Interventional Strategies for Children with Progressive Pulmonary Hypertension Despite Optimal Therapy. An Official American Thoracic Society Clinical Practice Guideline.

Background: Pulmonary hypertension in children often progresses despite optimal therapy. This document provides an evidence-based clinical practice guideline for the management of children with progressive pulmonary hypertension despite optimal therapy.

Methods: A multidisciplinary panel identified pertinent questions regarding the management of children with pulmonary hypertension that has progressed despite optimal therapy, conducted systematic reviews of the relevant literature, and applied the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) approach to develop clinical recommendations.

Improvement in Echocardiographic and Diagnostic Biomarkers after Systemic Glucocorticoid Therapy in Infants with Pulmonary Hypertension.

Objective: To assess the effect of treating pulmonary hypertension (PH) in infants younger than 1 year of age with systemic glucocorticoids while using echocardiographic and diagnostic biomarkers as measures of efficacy.

Study Design: A retrospective chart review was performed on 17 hospitalized infants younger than 1 year of age at St Louis Children's Hospital who received a 5- to 7-day course of systemic glucocorticoid treatment followed by a 3-week taper with no significant intracardiac shunts from January 1, 2017, to December 31, 2021. Quantitative echocardiographic indices for PH, N-terminal pro b-type natriuretic peptide, and/or b-type natriuretic peptide levels were collected before glucocorticoid treatment, after the glucocorticoid burst, and after the 21-day taper.

Biobased and compostable trilayer thermoplastic films based on poly(3-hydroxybutyrate-co-3-hydroxyvalerate) (PHBV) and thermoplastic starch (TPS).

Food preservation is crucial in safeguarding the global food supply and security. Current regulations do not encourage the use of chemical food preservatives. Therefore, creating a physical barrier in the form of packaging remains a necessary measure to prevent food contact with biological and physical contaminants.

Monitoring and evaluation of the surgical Potts shunt physiology using 4-dimensional flow magnetic resonance imaging.

Objective: The reversed Potts shunt is an increasingly applied mode of surgical palliation of severe pulmonary hypertension (PH). However, the long-term flow hemodynamic effect of the Potts shunt physiology and desirable long-term hemodynamic end points are not defined. The purpose of this descriptive study was to analyze a series of pediatric patients who underwent surgical Potts shunt as a part of end-stage PH palliation using 4-dimensional (4D)-flow magnetic resonance imaging (MRI) to (1) quantitate the flow through the anastomosis, (2) correlate the shunting pattern with phases of cardiac cycle and PH comorbidities, and (3) describe chronologic changes in shunting pattern.

Midterm outcomes of the Potts shunt for pediatric pulmonary hypertension, with comparison to lung transplant.

Objective: For children with severe pulmonary hypertension, addition of Potts shunt to a comprehensive palliation strategy might improve the outcomes afforded by medications and delay lung transplantation.

Methods: A prospective analysis was conducted of all children undergoing Potts shunt (first performed in 2013) or bilateral lung transplant for pulmonary hypertension from 1995 to present.

Results: A total of 23 children underwent Potts shunt (20 surgical, 3 transcatheter), and 31 children underwent lung transplant.

Rapid development of pulmonary hypertension during treatment of paediatric cancer.

Paediatric pulmonary hypertension has been described as a secondary complication of multiple diseases and their treatment. Limited information exists about the relationship between pulmonary hypertension and cancer in children. A review of charts was performed in all patients treated for cancer and developed pulmonary hypertension.

Potts Shunt Improves Right Ventricular Function and Coupling With Pulmonary Circulation in Children With Suprasystemic Pulmonary Arterial Hypertension.

Background: Pulmonary arterial hypertension is a progressive disease with poor outcomes in children. Right ventricular (RV) function is the most important determinant of the prognosis. Novel application of Potts shunt between left pulmonary artery and descending aorta has been introduced in an attempt to improve the survival.

Pulmonary arteriovenous malformations in children with hereditary hemorrhagic telangiectasia: a longitudinal study.

