Fetal Characteristics and Perinatal Outcomes in Tetralogy of Fallot Without a Ductus Arteriosus.

Absence of the ductus arteriosus (DA) is common in tetralogy of Fallot (TOF), occurring in up to 30% of cases. Yet, the clinical course and fetal echocardiographic features are not well described, limiting prenatal counseling. This study examines the fetal echocardiographic characteristics and perinatal outcomes in children with TOF absent DA (TOF/ADA), comparing them to those with a DA (TOF/DA).

Clinical Care for Cardiovascular Disease in Patients With Williams-Beuren Syndrome.

Williams-Beuren syndrome (WBS) is a congenital multisystem disorder affecting the cardiovascular, central nervous, and musculoskeletal systems. Cardiovascular abnormalities, which consist principally of vascular stenoses, occur in approximately 80% of people with WBS and are the predominant cause of early morbidity and mortality. Supravalvar aortic stenosis and peripheral pulmonary artery stenosis are the most common stenotic lesions in WBS, though other stenoses often occur, including stenoses of the coronary arteries.

Impact of burnout and professional fulfillment on intent to leave among pediatric physicians: The findings of a quality improvement initiative.

Background And Objectives: Physician burnout is rampant, and physician retention is increasingly hard. It is unclear how burnout impacts intent to leave an organization. We sought to determine how physician burnout and professional fulfillment impact pediatric physicians' intent to leave (ITL) an organization.

Pericardial effusion in children admitted with juvenile idiopathic arthritis: A multicenter retrospective cohort study from the pediatric health information system.

Objectives: This study aimed to determine if the presence of a pericardial effusion is associated with adverse outcomes among children admitted with juvenile idiopathic arthritis.

Patients And Methods: The multicenter, retrospective cohort study was conducted with 4,332 patients (1,554 males, 2,778 females; median age: 12 years; IQR, 7, 15 years) using the Pediatric Health Information System. Data from hospital admissions between January 1, 2004, and September 15, 2015, were obtained for patients with an International Disease Classification, Ninth Revision code for juvenile idiopathic arthritis.

Intensive Care Unit Analgosedation After Cardiac Surgery in Children with Williams Syndrome : a Matched Case-Control Study.

Objective: Cardiovascular abnormalities are common in patients with Williams syndrome and frequently require surgical intervention necessitating analgesia and sedation in a population with a unique neuropsychiatric profile, potentially increasing the risk of adverse cardiac events during the perioperative period. Despite this risk, the overall postoperative analgosedative requirements in patients with WS in the cardiac intensive care unit have not yet been investigated. Our primary aim was to examine the analgosedative requirement in patients with WS after cardiac surgery compared to a control group.

Oxidative balance scores and neural crest cell-related congenital anomalies.

Oxidative stress and redox imbalance adversely affect embryonic development. We developed two oxidative balance scores (OBS) that include dietary and nondietary exposures. We hypothesized that higher scores (i.

Arrhythmias in Williams Syndrome.

Williams syndrome (WS) is a congenital, multisystem disorder in which 80% of patients have cardiovascular abnormalities. Sudden cardiac death occurs 25 to 100 times more often in WS than in the general population, and cardiac repolarization is abnormal in WS. We sought to determine the prevalence of primary arrhythmias in patients with WS and whether QTc prolongation impacts arrhythmia risk.

Williams Syndrome: Supravalvar Aortic, Aortic Arch, Coronary and Pulmonary Arteries: Is Comprehensive Repair Advisable and Achievable?

Williams syndrome, and various elastin protein mediated arteriopathies, presents a clinical challenge to pediatric cardiovascular specialists. In the severest phenotypes, multilevel obstruction to the systemic and pulmonic arterial systems result in biventricular dysfunction which can be imminently life-threatening. As a longstanding, quaternary referral center for complex pulmonary arteriopathies and pediatric connective tissue disease, Stanford Medicine Children's Health has developed a sizeable experience managing these patients.

Formation of Type II Silicon Clathrate with Lithium Guests through Thermal Diffusion.

At low guest atom concentrations, Si clathrates can be viewed as semiconductors, with the guest atoms acting as dopants, potentially creating alternatives to diamond Si with exciting optoelectronic and spin properties. Studying Si clathrates with different guest atoms would not only provide insights into the electronic structure of the Si clathrates but also give insights into the unique properties that each guest can bring to the Si clathrate structure. However, the synthesis of Si clathrates with guests other than Na is challenging.

Impact of liver dysfunction on outcomes in children with Alagille syndrome undergoing congenital heart surgery.

