Identification of research priorities in CHD: empowering patients and families through participation in the development of formal research agendas.

Background: Conquering CHD, formerly known as the Pediatric Congenital Heart Association (PCHA), is the leading congenital heart disease (CHD) patient advocacy organisation in the United States of America, and places high priority on patient engagement in the research process. Participatory design is an approach to problem-solving that utilises the knowledge and opinions of groups of people to generate plans and new ideas. Utilising this mode of patient engagement, patients and families engaged with Conquering CHD assisted in developing a list of research priorities which was then distributed to the larger membership with instructions to rank the priorities in order of importance.

Multisystem inflammatory syndrome in children and Kawasaki disease: a critical comparison.

Children and adolescents infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are predominantly asymptomatic or have mild symptoms compared with the more severe coronavirus disease 2019 (COVID-19) described in adults. However, SARS-CoV-2 is also associated with a widely reported but poorly understood paediatric systemic vasculitis. This multisystem inflammatory syndrome in children (MIS-C) has features that overlap with myocarditis, toxic-shock syndrome and Kawasaki disease.

Six Month Follow-up of Patients With Multi-System Inflammatory Syndrome in Children.

Background And Objectives: Myocardial dysfunction and coronary abnormalities are prominent features of multisystem inflammatory syndrome in children (MIS-C). In this study we aim to evaluate the early and midterm outcomes of MIS-C.

Methods: This is a longitudinal 6-month cohort study of all children admitted and treated for MIS-C from April 17 to June 20, 2020.

Continuous and intermittent administration of intravenous sildenafil in critically ill infants with pulmonary hypertension.

Unlabelled: Intravenous (IV) sildenafil may be administered as a continuous infusion or intermittent bolus dosing in infants with pulmonary hypertension (PH). We aimed to compare these delivery methods.

Methods: We retrospectively evaluated subjects less than 12 months old treated with IV sildenafil for PH.

Didactic education in paediatric cardiology during the COVID-19 pandemic: a national fellow survey.

The coronavirus disease 2019 (COVID-19) pandemic has had a profound impact on medical educational curricula. We aimed to examine the impact of these unprecedented changes on the formal education of paediatric cardiology fellows through a nationwide survey. A REDCap™-based voluntary anonymous survey was sent to all current paediatric cardiology fellows in the United States of America in May, 2020.

Highly Sensitive Blocker Displacement Amplification and Droplet Digital PCR Reveal Low-Level Parental FOXF1 Somatic Mosaicism in Families with Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins.

Detection of low-level somatic mosaicism [alternate allele fraction (AAF) ≤ 10%] in parents of affected individuals with the apparent de novo pathogenic variants enables more accurate estimate of recurrence risk. To date, only a few systematic analyses of low-level parental somatic mosaicism have been performed. Herein, highly sensitive blocker displacement amplification, droplet digital PCR, quantitative PCR, long-range PCR, and array comparative genomic hybridization were applied in families with alveolar capillary dysplasia with misalignment of pulmonary veins.

Comparison of Echocardiographic Diagnostic Criteria of Left Ventricular Noncompaction in a Pediatric Population.

Background: There is controversy regarding the best echocardiographic diagnostic criteria for left ventricular noncompaction (LVNC). We assessed the diagnostic utility and reproducibility of the previously proposed echocardiographic diagnostic criteria in a pediatric population using a segmental approach.

Methods: Echocardiograms were matched for patients with and without a clinical diagnosis of LVNC.

Subclinical Decrease in Myocardial Function in Asymptomatic Infants of Diabetic Mothers: A Tissue Doppler Study.

Infants of diabetic mothers (IDMs) with hypertrophic cardiomyopathy are recognized to have impaired myocardial performance, but less is known about ventricular function in IDMs without hypertrophy. We hypothesized that in asymptomatic newborns with normal two-dimensional echocardiographic evaluations, pulsed wave tissue Doppler imaging (TDI) would suggest a subclinical decrease in the cardiac function of IDMs compared to infants of non-diabetics (nIDMs). This is a retrospective cohort study of asymptomatic neonates ≥36 weeks gestation, at 0-7 days of life, with normal standard echocardiograms.

Assessing Myocardial Function in Infants with Pulmonary Hypertension: The Role of Tissue Doppler Imaging and Tricuspid Annular Plane Systolic Excursion.

Transthoracic echocardiography is the most common noninvasive method of evaluating pulmonary hypertension (PH) in infants. Identification of reliable, quantitative indices of myocardial function may enhance the diagnostic value of echocardiography in this population. We hypothesized that pulsed wave tissue Doppler imaging (TDI) and tricuspid annular plane systolic excursion (TAPSE) would be reproducible measurements and would suggest decreased ventricular function, in infants with PH.

Pathogenetics of alveolar capillary dysplasia with misalignment of pulmonary veins.

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is a lethal lung developmental disorder caused by heterozygous point mutations or genomic deletion copy-number variants (CNVs) of FOXF1 or its upstream enhancer involving fetal lung-expressed long noncoding RNA genes LINC01081 and LINC01082. Using custom-designed array comparative genomic hybridization, Sanger sequencing, whole exome sequencing (WES), and bioinformatic analyses, we studied 22 new unrelated families (20 postnatal and two prenatal) with clinically diagnosed ACDMPV. We describe novel deletion CNVs at the FOXF1 locus in 13 unrelated ACDMPV patients.

Echocardiographic Assessment of Right Atrial Pressure in a Pediatric and Young Adult Population.

Right atrial pressure (RAP) reflects right-sided cardiac hemodynamics and is useful in management of patients with cardiac and systemic disease. Studies in older adults demonstrated that inferior vena cava (IVC) diameter, IVC collapsibility index, hepatic vein systolic filling fraction (SFF), and right atrial volume (RAV) correlated with mean RAP at catheterization. This study aimed to assess the utility of echocardiographic parameters for assessment of RAP in children and young adults.

Constriction of the ductus arteriosus, severe right ventricular hypertension, and a right ventricular aneurysm in a fetus after maternal use of a topical treatment for striae gravidarum.

Fetal constriction of the ductus arteriosus is a complication of maternal non-steroidal anti-inflammatory drug use and polyphenol-rich food intake. It is unclear as to whether polyphenol-containing topical treatments have similar effects. We present a case of fetal constriction of the ductus arteriosus, severe right ventricular hypertension, and a right ventricular aneurysm associated with maternal use of a topical treatment for striae gravidarum.

Repair of Anomalous Left Coronary Artery From the Right Pulmonary Artery: A Series of Nine Cases.

Background: Repair of anomalous left coronary artery from the right pulmonary artery presents a particular technical challenge to the congenital cardiac surgeon. There is disagreement in the literature over the optimal technique for this defect, with some authors advocating for unroofing of the periaortic segment of coronary artery, while others prefer direct aortic reimplantation of the artery.

Methods: We performed a retrospective study examining outcomes of patients who were repaired for this anomaly at our institution.

Earlier arterial switch operation improves outcomes and reduces costs for neonates with transposition of the great arteries.

Objectives: This study sought to examine the impact of surgical timing on major morbidity and hospital reimbursement for late preterm and term infants with dextrotransposition of the great arteries (d-TGA).

Background: Neonatal arterial switch operation is the standard of care for d-TGA. Little is known about the effects of age at operation on clinical outcomes or costs for these neonates.