2017 ACC/AAP/AHA Health Policy Statement on Opportunities and Challenges in Pediatric Drug Development: Learning From Sildenafil.

The STARTS-1 and -2 trials (Sildenafil in Treatment-Naive Children, Aged 1 to 17 Years, With Pulmonary Arterial Hypertension) and subsequent 2012 U.S. Food and Drug Administration (FDA) product labeling for sildenafil use in pediatric patients with pulmonary hypertension highlight many of the challenges to the development and approval of medications for children.

2017 ACC/AAP/AHA Health Policy Statement on Opportunities and Challenges in Pediatric Drug Development: Learning From Sildenafil.

The STARTS-1 and -2 trials (Sildenafil in Treatment-Naive Children, Aged 1 to 17 Years, With Pulmonary Arterial Hypertension) and subsequent 2012 U.S. Food and Drug Administration (FDA) product labeling for sildenafil use in pediatric patients with pulmonary hypertension highlight many of the challenges to the development and approval of medications for children.

Incremental reduction in the incidence of stroke in children supported with the Berlin EXCOR ventricular assist device.

Background: Cerebrovascular events (CVEs) are common among children supported with the Berlin EXCOR (Berlin Heart GmbH, Berlin, Germany) ventricular assist device (VAD). Given the high incidence of CVEs associated with this device, we sought to describe our institutional experience in incrementally reducing CVEs in children supported with the Berlin EXCOR VAD.

Methods: We collected pertinent data on 39 consecutive patients who underwent Berlin EXCOR VAD implantation at a single center.

Parvovirus B19 myocarditis causes significant morbidity and mortality in children.

Although parvovirus B19 (PVB19) currently is the most common cause of viral myocarditis, limited pediatric data exist. Whereas other viruses infect cardiomyocytes, PVB19 targets coronary endothelium, leading to myocardial ischemia and dysfunction. A retrospective review investigated patients with polymerase chain reaction (PCR)-verified PVB19 myocarditis at Texas Children's Hospital and Arkansas Children's Hospital (January 2005 to August 2008).

Rapid reduction in donor-specific anti-human leukocyte antigen antibodies and reversal of antibody-mediated rejection with bortezomib in pediatric heart transplant patients.

Background: High titer donor-specific antibodies (DSA) and positive crossmatch in cardiac transplant recipients is associated with increased mortality from antibody-mediated rejection (AMR). Although treatment to reduce anti-human leukocyte antigen antibodies using plasmapheresis, intravenous immunoglobulin, and rituximab has been reported to be beneficial, in practice these are often ineffective. Moreover, these interventions do not affect the mature antibody producing plasma cell.

The technology path to deep greenhouse gas emissions cuts by 2050: the pivotal role of electricity.

Several states and countries have adopted targets for deep reductions in greenhouse gas emissions by 2050, but there has been little physically realistic modeling of the energy and economic transformations required. We analyzed the infrastructure and technology path required to meet California's goal of an 80% reduction below 1990 levels, using detailed modeling of infrastructure stocks, resource constraints, and electricity system operability. We found that technically feasible levels of energy efficiency and decarbonized energy supply alone are not sufficient; widespread electrification of transportation and other sectors is required.

Extracorporeal membrane oxygenation support for intractable primary arrhythmias and complete congenital heart block in newborns and infants: short-term and medium-term outcomes.

Objectives: To describe the experience with extracorporeal membrane oxygenation support for intractable primary arrhythmias in newborns and infants.

Design: Retrospective study.

Setting: A tertiary care pediatric hospital.

The impact of heart failure severity at time of listing for cardiac transplantation on survival in pediatric cardiomyopathy.

Background: The survival benefit of heart transplantation in adult heart failure is greatest for the sickest patients and negligible for patients not requiring inotropic or mechanical support. We hypothesized a similar survival benefit of heart transplantation for childhood cardiomyopathies with heart failure.

Methods: A merged data set of children registered in both the Pediatric Cardiomyopathy Registry and the Pediatric Heart Transplant Study was used to assess differences in mortality before and after transplant in patients with different levels of heart failure severity.

Cardiopulmonary bypass flow rate: a risk factor for hyperlactatemia after surgical repair of secundum atrial septal defect in children.

Objective: Early postoperative hyperlactatemia is seen in some children after surgical repair of secundum atrial septal defect despite apparently normal cardiac output. The objective of the study was to investigate the intraoperative risk factors for hyperlactatemia in patients undergoing atrial septal defect repair.

Methods And Results: A retrospective review of 68 consecutive patients who underwent isolated atrial septal defect repair at Arkansas Children's Hospital between January 2001 and March 2006 was performed.

The effect of age, diagnosis, and previous surgery in children and adults undergoing heart transplantation for congenital heart disease.

Objectives: We sought to evaluate the outcomes and identify risk factors for mortality after heart transplantation (HT) for congenital heart disease (CHD) in infants, children, and adults.

Background: CHD is considered a risk factor for mortality after HT, yet this unique group of patients represents a spectrum of complexity.

Methods: There were 488 patients transplanted for CHD from the combined Pediatric Heart Transplant Study (1993 to 2002, n = 367) and the Cardiac Transplant Registry Database (1990 to 2002, n = 121) who were analyzed.

National-level infrastructure and economic effects of switchgrass cofiring with coal in existing power plants for carbon mitigation.

