following steps: 1) measurement of the magnetic characteristics of electrical steel sheets in a state where stress is added; 2) calculation of deformation and stress distribution using structural analysis; 3) preprocessing of the magnetic field analysis, which generates the FEM mesh taking account of the deformation by stress and the changes of the magnetic properties in each of the core elements corresponding to stress distribution; and 4) magnetic field analysis using the data measured in step 1) and the FEM mesh generated in step 3). As an example, a result of a permanent magnet motor's cogging torque calculation is shown, focusing on stress in the stator core resulting from shrink fitting into the frame. As a result, the cogging torque waveform was shown to differ depending on the shape and the materials of the frame.

The stress that acts on the core by installing a stator in a frame influences the performance of a motor. To reflect this influence in the equipment design, the stress dependence of the magnetic property was examined for non-oriented electrical steels, which are the core materials. This time, the stress dependence of the magnetic power loss in the range of ±225 MPa was evaluated for high-grade non-oriented electrical steel sheets of 0.5 mm thickness, and the following results were obtained. (1) The influence on the magnetic power loss is larger under compression than under tensile stress. The loss increases rapidly with an increase in the compressive stress. However, when the stress exceeds -50 MPa, the saturation tendency of the loss gradually becomes strong. (2) The influence of the stress on the magnetic power loss is different in the rolling direction and the traverse direction. (3) A greater magnetic power loss that occurs with compressive stress is mainly due to an increase in hysteresis loss, and the eddy current loss also increases slightly. It is thought that this slight increase in the eddy current loss that occurs with compressive stress depends on the change in the anomalous loss.