Pediatric Pulmonary Arterial Hypertension; is it Possible to Predict its Outcome?

Objectives: To assess the outcome of pediatric pulmonary arterial hypertension (PAH) and to identify the predictors of morbidity and mortality of this progressive disease.

Patients And Methods: This prospective observational cohort study was conducted on consecutive pediatric patients with PAH. Medical history was taken with a grading of the WHO functional class as well as the serum N-terminal pro-BNP (NT pro-BNP), 6 min' walk test (6MWT), and echocardiography at the initial assessment and at follow-up.

Seasons of Kawasaki Disease during the COVID-19 pandemic.

The incidence of Kawasaki Disease has a peak in the winter months with a trough in late summer/early fall. Environmental/exposure factors have been associated with a time-varying incidence. These factors were altered during the COVID-19 pandemic.

Factors Associated With Shock at Presentation in Kawasaki Disease Versus Multisystem Inflammatory Syndrome in Children Associated With Covid-19.

Background: While clinical overlap between Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) has been evident, information regarding those presenting with shock has been limited. We sought to determine associations with shock within and between diagnosis groups.

Methods: The International KD Registry enrolled contemporaneous patients with either KD or MIS-C from 39 sites in 7 countries from January 1, 2020, to January 1, 2023.

Variation in the management and treatment of children with giant coronary artery aneurysm following Kawasaki disease.

Objectives: Giant coronary artery aneurysms are rare but potentially fatal complications of Kawasaki disease. The lack of evidence-based recommendations on their management and treatment cause guidelines and practices to differ. We aimed to assess these variations.

Mechanical Mapping of the Common Carotid Artery in Healthy Individuals Aged 2 to 40 Years.

: In 2022, the World Stroke Organization said there were more than 12.2 million new cases of stroke each year, between all ages and sexes. Six and a half million people die each year from stroke.

Organ-On-A-Chip Devices: Technology Progress and Challenges.

Organ-On-a-Chip (OOC) is a multichannel 3D-microfluidic cell-culture system incorporated in a chip that simulates the behavior of an organ. This technology relies on a multidisciplinary science that benefits from and contributes in the progress of many fields including microbiology, microfluidics, biomaterials, and bioengineering. This review article summarizes the progress and achievements of various organ-on-chip technologies.

High Reimplantation of Coronary Arteries After the Switch Procedure and Associated Stenoses.

Surgical repair through the arterial switch operation (ASO) is the only definitive treatment in patients with dextro-transposition of the great arteries (d-TGA). A crucial step during the reimplantation process is transfer of coronary arteries (CA) to the neo-aorta. A potential cause of CA stenosis is the presence of a high implantation of CA (HICA), defined by the presence of coronary ostium located above the sinotubular junction (STJ) of the aorta.

Left Pulmonary Artery Stenting for Left Pulmonary Artery Stenosis Following Patent Ductus Arteriosus Device Closure: Case Series and Review of the Literature.

Percutaneous device occlusion is currently the standard of care for most cases of patent ductus arteriosus (PDA). Albeit infrequent, device-related left pulmonary artery (LPA) stenosis is a known complication of this procedure, occasionally requiring stent placement to relieve the obstruction. We present a series of four patients who required left pulmonary stenting after ductus arteriosus device closure.

Pharmacogenomics of coronary artery response to intravenous gamma globulin in kawasaki disease.

Kawasaki disease (KD) is a multisystem inflammatory illness of infants and young children that can result in acute vasculitis. The mechanism of coronary artery aneurysms (CAA) in KD despite intravenous gamma globulin (IVIG) treatment is not known. We performed a Whole Genome Sequencing (WGS) association analysis in a racially diverse cohort of KD patients treated with IVIG, both using AHA guidelines.

Interrelation of adipose tissue macrophages and fibrosis in obesity.

Obesity is characterized by adipose tissue expansion, extracellular matrix remodelling and unresolved inflammation that contribute to insulin resistance and fibrosis. Adipose tissue macrophages represent the most abundant class of immune cells in adipose tissue inflammation and could be key mediators of adipocyte dysfunction and fibrosis in obesity. Although macrophage activation states are classically defined by the M1/M2 polarization nomenclature, novel studies have revealed a more complex range of macrophage phenotypes in response to external condition or the surrounding microenvironment.

A Tale of a Trail on How It Takes 5 Days of Kawasaki Disease to Initiate Coronary Artery Injury and Change the Lives of Children.

Many articles written on Kawasaki disease explain the disease and the history of an acute inflammatory dysregulation that typically affects preschool children and does not spare older ones. Six decades have passed since the discovery of the disease in Japan, yet there are parts of the world where the disease passes unacknowledged, diagnosis is delayed, or basic treatments are not readily available. The burden of Kawasaki disease is on every health-care provider who attends to children's health.

Adherence to clinical practice guidelines for pulmonary valve intervention after tetralogy of Fallot repair: A nationwide cohort study.

Objectives: To determine guideline adherence pertaining to pulmonary valve replacement (PVR) referral after tetralogy of Fallot (TOF) repair.

Methods: Children and adults with cardiovascular magnetic resonance imaging scans and at least moderate pulmonary regurgitation were prospectively enrolled in the Comprehensive Outcomes Registry Late After TOF Repair (CORRELATE). Individuals with previous PVR were excluded.

Resource use and disease severity of children hospitalized for COVID-19 versus multisystem inflammatory syndrome in children (MIS-C) in Canada.

Background: Direct comparisons of paediatric hospitalizations for acute coronavirus disease 2019 (COVID-19) and multisystem inflammatory syndrome in children (MIS-C) can inform health system planning. We describe the absolute and relative hospital burden of acute paediatric COVID-19 and MIS-C in Canada.

Methods: This national prospective study was conducted via the Canadian Paediatric Surveillance Program from March 2020-May 2021.

Pharmacogenomics of intravenous immunoglobulin response in Kawasaki disease.

Introduction: Kawasaki disease (KD) is a diffuse vasculitis in children. Response to high dose intravenous gamma globulin (IVIG), the primary treatment, varies according to genetic background. We sought to identify genetic loci, which associate with treatment response using whole genome sequencing (WGS).

Cardiac Biomarkers Aid in Differentiation of Kawasaki Disease from Multisystem Inflammatory Syndrome in Children Associated with COVID-19.

Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19 show clinical overlap and both lack definitive diagnostic testing, making differentiation challenging. We sought to determine how cardiac biomarkers might differentiate KD from MIS-C. The International Kawasaki Disease Registry enrolled contemporaneous KD and MIS-C pediatric patients from 42 sites from January 2020 through June 2022.

Obesity and Outcomes of Kawasaki Disease and COVID-19-Related Multisystem Inflammatory Syndrome in Children.

Importance: Obesity may affect the clinical course of Kawasaki disease (KD) in children and multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19.

Objective: To compare the prevalence of obesity and associations with clinical outcomes in patients with KD or MIS-C.

Design, Setting, And Participants: In this cohort study, analysis of International Kawasaki Disease Registry (IKDR) data on contemporaneous patients was conducted between January 1, 2020, and July 31, 2022 (42 sites, 8 countries).