{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T02:34:15Z","timestamp":1780367655602,"version":"3.54.1"},"reference-count":87,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2022,10,17]],"date-time":"2022-10-17T00:00:00Z","timestamp":1665964800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"JSPS KAKENHI","award":["22K06701"],"award-info":[{"award-number":["22K06701"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pharmaceuticals"],"abstract":"<jats:p>Pulmonary arterial hypertension (PAH) is a disease in which stenosis or obstruction of the pulmonary arteries (PAs) causes an increase in PA pressure, leading to right-sided heart failure and death. Basic research has revealed a decrease in the levels of endogenous vasodilators, such as prostacyclin, and an increase in the levels of endogenous vasoconstrictors, such as endothelin, in patients with PAH, leading to the development of therapeutic agents. Currently, therapeutic agents for PAH target three pathways that are selective for PAs: the prostacyclin, endothelin, and nitric oxide pathways. These treatments improve the prognosis of PAH patients. In this review, we introduce new drug therapies and provide an overview of the current therapeutic agents.<\/jats:p>","DOI":"10.3390\/ph15101277","type":"journal-article","created":{"date-parts":[[2022,10,18]],"date-time":"2022-10-18T00:31:01Z","timestamp":1666053061000},"page":"1277","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Recent Advances in the Treatment of Pulmonary Arterial Hypertension"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0127-2118","authenticated-orcid":false,"given":"Naoyuki","family":"Otani","sequence":"first","affiliation":[{"name":"Department of Cardiology, Dokkyo Medical University Nikkyo Medical Center, 632 Takatoku, Nikko 321-2593, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"Tomoe","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Dokkyo Medical University Nikkyo Medical Center, 632 Takatoku, Nikko 321-2593, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsuhiko","family":"Kawabe","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Dokkyo Medical University Nikkyo Medical Center, 632 Takatoku, Nikko 321-2593, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takushi","family":"Sugiyama","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Dokkyo Medical University Nikkyo Medical Center, 632 Takatoku, Nikko 321-2593, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuto","family":"Horie","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Dokkyo Medical University Nikkyo Medical Center, 632 Takatoku, Nikko 321-2593, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroyuki","family":"Sugimura","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Dokkyo Medical University Nikkyo Medical Center, 632 Takatoku, Nikko 321-2593, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1940-2347","authenticated-orcid":false,"given":"Takanori","family":"Yasu","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Dokkyo Medical University Nikkyo Medical Center, 632 Takatoku, Nikko 321-2593, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takaaki","family":"Nakamoto","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Dokkyo Medical University Nikkyo Medical Center, 632 Takatoku, Nikko 321-2593, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,10,17]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1533","DOI":"10.1016\/S0140-6736(03)13167-4","article-title":"Primary pulmonary hypertension","volume":"361","author":"Runo","year":"2003","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1056\/NEJM199207093270203","article-title":"The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension","volume":"327","author":"Rich","year":"1992","journal-title":"N. 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