Complement (C1q) Binding De Novo Donor-Specific Antibodies and Cardiac-Allograft Vasculopathy in Pediatric Heart Transplant Recipients.

Background: We hypothesized C1q binding de novo donor-specific antibody (DSA) after heart transplant (HT) is a higher risk for development of coronary artery vasculopathy (CAV) in children.

Methods: A retrospective analysis of 127 pediatric HT recipients transplanted between January 2005 and December 2014 was used to determine complement (C1q)-binding de novo DSA on the outcomes of HT and the ability of the C1q assay to predict CAV development.

Results: Of 127 patients, 59 (46.

Persistent Epstein-Barr viral load in Epstein-Barr viral naïve pediatric heart transplant recipients: Risk of late-onset post-transplant lymphoproliferative disease.

Aim: To examine the risk of late-onset post-transplant lymphoproliferative disorder (PTLD) in the presence of persisting high Epstein-Barr virus (EBV) in EBV naïve pediatric heart transplant (HT) recipients.

Methods: A retrospective review of the medical records of the 145 pediatric HT recipients who had serial EBV viral load monitoring at our center was performed. We defined EBV naive patients whose EBV serology either IgM or IgG in the blood were negative at the time of HT and excluded passive transmission from mother to child in subjects less than 6 mo of age.

Prenatal diagnosis, hospital characteristics, and mortality in transposition of the great arteries.

Background: The role of prenatal diagnosis in reducing neonatal mortality from transposition of the great arteries (TGA) is controversial. Factors affected by prenatal diagnosis such as proximity at birth to a cardiac surgical center (CSC) and CSC volume are associated with mortality in congenital heart disease. The purpose of the study was to determine the associations between prenatal diagnosis, distance from birthplace to a CSC, CSC TGA volume, and neonatal mortality in patients with TGA.

Donor-specific HLA alloantibodies: Impact on cardiac allograft vasculopathy, rejection, and survival after pediatric heart transplantation.

Background: There is increasing evidence that donor-specific anti-HLA antibodies (DSA) are associated with poor outcomes after cardiac transplantation in adults, but data are limited in children. The objective of this study was to examine the development and consequences of de novo DSA in pediatric recipients of heart transplants.

Methods: We analyzed 105 pediatric patients who received heart transplants at our center from January 2002 to December 2012.

Age at referral and mortality from critical congenital heart disease.

Background And Objective: Newborn pulse oximetry screening is recommended to promote early referral of neonates with critical congenital heart disease (CCHD) and reduce mortality; however, the impact of late referral on mortality is not well defined. The purpose of this population-based study was to describe the association between timing of referral to a cardiac center and mortality in 2360 liveborn neonates with CCHD.

Methods: Neonates with CCHD born before pulse oximetry screening (1996-2007) were selected from the Texas Birth Defects Registry and linked to state birth and death records.

Late detection of critical congenital heart disease among US infants: estimation of the potential impact of proposed universal screening using pulse oximetry.

Importance: Critical congenital heart disease (CCHD) was added to the Recommended Uniform Screening Panel for Newborns in the United States in 2011. Many states have recently adopted or are considering requirements for universal CCHD screening through pulse oximetry in birth hospitals. Limited previous research is directly applicable to the question of how many US infants with CCHD might be identified through screening.

Racial/ethnic disparities in timing of death during childhood among children with congenital heart defects.

Background: Infants with congenital heart defects (CHD) have increased risk of morbidity and mortality. Little is known about racial/ethnic differences in timing of death during childhood. Our intent was to investigate racial/ethnic differences in mortality for CHDs during specific time periods in childhood.

Prenatal diagnosis, birth location, surgical center, and neonatal mortality in infants with hypoplastic left heart syndrome.

Background: Most studies have not demonstrated improved survival after prenatal diagnosis of critical congenital heart disease, including hypoplastic left heart syndrome (HLHS). However, the effect of delivery near a cardiac surgical center (CSC), the recommended action after prenatal diagnosis, on HLHS mortality has been poorly investigated.

