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In recent years, prognosis has improved, coinciding with the introduction of new PAH\u2010targeted therapies, although much of their use in children is off\u2010label. Evidence to guide the treatment of children with PAH is less extensive than for adults. The goal of this review is to discuss the treatment recommendations for children with PAH, as well as the evidence supporting the use of prostanoids, endothelin receptor antagonists (ERAs), and phosphodiesterase type 5 inhibitors (PDE5i) in this setting.<\/jats:p><\/jats:sec><jats:sec><jats:title>Data Sources<\/jats:title><jats:p>Nonsystematic PubMed literature search and authors\u2019 expertise.<\/jats:p><\/jats:sec><jats:sec><jats:title>Study Selection<\/jats:title><jats:p>Articles were selected concentrating on the nitric oxide (NO)\u2010soluble guanylate cyclase (sGC)\u2010cyclic guanosine monophosphate (cGMP) pathway in PAH. The methodology of an ongoing study evaluating the sGC stimulator riociguat in children with PAH is also described.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Despite recent medical advances, improved therapeutic strategies for pediatric PAH are needed. The efficacy and tolerability of riociguat in adults with PAH have been well trialed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The pooling of data across trials, supplemented by registry data, will help to confirm the safety and tolerability of prostanoids, ERAs, and PDE5i in children. Ongoing studies will clarify the place of sGC stimulators in the treatment strategy for pediatric PAH.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.24442","type":"journal-article","created":{"date-parts":[[2019,7,17]],"date-time":"2019-07-17T06:05:29Z","timestamp":1563343529000},"page":"1516-1526","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Treatment of pediatric pulmonary arterial hypertension: A focus on the NO\u2010sGC\u2010cGMP pathway"],"prefix":"10.1002","volume":"54","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1841-0927","authenticated-orcid":false,"given":"Maurice","family":"Beghetti","sequence":"first","affiliation":[{"name":"Pediatric Cardiology Unit and Centre Universitaire de Cardiologie et Chirurgie Cardiaque P\u00e9diatrique Children's University Hospital  Geneva Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3326-5959","authenticated-orcid":false,"given":"Matthias","family":"Gorenflo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics II, Pediatric Cardiology and Congenital Heart Defects, Center for Pediatrics University Hospital Heidelberg  Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2541-3402","authenticated-orcid":false,"given":"D. 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