HTP

Paediatric pulmonary arterial hypertension (PAH) shares common features of 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 the care of children with PAH, as presented by the Paediatric Task Force of the 6th World Symposium on Pulmonary Hypertension. We provide updates of the current definition, epidemiology, classification, diagnostics and treatment of paediatric PAH, and identify critical knowledge gaps...

Pulmonary hypertension is a severe condition that can develop during childhood due to several different etiologies. During the last two decades, based on a better understanding of the underlying pathobiology leading to pulmonary arterial hypertension, targeted therapies have been developed and have improved the dreadful prognosis of the condition. However, curative therapy remains elusive. Areas covered: In this article, we will review the current available drugs in paediatric pulmonary arterial hypertension and discuss the recommended management strategies...

National guidelines for the diagnosis and management of hypertension in children have been available for nearly 40 years. Unfortunately, knowledge and recognition of the problem by clinicians remain poor. Prevalence estimates are highly variable because of differing standards, populations, and blood pressure (BP) measurement techniques. Estimates in the United States range from 0.3% to 4.5%. Risk factors for primary hypertension include overweight and obesity, male sex, older age, high sodium intake, and African American or Latino ancestry...

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Persistent pulmonary hypertension of the newborn (PPHN) is the most common neonatal form and mostly reversible after a few days with improvement of the underlying pulmonary condition. When pulmonary hypertension (PH) persists despite adequate treatment, the severity of parenchymal lung disease should be assessed by chest CT. Pulmonary vein stenosis may need to be ruled out by cardiac catheterisation and lung biopsy, and genetic workup is necessary when alveolar capillary dysplasia is suspected. In PPHN, optimisation of the cardiopulmonary situation including surfactant therapy should aim for preductal SpO2between 91% and 95% and severe cases without post-tricuspid-unrestrictive shunt may receive prostaglandin E1 to maintain ductal patency in right heart failure...

Pulmonary hypertension (PH) is a condition of multiple aetiologies with underestimated prevalence and incidence. Indeed, despite access to modern therapies, pulmonary hypertensive vascular disease (PHVD) remains a progressive, usually life-limiting condition, severely impacting on the patients' well-being. We herein provide practical, expert consensus recommendations on the initial diagnostic work-up, clinical management and follow-up of children and adolescents with PH/PHVD, including a diagnostic algorithm...

Acute pulmonary hypertension (PH) complicates the course of several cardiovascular, pulmonary and other systemic diseases in children. An acute rise of RV afterload, either as exacerbating chronic PH of different aetiologies (eg, idiopathic pulmonary arterial hypertension (PAH), chronic lung or congenital heart disease), or pulmonary hypertensive crisis after corrective surgery for congenital heart disease, may lead to severe circulatory compromise. Only few clinical studies provide evidence on how to best treat children with acute severe PH and decompensated RV function, that is, acute RV failure...

Treatment of children and adults with pulmonary hypertension (PH) with or without cardiac dysfunction has improved in the last two decades. The so-called pulmonary arterial hypertension (PAH)-specific medications currently approved for therapy of adults with PAH target three major pathways (endothelin, nitric oxide, prostacyclin). Moreover, some PH centres may use off-label drugs for compassionate use. Pulmonary hypertensive vascular disease (PHVD) in children is complex, and selection of appropriate therapies remains difficult...

Pulmonary hypertension and related pulmonary vascular diseases cause significant morbidities and high mortality and present many unique challenges toward improving outcomes in neonates, infants, and children. Differences between pediatric and adult disease are reflected in controversies regarding etiologies, classification, epidemiology, diagnostic evaluations, and therapeutic interventions. This brief review highlights several key topics reflecting recent advances in the field and identifies persistent gaps in our understanding of clinical pediatric pulmonary hypertension...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

Pediatric pulmonary arterial hypertension (PAH) is an uncommon disease that can occur in neonates, infants, and children, and is associated with high morbidity and mortality. Despite advances in treatment strategies over the last two decades, the underlying structural and functional changes to the pulmonary arterial circulation are progressive and lead eventually to right heart failure. The management of PAH in children is complex due not only to the developmental aspects but also because most evidence-based practices derive from adult PAH studies...

Pulmonary arterial hypertension (PAH) is a rare, progressive disease affecting both adults and children. While overall survival has improved in recent years, the need for improved therapeutic approaches remains. Treatments for paediatric PAH have not yet been sufficiently examined, particularly regarding potential toxicities and optimal dosing, and there is a lack of appropriate clinical trial end-points and validated treatment goals that might enable a goal-oriented therapeutic approach. Adult randomised controlled trials in PAH are demonstrating a shift towards more long-term designs, focusing on mortality and morbidity end-points rather than changes in 6-min walking distance...

Pulmonary arterial hypertension (PAH) is a rare disease leading to right heart failure and death. Prognosis remains poor, particularly for patients with severe disease, i.e. World Health Organization functional class IV. There have been significant improvements in treatment options. Several agents are available that target the three main established PAH disease pathways, and can be combined sequentially or upfront. Strong scientific evidence supports the use of intravenous epoprostenol in severe PAH; however, despite recommendations, many patients do not receive parenteral prostanoids and there is a lack of evidence from randomised clinical trials supporting the value of other PAH medications alone in severe PAH...

Pulmonary arterial hypertension (PAH) is a right heart failure syndrome. In early-stage PAH, the right ventricle tends to remain adapted to afterload with increased contractility and little or no increase in right heart chamber dimensions. However, less than optimal right ventricular (RV)-arterial coupling may already cause a decreased aerobic exercise capacity by limiting maximum cardiac output. In more advanced stages, RV systolic function cannot remain matched to afterload and dilatation of the right heart chamber progressively develops...

Pulmonary arterial hypertension (PAH) is a complex, progressive disease with several pathobiological mechanisms, including the endothelin, nitric oxide and prostacyclin pathways. Current treatments for PAH target one of these pathways and, in more severe cases or instances of disease worsening, may be combined with a view to target multiple pathways in parallel. Treatment combination is performed sequentially (as an intensification from initial monotherapy) or upfront (use of two or more therapies in treatment-naïve patients)...

In recent years, many new, effective therapies for pulmonary arterial hypertension (PAH) have become available and are widely used, yet the long-term prognosis for patients with PAH remains poor. In the absence of a cure, physicians' expectations of PAH-specific therapies are to: 1) improve patients' symptoms and functional capacity; 2) slow disease progression; and 3) improve survival. However, patients with PAH may prioritise other more tangible needs, such as improvements in their ability to carry out their daily tasks and increase their quality of life...