Lessons Learned From Newborn Screening for Critical Congenital Heart Defects.

Newborn screening for critical congenital heart defects (CCHD) was added to the US Recommended Uniform Screening Panel in 2011. Within 4 years, 46 states and the District of Columbia had adopted it into their newborn screening program, leading to CCHD screening being nearly universal in the United States. This rapid adoption occurred while there were still questions about the effectiveness of the recommended screening protocol and barriers to follow-up for infants with a positive screen.

Radiofrequency ablation versus cryoablation for atrioventricular nodal re-entrant tachycardia in children: a value comparison.

Background: It is unclear whether cryoablation or radiofrequency ablation offers better value for treating atrioventricular nodal re-entrant tachycardia in children. We aimed to compare the value of these procedures for treating atrioventricular nodal re-entrant tachycardia in children, with value being outcomes relative to costs.

Methods: We performed a retrospective cohort study of all atrioventricular nodal re-entrant tachycardia ablations for children (age⩽18 years) from July, 2009 to June, 2011 at our institution.

Limitations, depressive symptoms, and quality of life among a population-based sample of young adults with congenital heart defects.

Background: Little population-based data exist on limitations and health-related quality of life (HRQoL) in adults with congenital heart defects (CHD).

Methods: We used 2004 to 2012 Medical Expenditure Panel Survey data to identify a population-based sample of young adults ages 18 to 40 years reporting health symptoms or healthcare encounters in the previous year. Comparing adults reporting CHD to others, we examined the prevalence of cognitive, physical, and activity limitations, depressive symptoms, and physical and mental HRQoL.

Force-producing ADP state of myosin bound to actin.

Molecular motors produce force when they interact with their cellular tracks. For myosin motors, the primary force-generating state has MgADP tightly bound, whereas myosin is strongly bound to actin. We have generated an 8-Å cryoEM reconstruction of this state for myosin V and used molecular dynamics flexed fitting for model building.

Toward improved phosphorus efficiency in monogastrics-interplay of serum, minerals, bone, and immune system after divergent dietary phosphorus supply in swine.

Phosphorus (P) is of vital importance for many aspects of metabolism, including bone mineralization, blood buffering, and energy utilization. In order to identify molecular routes affecting intrinsic P utilization, we address processes covering P intake, uptake, metabolism, and excretion. In particular, the interrelation of bone tissue and immune features is of interest to approximate P intake to animal's physiology and health status.

Screening for Critical Congenital Heart Disease.

Screening for critical congenital heart disease (CCHD) was added to the United States Recommended Uniform Screening Panel in 2011. Since that time, CCHD screening with pulse oximetry has become nearly universal for newborns born in the United States. There are various algorithms in use.

Association of Digoxin With Interstage Mortality: Results From the Pediatric Heart Network Single Ventricle Reconstruction Trial Public Use Dataset.

Background: Mortality for infants with single ventricle congenital heart disease remains as high as 8% to 12% during the interstage period, the time between discharge after the Norwood procedure and before the stage II palliation. The objective of our study was to determine the association between digoxin use and interstage mortality in these infants.

Methods And Results: We conducted a retrospective cohort study using the Pediatric Heart Network Single Ventricle Reconstruction Trial public use dataset, which includes data on infants with single right ventricle congenital heart disease randomized to receive either a Blalock-Taussig shunt or right ventricle-to-pulmonary artery shunt during the Norwood procedure at 15 institutions in North America from 2005 to 2008.

Transcatheter Versus Surgical Closure of Atrial Septal Defects in Children: A Value Comparison.

Objectives: The purpose of this study was to determine whether a transcatheter procedure or surgical closure offers a better value proposition for atrial septal defect (ASD) closure.

Background: Secundum ASDs are common congenital heart defects with both transcatheter and surgical treatment options. Although both options have been shown to have excellent results in children, the relative value of the 2 procedures is unclear.

Syncope Best Practices: A Syncope Clinical Practice Guideline to Improve Quality.

Objective: To determine whether implementation of a standardized clinical practice guideline (CPG) for the evaluation of syncope would decrease practice variability and resource utilization.