Pulmonary arteriovenous malformations (PAVMs) often occur in children with hereditary hemorrhagic telangiectasia (HHT). A 14-year longitudinal study of PAVMs in children with HHT was undertaken to assess the prevalence, the clinical impact, and progression of these malformations. This was a retrospective, single-center study from May 2002 to December 2016 of 129 children with HHT diagnosed using Curacao criteria and/or confirmed by genetic testing.

Treatment of Pediatric Pulmonary Hypertension.

Pulmonary hypertension (PH) is an increasingly recognized problem in children, particularly within tertiary pediatric hospitals. This increase is, in large part, due to ever improving survival among previously fatal conditions, such as extreme prematurity and complicated congenital heart disease. This increased recognition has paralleled burgeoning pharmacologic and interventional PH-specific treatment options.

Infants with Atypical Presentations of Alveolar Capillary Dysplasia with Misalignment of the Pulmonary Veins Who Underwent Bilateral Lung Transplantation.

Objective: To describe disease course, histopathology, and outcomes for infants with atypical presentations of alveolar capillary dysplasia with misalignment of the pulmonary veins (ACDMPV) who underwent bilateral lung transplantation.

Study Design: We reviewed clinical history, diagnostic studies, explant histology, genetic sequence results, and post-transplant course for 6 infants with atypical ACDMPV who underwent bilateral lung transplantation at St. Louis Children's Hospital.

Isolated Major Aortopulmonary Collateral as the Sole Pulmonary Blood Supply to an Entire Lung Segment.

Congenital systemic-to-pulmonary collateral arteries or major aortopulmonary collaterals are associated with cyanotic congenital heart disease with decreased pulmonary blood flow. Though it is usually associated with congenital heart diseases, there is an increased incidence of isolated acquired aortopulmonary collaterals in premature infants with chronic lung disease. Interestingly, isolated congenital aortopulmonary collaterals can occur without any lung disease, which may cause congestive heart failure and require closure.

Pulmonary Artery Acceleration Time Provides a Reliable Estimate of Invasive Pulmonary Hemodynamics in Children.

Background: Pulmonary artery acceleration time (PAAT) is a noninvasive method to assess pulmonary hemodynamics, but it lacks validity in children. The aim of this study was to evaluate the accuracy of Doppler echocardiography-derived PAAT in predicting right heart catheterization (RHC)-derived pulmonary artery pressure (PAP), pulmonary vascular resistance (PVR), and compliance in children.

Methods: Prospectively acquired and retrospectively measured Doppler echocardiography-derived PAAT and RHC-derived systolic PAP, mean PAP (mPAP), indexed PVR (PVRi), and compliance were compared using regression analysis in a derivation cohort of 75 children (median age, 5.

Recommendations for utilization of the paracorporeal lung assist device in neonates and young children with pulmonary hypertension.

The management of decompensating critically ill children with severe PH is extremely challenging and requires a multidisciplinary approach. Unfortunately, even with optimal care, these children might continue to deteriorate and develop inadequate systemic perfusion and at times cardiac arrest secondary to a pulmonary hypertensive crisis. Tools to support these children are limited, and at times, the team should proceed with offering extracorporeal support, especially in newly diagnosed patients who have not benefitted from medical therapy prior to their acute deterioration, in patients with severe pulmonary venous disease and in patients with alveolar capillary dysplasia.

Α-Dystrobrevin-1 recruits Grb2 and α-catulin to organize neurotransmitter receptors at the neuromuscular junction.

Neuromuscular junctions (NMJs), the synapses made by motor neurons on muscle fibers, form during embryonic development but undergo substantial remodeling postnatally. Several lines of evidence suggest that α-dystrobrevin, a component of the dystrophin-associated glycoprotein complex (DGC), is a crucial regulator of the remodeling process and that tyrosine phosphorylation of one isoform, α-dystrobrevin-1, is required for its function at synapses. We identified a functionally important phosphorylation site on α-dystrobrevin-1, generated phosphorylation-specific antibodies to it and used them to demonstrate dramatic increases in phosphorylation during the remodeling period, as well as in nerve-dependent regulation in adults.