We update a previously presented Linear Programming (LP) methodology for estimating state level costs for reducing CO2 emissions from existing coal-fired power plants by cofiring switchgrass, a biomass energy crop, and coal. This paper presents national level results of applying the methodology to the entire portion of the United States in which switchgrass could be grown without irrigation. We present incremental switchgrass and coal cofiring carbon cost of mitigation curves along with a presentation of regionally specific cofiring economics and policy issues.

Lessons learned from the pediatric heart transplant study.

The Pediatric Heart Transplant Study (PHTS) group was founded in 1991 as a voluntary, collaborative effort dedicated to the advancement of the science and treatment of children following listing for heart transplantation. Since 1993, the PHTS has collected data in an international, prospective, event-driven database that examines risk factors for outcome events following listing for transplantation. The events include transplantation, death, rejection, infection, malignancy, graft vasculopathy, and retransplantation.

State-level infrastructure and economic effects of switchgrass cofiring with coal in existing power plants for carbon mitigation.

This paper presents a linear programming (LP) methodology for estimating the cost of reducing a state's coal-fired power plant carbon dioxide emissions by cofiring switchgrass and coal. LP modeling allows interplay between regionally specific switchgrass production forecasts, coal plant locations, and individual coal plant historic performance data to determine an allocation of switchgrass minimizing cost or maximizing carbon reduction. The LP methodology is applied to two states, Pennsylvania (PA) and Iowa (IA), and results are presented with a discussion of modeling assumptions, techniques, and carbon mitigation policy implications.

Determinants of renal function in pediatric heart transplant recipients: long-term follow-up study.

Background: Renal insufficiency (RI) is a known complication in heart transplant recipients. We sought to determine the prevalence and risk factors for RI in pediatric heart transplant recipients over a long-term follow-up period.

Methods: The study cohort included 77 pediatric heart transplant recipients (35 girls, 18 African Americans) who had a minimum follow-up of 1 year.

Ruptured tricuspid valve papillary muscle: a treatable cause of neonatal cyanosis.

Severe tricuspid regurgitation resulting from a flail leaflet is a rare cause of neonatal cyanosis. We report two neonates with profound cyanosis and severe tricuspid regurgitation caused by rupture of the papillary muscle supporting the anterior leaflet, without other structural heart defects. Ductal patency could not be established.

Noninvasive cerebral oximeter as a surrogate for mixed venous saturation in children.

We evaluated the relationship between regional cerebral oxygen saturation (rSO(2)) measured by near-infrared spectroscopy (NIRS) cerebral oximeter with superior vena cava (SVC), inferior vena cava (IVC), right atrium (RA), and pulmonary artery (PA) saturation measured on room air and 100% inspired oxygen administered via a non-rebreather mask (NRB) in children. Twenty nine pediatric post-orthotopic heart transplant patients undergoing an annual myocardial biopsy were studied. We found a statistically significant correlation between rSO(2) and SVC saturations at room air and 100% inspired oxygen concentration via NRB (r = 0.

Report of the Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease.

A member of the Tennessee state legislature recently proposed a bill that would mandate all newborn infants to undergo pulse oximetry screening for the purpose of identifying those with critical structural heart disease before discharge home. The Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Defects was convened on September 29, 2005. This group reviewed the current medical literature on this topic, as well as data obtained from the Tennessee Department of Health, and debated the merits and potential detriments of a statewide screening program.

Modeling switchgrass derived cellulosic ethanol distribution in the United States.

Discussions of alternative fuel and propulsion technologies for transportation often overlook the infrastructure required to make these options practical and cost-effective. We estimate ethanol production facility locations and use a linear optimization model to consider the economic costs of distributing various ethanol fuel blends to all metropolitan areas in the United States. Fuel options include corn-based E5 (5% ethanol, 95% gasoline) to E16 from corn and switchgrass, as short-term substitutes for petroleum-based fuel.

The first successful DeBakey VAD child implantation as a bridge to transplant.

A 14-year-old boy with repaired transposition of the great arteries and ventricular septal defect presented with atrial flutter and severe congestive heart failure. Despite successful cardioversion and optimal medical therapy, the patient deteriorated and was supported with extracorporeal membrane oxygenation (ECMO). Two days after initiating ECMO support, we implanted the DeBakey VAD Child ventricular assist device (MicroMed Technology, Inc.

The impact and outcome of transplant coronary artery disease in a pediatric population: a 9-year multi-institutional study.

Background: Transplant coronary artery disease (TCAD) limits survival in heart transplant recipients; however, its incidence in children is unknown. The purpose of this study was to determine the angiographic incidence of TCAD, potential risk factors, and outcomes in a large pediatric cohort.

Methods: From January 1993 to December 1, a total of 1,222 children, aged newborn to 17 years, underwent primary cardiac transplantation at 20 institutions.

Angiographic and hemodynamic predictors for successful outcome of transcatheter occlusion of patent ductus arteriosus in infants less than 8 kilograms.

Transcatheter occlusion of patent ductus arteriosus (PDA) using Gianturco coils (GCs) has been performed for the past decade. However, little has been written regarding anatomical and hemodynamic predictors for successful occlusion of the PDA in infants. This report is to evaluate the outcome of transcatheter occlusion of PDA in symptomatic infants less than 8 kg and to assess predictors of successful occlusion.

Pediatric arteriovenous extracorporeal membrane oxygenation (ECMO) as a bridge to cardiac transplantation.

Background: Since 1990, extracorporeal membrane oxygenation (ECMO) has been used as a bridge to cardiac transplantation in 47 patients.

Methods: A review of the ECMO database, approved by the Arkansas Children's Hospital institutional review board, forms the basis of this report. We made statistical comparison using Fisher's exact probability testing.