The pediatric heart network's study on long-term outcomes of children with HLHS and the impact of Norwood Shunt type in the single ventricle reconstruction trial cohort (SVRIII): Design and adaptations.

Background: The Single Ventricle Reconstruction (SVR) Trial was the first randomized clinical trial of a surgical approach for treatment of congenital heart disease. Infants with hypoplastic left heart syndrome (HLHS) and other single right ventricle (RV) anomalies were randomized to a modified Blalock Taussig Thomas shunt (mBTTS) or a right-ventricular-to-pulmonary-artery shunt (RVPAS) at the time of the Norwood procedure. The aim of the Long-term Outcomes of Children with HLHS and the Impact of Norwood Shunt Type (SVR III) study is to compare early adolescent outcomes including measures of cardiac function, transplant-free survival, and neurodevelopment, between those who received a mBTTS and those who received an RVPAS.

Analysis of clinical features of neonates with congenital heart disease who develop necrotizing enterocolitis: a retrospective case-control study.

Background: Infants with congenital heart disease (CHD) are known to have higher rates of necrotizing enterocolitis (NEC). Although the etiology is recognized as distinct from the premature neonatal population, there is not a universal consensus regarding etiology or specific risk factors. To analyze the clinical features of neonates with CHD who develop NEC.

Trial of Therapy on Trial: Inconsistent Thresholds for Discussing Withdrawal of Life-Sustaining Therapies in the Neonatal Intensive Care Unit.

Objective: This study aimed to establish the degree of variability in thresholds for discussing withdrawal of life-sustaining therapies (WLST) in periviable infants among neonatal intensive care unit (NICU) personnel.

Study Design: A vignette-style survey was administered to NICU personnel at two urban NICUs assessing likelihood of discussing WLST or support for discussing WLST (on a scale from 1, not at all likely/supportive to 10, extremely likely/supportive) in 10 clinical scenarios.

Results: Response rates ranged by clinical role from 26 to 89%.

Contemporary Multicenter Outcomes for Truncus Arteriosus With Interrupted Aortic Arch.

Background: Truncus arteriosus with interrupted aortic arch (TA-IAA) is a rare congenital heart defect with historically poor outcomes. Contemporary multicenter data are limited.

Methods: A retrospective cohort study of children who underwent repair of TA-IAA between 2009 and 2016 at 12 tertiary care referral centers within the United States was performed.

Impact of tight glycemic control and hypoglycemia after pediatric cardiac surgery on neurodevelopmental outcomes at three years of age: Findings from a randomized clinical trial.

Background: Studies examining the impact of randomization As per standard instruction, city is required for affiliations; however, this information is missing in affiliation 6. Please check if the provided city is correct and amend if necessary. to tight glycemic control (TGC) and resultant hypoglycemia on later neurodevelopmental outcomes have produced mixed results.

Risk calculator for advanced neonatal resuscitation.

In order to predict which newborns will require advanced resuscitation (ANR), we developed an ANR risk calculator (calculator) using a bootstrap sample size of 52 973 from a case-control study of newborns ≥34 weeks gestation. Multivariable logistic regression coefficients were obtained for the 10 original risk factors and two interaction terms. The area under the receiving-operating characteristic curve predicting ANR was 0.

Validating the Simplified Endoscopic Mucosal Assessment for Crohn's Disease: A Novel Method for Assessing Disease Activity.

Background: To demonstrate treatment efficacy in Crohn's disease (CD), regulatory authorities require that trials include an endoscopic remission/response end point; however, standardized endoscopic assessment of disease activity, such as the Simple Endoscopic Score for Crohn's Disease (SES-CD), is not typically recorded by clinicians in practice or outside of clinical trials. The novel Simplified Endoscopic Mucosal Assessment for Crohn's Disease (SEMA-CD) was developed to be easy to use in routine clinical practice and as a trial end point. We conducted a study to assess and validate the reliability and feasibility of SEMA-CD as a measure of endoscopic disease activity.

Outcomes for patients with infantile idiopathic scoliosis by casting table type.

Purpose: Serial casting is an effective treatment for infantile idiopathic scoliosis. The most common casting table types are Mehta, Risser, and spica tables. We compared major curve correction between patients with infantile idiopathic scoliosis treated using pediatric hip spica tables versus Risser or Mehta tables.

Nelarabine, etoposide, and cyclophosphamide in relapsed pediatric T-acute lymphoblastic leukemia and T-lymphoblastic lymphoma (study T2008-002 NECTAR).

Children with relapse of T-cell acute lymphoblastic leukemia (T-ALL) or lymphoblastic lymphoma (T-LBL) have a dismal prognosis, largely due to difficulty attaining second remission. We hypothesized that adding etoposide and cyclophosphamide to the nucleoside analog nelarabine could improve response rates over single-agent nelarabine for relapsed T-ALL and T-LBL. This phase I dose-escalation trial's primary objective was to evaluate the dose and safety of nelarabine given in combination with etoposide at 100 mg/m /day and cyclophosphamide at 330-400 mg/m /day, each for 5 consecutive days in children with either T-ALL (13 patients) or T-LBL (10 patients).