Potts Shunt and Pediatric Pulmonary Hypertension: What We Have Learned.

Background: A Potts shunt has been proposed as effective palliative therapy in children with severe pulmonary hypertension (PH) who have suprasystemic right ventricular pressures.

Methods: A retrospective single-center study was performed to assess outcomes in 5 children who underwent a Potts shunt for severe PH.

Results: All 5 children were in World Health Organization functional class IV.

Two deletions overlapping a distant FOXF1 enhancer unravel the role of lncRNA LINC01081 in etiology of alveolar capillary dysplasia with misalignment of pulmonary veins.

Position effects due to disruption of distant cis-regulatory regions have been reported for over 40 human gene loci; however, the underlying mechanisms of long-range gene regulation remain largely unknown. We report on two patients with alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) caused by overlapping genomic deletions that included a distant FOXF1 transcriptional enhancer mapping 0.3 Mb upstream to FOXF1 on 16q24.

Molecular and phenotypic characterization of atypical Williams-Beuren syndrome.

Williams-Beuren syndrome (WBS) is a multisystemic genomic disorder typically caused by a recurrent ˜1.5-1.8 Mb deletion on 7q11.

Paracorporeal lung assist devices as a bridge to recovery or lung transplantation in neonates and young children.

Objective: To evaluate paracorporeal lung assist devices to treat neonates and children with decompensated respiratory failure as a bridge to recovery or lung transplantation.

Methods: One neonate (23 days old) and 3 young children (aged 2, 9, and 23 months) presented with primary lung disease with pulmonary hypertension, including alveolar capillary dysplasia in 2 and right pulmonary hypoplasia and primary pulmonary hypertension in 1. The patients were listed for lung transplantation but decompensated and required extracorporeal membrane oxygenation (ECMO).

Lung transplant is a viable treatment option for patients with congenital and acquired pulmonary vein stenosis.

Background: Congenital pulmonary vein stenosis (PVS) is associated with high mortality because surgical repair is usually not feasible or is ineffective. In addition, acquired PVS after repair of congenital heart disease is a potential complication that occurs in 5% to 10% of patients and carries a poor prognosis. Lung transplantation has been proposed as a viable option.

Outcomes of congenital diaphragmatic hernia in the modern era of management.

Objective: To identify clinical factors associated with pulmonary hypertension (PH) and mortality in patients with congenital diaphragmatic hernia (CDH).

Study Design: A prospective cohort of neonates with a diaphragm defect identified at 1 of 7 collaborating medical centers was studied. Echocardiograms were performed at 1 month and 3 months of age and analyzed at a central core by 2 cardiologists independently.

Neonatal paracorporeal lung assist device for respiratory failure.

Neonates who experience respiratory failure despite maximal ventilatory support have only extracorporeal membrane oxygenation as a rescue therapy, but it has very poor outcomes as a bridge to transplantation. A pumpless lung-assist device has been used in adults as a bridge to lung transplantation. An alternative membrane oxygenator, the Quadrox iD, is a suitable size for neonatal blood flow.

Noncardiac chest pain in children and adolescents: a biopsychosocial conceptualization.

Pediatric NCCP may be characterized by recurrent pain accompanied by emotional distress and functional impairment. This paper reviews and critiques literature on pediatric noncardiac chest pain (NCCP) and introduces a theoretical conceptualization to guide future study of NCCP in children and adolescents. A developmentally informed biopsychosocial conceptualization of NCCP etiology is proposed based on a synthesis of empirical evidence and clinical observations of pediatric NCCP within the context of relevant findings from the broader pediatric pain and anxiety literature.

Lung transplantation in children with idiopathic pulmonary arterial hypertension: an 18-year experience.

Background: The natural history of idiopathic pulmonary arterial hypertension (IPAH) in patients of all ages is one of relentless progression. For those who fail medical therapy, lung transplantation remains the ultimate palliation. In the USA, IPAH is the second leading indication for lung transplantation in children and first for children 1 to 5 years of age.