Methods And Results: Using Texas Birth Defects Registry data, 1999 through 2007, which monitored >3.

The importance of the muscle and ventilatory blood pumps during exercise in patients without a subpulmonary ventricle (Fontan operation).

Objectives: The aim of this study was to determine the relative contribution of the muscle and ventilatory pumps to stroke volume in patients without a subpulmonic ventricle.

Background: In patients with Fontan circulation, it is unclear how venous return is augmented to increase stroke volume and cardiac output during exercise.

Methods: Cardiac output (acetylene rebreathing), heart rate (electrocardiography), oxygen uptake (Douglas bag technique), and ventilation were measured in 9 patients age 15.

Effect of acculturation and distance from cardiac center on congenital heart disease mortality.

Background And Objective: Despite improvements in congenital heart disease (CHD) survival over the past 4 decades, ethnic disparities persist. Several studies have shown higher postoperative CHD adjusted mortality in black and Hispanic children. Others noted that non-English-speaking language at home was associated with appointment noncompliance, which the parents attributed to misunderstanding and living too far from a health center.

Use of a highly sensitive assay for cardiac troponin T and N-terminal pro-brain natriuretic peptide to diagnose acute rejection in pediatric cardiac transplant recipients.

Background: Biomarkers have been proposed to augment or replace endomyocardial biopsy (EMB) to diagnose acute transplant rejection (AR). A new, highly sensitive assay for troponin T detects levels of cardiac troponin T (cTnT) 10- to 100-fold lower than standard assays but has not been investigated in transplant patients. N-terminal pro-brain natriuretic peptide (NT-proBNP) has not been evaluated in pediatric transplant patients.

Racial/Ethnic disparities in risk of early childhood mortality among children with congenital heart defects.

Background: Infants with congenital heart defects (CHDs) have increased risk of childhood morbidity and mortality. However, little is known about racial/ethnic differences in early childhood mortality.

Patients And Methods: We conducted a retrospective cohort study with data from the Texas Birth Defect Registry on 19 530 singleton, live-born infants with a CHD and born January 1, 1996, to December 31, 2003, to non-Hispanic (NH) white, NH black, and Hispanic women.

Mortality resulting from congenital heart disease among children and adults in the United States, 1999 to 2006.

Background: Previous reports suggest that mortality resulting from congenital heart disease (CHD) among infants and young children has been decreasing. There is little population-based information on CHD mortality trends and patterns among older children and adults.

Methods And Results: We used data from death certificates filed in the United States from 1999 to 2006 to calculate annual CHD mortality by age at death, race-ethnicity, and sex.

Mortality among infants with birth defects: Joint effects of size at birth, gestational age, and maternal race/ethnicity.

Background: We examined the separate and joint effects of gestational age, size at birth and maternal race/ethnicity on early childhood survival among 48,391 singleton infants with major birth defects.

Methods: Texas Birth Defects Registry data were linked to death records and the National Death Index to ascertain deaths. Gestational age categories were preterm or term birth; size at birth included small for gestational age (SGA), appropriate for gestational age (AGA), and large for gestational age (LGA).

Mortality in first 5 years in infants with functional single ventricle born in Texas, 1996 to 2003.

Background: Infants with functional single ventricle have a high risk of death during the early years of life. Studies have reported improvement in postoperative survival, but they do not include preoperative deaths or those occurring before transfer. The purpose of this population-based study was to estimate 5-year survival in infants with functional single ventricle, to define factors associated with survival, and to estimate improvement in outcome.

3-Acetyl-benzoic acid.

In the crystal structure of the title compound, C(9)H(8)O(3), essentially planar mol-ecules [the carboxyl group makes a dihedral angle of 4.53 (7)° with the plane of the ring, while the acid group forms a dihedral angle of 3.45 (8)° to the ring] aggregate by centrosymmetric hydrogen-bond pairing of ordered carboxyl groups.

