How Is Pulmonary Hypertension Characterised and Treated in Children With Trisomy 21? Observations From the TOPP Registry (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension).

Pulmonary hypertension is common in children with Trisomy 21, frequently with multifactorial aetiologies. Registry data provide better understanding of disease development, diagnostic workup and treatment patterns in children with Trisomy 21. TOPP (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) is a centre-based, comprehensive registry.

Enhancing Drug Development for Paediatric Pulmonary Hypertension-An Integrative Perspective.

As with adult pulmonary hypertension (PH), high morbidity and mortality persist with diverse types of paediatric PH. Despite major advances in pharmacologic therapies based on extensive studies in adult PH, few drugs have been comprehensively studied in neonates, infants, and children, leaving current paediatric PH care largely dependent on small observational studies and extrapolation of evidence from adult clinical trials. Challenges in developing successful clinical trials in children include the need to define distinct disease phenotypes with well-characterised natural history and outcomes, the lack of established age- and disease-specific study endpoints, small and heterogeneous paediatric populations, and the common off-label use of PH-targeted drug therapies without regulatory approval.

The Impact of Cardiac Output Methods on the Classification of Pulmonary Hypertension.

Cardiac output is essential to calculate pulmonary vascular resistance (PVR) and classify pulmonary hypertension (PH). Recent evidence has shown a lower agreement between thermodilution (COTD) and direct Fick (CODF) methods than historically estimated. The influence of the cardiac output measurement method on the classification of PH is poorly explored.

Pulmonary hypertension in pediatrics: from clinical suspicion to management.

Pediatric pulmonary hypertension differs from adult pulmonary hypertension in many ways, including multifactorial etiologies and comorbidities that can impact diagnosis, response to therapy, and outcome. The main etiologies of pediatric PH are idiopathic pulmonary arterial hypertension (PAH), PAH associated with congenital heart disease (PAH-CDH) and developmental lung disorders. Thorough diagnostic evaluation is necessary to properly classify pulmonary hypertension, find a potential treatable cause, and guide therapy.

Pulmonary Hypertension Associated with Interstitial Lung Disease: A Review on Diagnosis and Treatment with a Focus on Emerging Therapies.

Background: Pulmonary hypertension (PH) complicating interstitial lung diseases (ILDs) is critical for symptom burden and prognosis. The prevalence of PH varies according to ILD subgroups and severities. Establishing the diagnosis of PH associated with ILDs (PH-ILDs) is complex due to overlapping symptoms, late clinical signs, and poor diagnostic performance of routine noninvasive diagnostic methods.

Inhibition of Sodium/Hydrogen Exchanger-1 in the Right Ventricle and Lung Dysfunction Induced by Experimental Pulmonary Arterial Hypertension in Rats.

Background: Life-threatening pulmonary arterial hypertension (PAH) still lacks a direct therapeutic approach targeted to the molecular defects associated with the disease. Here, we focus on the impaired regulation of intracellular acidity and sodium/calcium overload by testing the hypothesis that inhibiting NHE-1 (sodium/hydrogen exchanger isoform 1) with rimeporide enables the recovery of pulmonary and right ventricular dysfunctions in the Sugen5416/hypoxia PAH model in rats.

Methods And Results: Adult Sprague-Dawley male rats (n=44) rats were divided into 2 broad groups: control and Sugen5416/hypoxia.

Epicardial Fat Volume Assessed by MRI in Adolescents: Associations with Obesity and Cardiovascular Risk Factors.

: In adults, epicardial adipose tissue (EAT) is associated with metabolic syndrome (MS) and coronary artery disease. EAT thickness is increased in obese youth, but total EAT volume and its correlation with cardiovascular risk factors have not been studied. : To determine EAT volume in adolescents and its association with obesity and cardiovascular risk factors.

Embracing the challenges of neonatal and paediatric pulmonary hypertension.

Paediatric pulmonary arterial hypertension (PAH) shares common features with adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for caring for infants and children with PAH, as presented by the paediatric task force of the 7th World Symposium on Pulmonary Hypertension. We provide updates on diagnosing, classifying, risk-stratifying and treating paediatric pulmonary hypertension (PH) and identify critical knowledge gaps.

The evolution of clinical trials for pediatric pulmonary hypertension: are the needs of patients and their caregivers being met?

Introduction: Pediatric pulmonary hypertension is a rare condition. Survival remains poor in the current management era. There is a lack of data regarding the medical management of pediatric pulmonary hypertension and most pulmonary vasodilators are used off-label in children.

Significance of colonization by antibiotic-resistant organisms prior to congenital heart disease surgery in children from low- to middle-income countries sent by non-governmental organizations to Switzerland.