Objectives: Children with Alagille syndrome often have complex forms of congenital heart defects with the majority having peripheral pulmonary artery stenosis (PPAS) and pulmonary valve atresia (PA) or pulmonary valve stenosis. Children with Alagille syndrome also have variable amounts of liver dysfunction. The purpose of this study was to evaluate the impact of liver dysfunction on outcomes in children with Alagille syndrome undergoing congenital heart surgery.

Image-Based Stability Quantification.

Quantitative evaluation of human stability using foot pressure/force measurement hardware and motion capture (mocap) technology is expensive, time consuming, and restricted to the laboratory. We propose a novel image-based method to estimate three key components for stability computation: Center of Mass (CoM), Base of Support (BoS), and Center of Pressure (CoP). Furthermore, we quantitatively validate our image-based methods for computing two classic stability measures, CoMtoCoP and CoMtoBoS distances, against values generated directly from laboratory-based sensor output (ground truth) using a publicly available, multi-modality (mocap, foot pressure, two-view videos), ten-subject human motion dataset.

Can 10 000 Healthy Steps a Day Slow Aortic Root Dilation in Pediatric Patients With Marfan Syndrome?

Background Stiffer aortas are associated with a faster rate of aortic root (AoR) dilation and higher risk of aortic dissection in patients with Marfan syndrome. We have previously shown that mild aerobic exercise reduces aortic stiffness and rate of AoR dilation in a Marfan mouse model. In this study, we investigated if these results could be translated to pediatric patients with Marfan syndrome.

Impact of Abnormal Potassium on Arrhythmia Risk During Pediatric Digoxin Therapy.

Digoxin is used in children with heart failure and tachyarrhythmia. Its use in patients with single ventricle anatomy has increased following evidence of improved interstage survival after the Norwood procedure. Digoxin has a narrow therapeutic window and may alter serum potassium balance, inducing arrhythmias.

Assessing associations between residential proximity to greenspace and birth defects in the National Birth Defects Prevention Study.

Background: Residential proximity to greenspace is associated with various health outcomes.

Objectives: We estimated associations between maternal residential proximity to greenspace (based on an index of vegetation) and selected structural birth defects, including effect modification by neighborhood-level factors.

Methods: Data were from the National Birth Defects Prevention Study (1997-2011) and included 19,065 infants with at least one eligible birth defect (cases) and 8925 without birth defects (controls) from eight Centers throughout the United States.

Differential newborn DNA methylation among individuals with complex congenital heart defects and childhood lymphoma.

Background: There is emerging evidence that children with complex congenital heart defects (CHDs) are at increased risk for childhood lymphoma, but the mechanisms underlying this association are unclear. Thus, we sought to evaluate the role of DNA methylation patterns on "CHD-lymphoma" associations.

Methods: From >3 million live births (1988-2004) in California registry linkages, we obtained newborn dried bloodspots from eight children with CHD-lymphoma through the California BioBank.

Importance of Formula-Specific Centile Thresholds for Evaluation of Heart Rate-Corrected QT Prolongation in Williams Syndrome.

Patients with Williams syndrome (WS) have a 25- to 100-fold higher risk of sudden death and prolonged heart rate-corrected QT (QTc). A recent study using the Fridericia formula for QT correction suggested that prolongation is principally an issue of heart rate. We used multiple published heart rate correction formulas to reevaluate the prevalence of QTc prolongation in our original dataset from our 2010 study at the Children's Hospital of Philadelphia.

Surgical repair of peripheral pulmonary artery stenosis: A 2-decade experience with 145 patients.

Background: Peripheral pulmonary artery stenosis (PPAS) is a relatively rare form of congenital heart disease often associated with Williams syndrome, Alagille syndrome, and elastin arteriopathy. This disease is characterized by stenoses at nearly all lobar and segmental ostia and results in systemic-level right ventricular pressures. The current study summarizes our experience with the surgical treatment of PPAS.

The number of postoperative surgical or diagnostic procedures following congenital heart surgery correlates with both mortality and hospital length of stay.

Background: Outcomes for congenital heart disease have dramatically improved over the past several decades. However, there are patients who encounter intraoperative or postoperative complications and ultimately do not survive. It was our hypothesis that the number of postoperative procedures (including surgical and unplanned diagnostic procedures) would correlate with hospital length of stay and operative mortality.

Referring Provider Opinions of Pediatric Cardiology Evaluations Performed by Nurse Practitioners.

In the setting of physician shortages, nurse practitioner (NP) roles have evolved, with increasing independence across most healthcare settings. We sought to characterize referring clinician perceptions of NP-performed outpatient pediatric cardiology consultations. We electronically distributed to pediatric and family medicine physicians and NPs in Arkansas our 11-item survey assessing the acceptability of pediatric cardiology consultations being completed by an NP under varying circumstances.