Supraglottic Airways Compared With Face Masks for Neonatal Resuscitation: A Systematic Review.

Background And Objectives: Positive pressure ventilation (PPV) is the most important component of neonatal resuscitation, but face mask ventilation can be difficult. Compare supraglottic airway devices (SA) with face masks for term and late preterm infants receiving PPV immediately after birth.

Methods: Data sources include Medline, Embase, Cochrane Databases, Database of Abstracts of Reviews of Effects, and Cumulative Index to Nursing and Allied Health Literature.

Variation in Case-Mix Across Hospitals: Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

Background: The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database was queried to document variation of patient characteristics, procedure types, and programmatic case-mix.

Methods: All index cardiac operations in patients less than 18 years of age in the STS Congenital Heart Surgery Database (July 2016 to June 2020) were eligible for inclusion except patients weighing ≤2.5 kg undergoing isolated patent ductus arteriosus closure.

Disparities in the Management of Pediatric Breast Masses.

Introduction: Disparities in surgical management have been documented across a range of disease processes. The objective of this study was to investigate sociodemographic disparities in young females undergoing excision of a breast mass.

Methods: A retrospective study of females aged 10-21 y who underwent surgery for a breast lesion across eleven pediatric hospitals from 2011 to 2016 was performed.

Development and implementation of a paediatric cardiac intensive care advanced practice provider curriculum.

Background: Education of paediatric advanced practice providers takes a generalist approach which lacks in-depth exposure to subspecialties like paediatric cardiac intensive care. This translates into a knowledge gap related to congenital cardiac physiology and management for APPs transitioning to the paediatric cardiac ICU.

Methods: A specialised interprofessional peer-reviewed curriculum was created and distributed through the Pediatric Cardiac Intensive Care Society.

Image-based prenatal predictors of postnatal survival, extracorporeal life support, and defect size in right congenital diaphragmatic hernia.

Objective: To determine the association between prenatal ultrasound (US) and magnetic resonance imaging (MRI) characteristics in right congenital diaphragmatic hernia (RCDH) with postnatal outcome.

Study Design: CDH Study Group data were reviewed for all RCDH infants (n = 156) born between 2015 and 2019. Prenatal US and MRI lung size measurements were correlated with survival, extracorporeal life support (ECLS), and defect size.

Newborn tandem mass spectroscopy screening for adenosine deaminase deficiency.

Background: Newborn screening (NBS) by means of T cell receptor excision circles (TREC) is now universal in the United States, Puerto Rico, and the Navajo Nation as a strategy to identify severe combined immunodeficiency (SCID) in newborns. Owing to the characteristics of adenosine deaminase (ADA) deficiency, a small but important number of cases can be missed by this screening.

Objective: To evaluate the results of the first year statewide NBS for ADA by means of dried blood spot NBS.

Adapting user-centered design principles to improve communication of peer parent narratives on pediatric tracheostomy.

Background: Parents who have to make tracheostomy decisions for their critically ill child may face forecasting errors and wish to learn from peer parents. We sought to develop an intervention with peer parent narratives to help parents anticipate and prepare for future challenges before making a decision.

Methods: To ensure that the intervention reflects parents' needs (rather than experts' opinions), we adapted a user-centered design (UCD) process to identify decision-critical information and refine the presentation format by interviewing parents who had tracheostomy decision making experience.

SCMR expert consensus statement for cardiovascular magnetic resonance of acquired and non-structural pediatric heart disease.

Cardiovascular magnetic resonance (CMR) is widely used for diagnostic imaging in the pediatric population. In addition to structural congenital heart disease (CHD), for which published guidelines are available, CMR is also performed for non-structural pediatric heart disease, for which guidelines are not available. This article provides guidelines for the performance and reporting of CMR in the pediatric population for non-structural ("non-congenital") heart disease, including cardiomyopathies, myocarditis, Kawasaki disease and systemic vasculitides, cardiac tumors, pericardial disease, pulmonary hypertension, heart transplant, and aortopathies.

Norwood Operation: Immediate vs Delayed Sternal Closure.

Background: The Norwood operation is a complex neonatal surgery. There are limited data to inform the timing of sternal closure. After the Norwood operation, delayed sternal closure (DSC) is frequent.

Toward an ontology of collaborative learning healthcare systems.

Objective: To establish a basis for a domain ontology - a formal, explicit specification of a shared conceptualization - of collaborative learning healthcare systems (CLHSs) in order to facilitate measurement, explanation, and improvement.

Methods: We adapted the "Methontology" approach to begin building an ontology of CLHSs. We specified the purpose of an ontology, acquired domain knowledge via literature review, conceptualized a common framework of CLHSs using a grounded approach, refined these concepts based on expert panel input, and illustrated concept application via four cases.