Purpose: Children with congenital heart disease (CHD) from low- to middle-income countries (LMIC) are suspected to have a high prevalence of antibiotic-resistant microorganisms (ARMOs) carriage, but data are currently lacking. Carriage of ARMOs could impact the post-operative course in pediatric intensive care unit (PICU). The aim of the study was to assess the prevalence of ARMOs carriage in children with CHD from LMIC and its impact on post-operative outcomes.

Three-dimensional printed moulds to obtain silicone hearts with congenital defects for paediatric heart-surgeon training.

Objectives: Many types of congenital heart disease are amenable to surgical repair or palliation. The procedures are often challenging and require specific surgical training, with limited real-life exposure and often costly simulation options. Our objective was to create realistic and affordable 3D simulation models of the heart and vessels to improve training.

Long-term safety and tolerability of ambrisentan treatment for pediatric patients with pulmonary arterial hypertension: An open-label extension study.

This open-label, extension study assessed long-term safety, tolerability, and efficacy of ambrisentan in a pediatric population (age 8- < 18 years) with pulmonary arterial hypertension (PAH). Following completion of a 6-month, randomized study, participants entered the long-term extension at individualized ambrisentan dosages (2.5/5/7.

[Risks of magistral preparations in pediatrics].

Vasoreactive pulmonary arterial hypertension (PAH) in children is a form of idiopathic PAH that responds to vasoreactive testing with nitric oxide (NO) by a significant decrease of pulmonary vascular resistances and pressure. Oral calcium channel antagonists (CCA) that allow pulmonary arterial vasodilation are the treatment of choice. The therapeutic effect is strongly depending on adequate drug intake.

Infective Endocarditis Due to .

Infective endocarditis due to is a rare but serious invasive infection that occurs mostly in children. Recent advances in nucleic acid amplification testing as well as in cardiac imaging have enabled more accurate diagnosis. A good understanding of the epidemiology and virulence factors remains crucial to guide the therapeutic approach.

Dual-energy computed tomography to detect early pulmonary vascular changes in children with sickle cell disease: a pilot study.

Introduction: Pulmonary hypertension (PH) is a rare but fatal complication of sickle cell disease (SCD) that is possibly reversible if treated early. Dual-energy computed tomography (DECT) is a valuable tool for diagnosing PH. We attempted to determine if DECT can detect early signs of PH in children with SCD.

Triple switch' aortic and pulmonary root inversion and modified Senning procedure for anatomically complex left transposition of the great arteries with inlet ventricular septal defect and pulmonary stenosis.

Left transposition of the great arteries with inlet ventricular septal defect and pulmonary stenosis is a relatively uncommon cardiac malformation. Two surgical treatments are available: double switch or physiological correction. The choice of surgical technique depends on the results of a discussion between the family and the surgeon.

Decompressive hemicraniectomy in pediatric malignant arterial ischemic stroke: a case-based review.

Purpose: Malignant stroke is a life-threatening emergency, with a high mortality rate (1-3). Despite strong evidence showing decreased morbidity and mortality in the adult population, decompressive hemicraniectomy (DCH) has been scarcely reported in the pediatric stroke population, and its indication remains controversial, while it could be a potential lifesaving option.

Methods And Results: We performed an extensive literature review on pediatric malignant arterial ischemic stroke (pmAIS) and selected 26 articles reporting 97 cases.

Successful heart transplant in a child with congenital core myopathy and delayed-onset restrictive cardiomyopathy due to recessive mutations in the titin (TTN) gene.

Background: Mutations in the TTN gene, encoding the muscle filament titin, are a major cause of inherited dilated cardiomyopathy. Early-onset skeletal muscle disorders due to recessive TTN mutations have recently been described, sometimes associated with cardiomyopathies.

Case Description: We report the case of a boy with congenital core myopathy due to compound heterozygosity for TTN variants.

Pulmonary vascular disease as a complication of pediatric congenital heart diseases.

Congenital and acquired heart diseases can cause pulmonary hypertension (PH) in children, either by increasing pulmonary blood flow (PBF), left atrial pressure (LAp), and/or pulmonary vascular resistance (PVR). Pathophysiological process of pulmonary vascular disease (PVD) in different types of congenital heart diseases (CHDs) are reviewed hereafter. As with other types of PH, a rigorous diagnostic evaluation is mandatory to characterize the etiology of the PH, rule out other or additional causes of PH, and establish a risk profile.

Use of CO-Derived Variables in Cardiac Intensive Care Unit: Pathophysiology and Clinical Implications.

Shock is a life-threatening condition, and its timely recognition is essential for adequate management. Pediatric patients with congenital heart disease admitted to a cardiac intensive care unit (CICU) after surgical corrections are particularly at risk of low cardiac output syndrome (LCOS) and shock. Blood lactate levels and venous oxygen saturation (ScVO) are usually used as shock biomarkers to monitor the efficacy of resuscitation efforts, but they are plagued by some limitations.