Design: A retrospective review of medical records of patients presenting to our practice for outpatient evaluation of syncope before and after implementation of the CPG. The guideline included elements of history, physical exam, electrocardiogram, and "red flags" for further testing.

Survival of Children With Hypoplastic Left Heart Syndrome.

Objective: To examine the survival of infants with hypoplastic left heart syndrome (HLHS) and potential influence of demographic and clinical characteristics on survival using population-based data.

Methods: Infants with nonsyndromic HLHS (n = 212) born between 1979 and 2005 were identified through the Metropolitan Atlanta Congenital Defects Program. Vital status was ascertained through 2009 based on linkage with vital records.

The risk associated with concomitant procedures performed during adult congenital heart surgery.

Background: Adult patients with CHD often require complex operations, and indications for particular aspects of the operation are sometimes unclear. The aims of our study were as follows: to characterise concomitant procedures performed during adult congenital cardiac surgery, and to better define the risk involved with performing concomitant procedures during a single operation.

Methods: We retrospectively studied 458 adult congenital cardiac surgical patients.

Association of Interstage Home Monitoring With Mortality, Readmissions, and Weight Gain: A Multicenter Study from the National Pediatric Cardiology Quality Improvement Collaborative.

Background: Daily home monitoring of oxygen saturation and weight has been reported to improve outcomes for patients with single-ventricle heart disease during the period between stage I palliation and stage II palliation. However, these studies have been limited to single institutions and used historical control subjects. Our objective was to determine the association of various interstage home monitoring strategies with outcomes using a multicenter cohort with contemporary control subjects.

Factors associated with inpatient hospitalizations among patients aged 1 to 64 years with congenital heart defects, Arkansas 2006 to 2011.

Background: Individuals with congenital heart defects (CHDs) have high hospital resource use. We sought to identify factors associated with hospital costs and multiple hospitalizations among individuals with CHDs.

Methods: Data from the 2006 to 2011 Healthcare Cost and Utilization Project Arkansas State Inpatient Databases were linked across encrypted patient identifiers to develop a cohort of Arkansas residents aged 1 to 64 years who were hospitalized at least once with a CHD during this time period.

Methylating micronutrient supplementation during pregnancy influences foetal hepatic gene expression and IGF signalling and increases foetal weight.

Purpose: Maternal diet during pregnancy impacts foetal growth and development. In particular, dietary levels of methylating micronutrients (methionine, folate, choline, vitamins B6, and B12) interfere with the availability and allocation of methyl groups for methylation reactions, thereby influencing normal transcription. However, the currently recommended methylating micronutrient supplementation regimen is haphazard and arbitrary at best.

Population-based study of hospital costs for hospitalizations of infants, children, and adults with a congenital heart defect, Arkansas 2006 to 2011.

Background: Congenital heart defects (CHDs) are common birth defects and are associated with high hospital costs. The objectives of this study were to assess hospitalization costs, across the lifespan, of patients with CHDs in Arkansas.

Methods: Data from the 2006 to 2011 Healthcare Cost and Utilization Project Arkansas State Inpatient Databases were used.

Estimated number of infants detected and missed by critical congenital heart defect screening.

Background And Objectives: In 2011, the US Secretary of Health and Human Services recommended universal screening of newborns for critical congenital heart defects (CCHDs), yet few estimates of the number of infants with CCHDs likely to be detected through universal screening exist. Our objective was to estimate the number of infants with nonsyndromic CCHDs in the United States likely to be detected (true positives) and missed (false negatives) through universal newborn CCHD screening.

Methods: We developed a simulation model based on estimates of birth prevalence, prenatal diagnosis, late detection, and sensitivity of newborn CCHD screening through pulse oximetry to estimate the number of true-positive and false-negative nonsyndromic cases of the 7 primary and 5 secondary CCHD screening targets identified through screening.

Gene expression profiling of porcine mammary epithelial cells after challenge with Escherichia coli and Staphylococcus aureus in vitro.

Postpartum Dysgalactia Syndrome (PDS) represents a considerable health problem of postpartum sows, primarily indicated by mastitis and lactation failure. The poorly understood etiology of this multifactorial disease necessitates the use of the porcine mammary epithelial cell (PMEC) model to identify how and to what extent molecular pathogen defense mechanisms prevent bacterial infections at the first cellular barrier of the gland. PMEC were isolated from three lactating sows and challenged with heat-inactivated potential mastitis-causing pathogens Escherichia coli (E.