Pulse oximetry screening at 4 hours of age to detect critical congenital heart defects.

Objective: The purpose of this prospective study was to assess the feasibility and reliability of pulse oximetry screening to detect critical congenital heart defects in a newborn nursery.

Methods: The study was performed in a large urban hospital with an exclusively inborn population. Stable neonates who had a gestational age of >or=35 weeks and birth weight of >or=2100 g and in whom a critical congenital heart defect was not suspected were admitted to the newborn nursery.

Infliximab treatment of intravenous immunoglobulin-resistant Kawasaki disease.

Objective: To investigate the safety, tolerability, and pharmacokinetics of the anti-tumor necrosis factor-alpha monoclonal antibody infliximab in subjects with intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD).

Study Design: We conducted a multicenter, randomized, prospective trial of second IVIG infusion (2 g/kg) versus infliximab (5 mg/kg) in 24 children with acute KD and fever after initial treatment with IVIG. Primary outcome measures were the safety, tolerability, and pharmacokinetics of infliximab.

Aspirin resistance in children with heart disease at risk for thromboembolism: prevalence and possible mechanisms.

Aspirin is used to prevent thromboembolism in children with heart disease without evidence supporting its efficacy. Studies in adults report a 5%-51% prevalence of aspirin resistance, yet the mechanisms involved are poorly understood. Our aims were to determine its prevalence in these children and to explore its possible mechanisms.

Antibodies against donor human leukocyte antigens and the outcome of cardiac allografts in adults and children.

Background: Mismatched histocompatibility antigens between donor organ and host stimulate the immune response that causes allograft rejection. Antibodies against human leukocyte antigen (HLA) are known to appear in the serum of heart transplant recipients.

Methods: We have tested stored sera with HLA bound to polystyrene microbeads in a retrospective analysis of heart recipients transplanted in our center to better understand the impact of antibodies against HLA on the posttransplant course.

Effect of proximity to hazardous waste sites on the development of congenital heart disease.

The authors sought to determine whether the risk of congenital heart disease (CHD) was greater for the children of mothers who lived close to a hazardous waste site (HWS) than for those who lived farther away. All cases (n = 1283) of confirmed CHD, and a random sample of 2,292 controls, born in Dallas County, Texas, from 1979-1984 were linked with 276 HWSs present during the study. The authors ascertained locations of households and determined the distance to the nearest HWS.

Epidemiology of noncomplex left ventricular outflow tract obstruction malformations (aortic valve stenosis, coarctation of the aorta, hypoplastic left heart syndrome) in Texas, 1999-2001.

Background: The left ventricular outflow tract (LVOT) malformations aortic valve stenosis (AVS), coarctation of the aorta (CoA), and hypoplastic left heart syndrome (HLHS) contribute significantly to infant mortality due to birth defects. Previous epidemiology data showed rate differences between male and female and white and black ethnic groups. The Texas Birth Defects Registry, an active surveillance program, enables study in a large, diverse population including Hispanics.

Trisomy 21 and congenital heart disease: effect of timing of initial echocardiogram.

Current recommendations for infants with trisomy 21 include an echocardiogram in the first month of life. The purposes of this study were to determine whether outcome and presence of transient shunting differed between infants with echocardiograms in the first month (Group I) and those performed later (Group II). Transient patent ductus arteriosus was more commonly seen in Group I.

Cardiopulmonary bypass to repair an atrial septal defect does not affect cognitive function in children.

Background: Changes in neurocognitive function after cardiopulmonary bypass (CPB) are difficult to assess in children with congenital cardiovascular malformations in part because identification of a suitable control group of children is difficult.

Methods And Results: We evaluated neurocognitive function in subjects aged 3 to 17 years both before and after surgical repair of an atrial septal defect (ASD). Eighteen subjects underwent testing within a median of 3 days (range, 1 to 27) before surgery and 5.