Is Clinic Visit Frequency Associated with Weight Gain During the Interstage Period? A Report from the Joint Council on Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative (JCCHD-NPCQIC).

Growth problems are prevalent among infants with congenital heart disease. We sought to determine whether frequency of outpatient clinic visits correlated with weight gain in patients with hypoplastic left heart syndrome or variant during the interstage period between discharge from stage I palliation and presentation for stage II palliation (SIIP). Using prospectively collected data from the JCCHD NPC-QIC database from June 2008 to July 2013, we performed a retrospective cohort study assessing the association of days between clinic visits (DBV) with the change in weight-for-age z-score (WAZ) during the interstage period.

Outpatient echocardiography in the evaluation of innocent murmurs in children: utilisation benchmarking.

Objectives: We sought to benchmark the utilisation of echocardiography in the outpatient evaluation of heart murmurs by evaluating two large paediatric cardiology centres.

Background: Although criteria exist for appropriate use of echocardiography, there are no benchmarking data demonstrating its utilisation.

Methods: We performed a retrospective cohort study of outpatients aged between 0 and 18 years at the Sibley Heart Center Cardiology and the Children's Hospital of Philadelphia Division of Cardiology, given a sole diagnosis of "innocent murmur" from 1 July, 2007 to 31 October, 2010.

Leaving Moderate Tricuspid Valve Regurgitation Alone at the Time of Pulmonary Valve Replacement: A Worthwhile Approach.

Background: Pulmonary valve disruption in patients with tetralogy of Fallot and congenital pulmonary stenosis often results in pulmonary insufficiency, right ventricular dilation, and tricuspid valve regurgitation. Management of functional tricuspid regurgitation at the time of subsequent pulmonary valve replacement remains controversial. Our aims were to (1) analyze tricuspid valve function after pulmonary valve replacement through midterm follow-up and (2) determine the benefits, if any, of concomitant tricuspid annuloplasty.

On the role of inelastic scattering in phase-plate transmission electron microscopy.

The phase contrast of Au nanoparticles on amorphous-carbon films with different thicknesses is analyzed using an electrostatic Zach phase plate in a Zeiss 912 Ω transmission electron microscope with in-column energy filter. Specifically, unfiltered and plasmon-filtered phase-plate transmission electron microscopy (PP TEM) images are compared to gain insight in the role of coherence after inelastic scattering processes. A considerable phase-contrast contribution resulting from a combined elastic-inelastic scattering process is found in plasmon-filtered PP TEM images.

Insulin Glulisine in Pregnancy - Experience from Clinical Trials and Post-marketing Surveillance.

Pregnancies complicated by gestational diabetes or pre-existing type 1 or type 2 diabetes mellitus are associated with a higher rate of adverse outcomes compared with pregnancies in the background population. These outcomes include miscarriage, pre-term delivery, pre-eclampsia, perinatal mortality and congenital malformations. Insulin glulisine (Apidra, Sanofi) is a rapid-acting insulin analogue indicated for the treatment of adults, adolescents and children 6 years or older with diabetes mellitus where treatment with insulin is required.

Syncope in the Pediatric Emergency Department - Can We Predict Cardiac Disease Based on History Alone?

Background: The American Heart Association recommends a "meticulous history" when evaluating patients with an initial episode of syncope. However, little is known about which historical features are most helpful in identifying children with undiagnosed cardiac syncope.

Objectives: Our objectives were 1) to describe the cardiac disease burden in Emergency Department (ED) syncope presentations, and 2) to identify which historical features are associated with a cardiac diagnosis.

The fight-or-flight response is associated with PBMC expression profiles related to immune defence and recovery in swine.

Defining phenotypes according to molecular features would promote the knowledge of functional traits like behaviour in both human and animal research. Beside physiological states or environmental factors, an innate predisposition of individual coping strategies was discussed, including the proactive and reactive pattern. According to backtest reactivity, animals assigned as high-resisting (proactive) and low-resisting (reactive) were immune challenged with tetanus toxoid in a time course experiment.