{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T17:25:31Z","timestamp":1790270731464,"version":"4.1.0"},"reference-count":35,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2022,5,3]],"date-time":"2022-05-03T00:00:00Z","timestamp":1651536000000},"content-version":"vor","delay-in-days":32,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2022,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Even though pulmonary arterial hypertension (PAH) remains an incurable disease, the combination of PAH\u2010specific therapies allowed treatment strategies to evolve from symptom\u2010based ones to others that aim to move patients to low\u2010risk conditions. Endothelin\u20101 (ET\u20101) receptor antagonists emerged as specific\u2010PAH drugs that can be used in combination with other specific therapies. This work aimed to perform a prospective clinical assessment of patients with PAH that switched from bosentan to macitentan (POTENT), due to inadequate response. POTENT is a prospective, open\u2010label, single\u2010arm, uncontrolled study including PAH patients from our ongoing SAUDIPH registry. It enrolled 50 PAH patients divided as follows: idiopathic\/heritable pulmonary arterial hypertension (I\/HPAH); <jats:italic>n<\/jats:italic>\u2009=\u200924; PAH associated with congenital heart disease, <jats:italic>n<\/jats:italic>\u2009=\u200919; PAH associated with connective tissue diseases, <jats:italic>n<\/jats:italic>\u2009=\u20095; and pulmonary veno\u2010occlusive disease and\/or pulmonary capillary haemangiomatosis (PVOD\/PCH), <jats:italic>n<\/jats:italic>\u2009=\u20092. At baseline, most patients were in World Health Organization Functional Class (WHO FC) II\/III (52.0%). After switching to macitentan, patients were more likely to be in WHO FC I\/II (78%) and 22% of the overall cohort moved to a lower risk condition, with three low risk stratification parameters. Mean 6\u2010min walking distance increased about 34\u2009m after 12 months, with a significant mean change over time (12.63\u2009\u00b1\u200911.69 at month 3 vs. 40.75\u2009\u00b1\u200912.57 at month 12, <jats:italic>p<\/jats:italic>\u2009=\u20090.002). Most haemodynamic parameters decreased over time, with corresponding negative mean changes (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). The safety of macitentan was confirmed by the absence of anaemia and liver injury; clinical worsening was observed only in a small group of patients. In general, macitentan might be a valid alternative to bosentan in PAH stable patients\u00a0on combination therapy with insufficient clinical response, and presenting intermediate and high\u2010risk parameters. We anticipate that studying this strategy in PAH subgroups would further clarify its potential and limitations.<\/jats:p>","DOI":"10.1002\/pul2.12083","type":"journal-article","created":{"date-parts":[[2022,4,18]],"date-time":"2022-04-18T16:23:12Z","timestamp":1650298992000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Prospective clinical assessment of patients with pulmonary arterial hypertension switched from bosentan to macitentan (POTENT)"],"prefix":"10.1002","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9658-081X","authenticated-orcid":false,"given":"Abdullah M.","family":"Aldalaan","sequence":"first","affiliation":[{"name":"Department of Medicine, Pulmonary Hypertension Program King Faisal Specialist Hospital &amp; Research Center Riyadh Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarfraz A.","family":"Saleemi","sequence":"additional","affiliation":[{"name":"Department of Medicine, Pulmonary Hypertension Program King Faisal Specialist Hospital &amp; Research Center Riyadh Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ihab","family":"Weheba","sequence":"additional","affiliation":[{"name":"Department of Medicine, Pulmonary Hypertension Program King Faisal Specialist Hospital &amp; Research Center Riyadh Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abeer","family":"Abdelsayed","sequence":"additional","affiliation":[{"name":"Department of Medicine, Pulmonary Hypertension Program King Faisal Specialist Hospital &amp; Research Center Riyadh Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maha M.","family":"Aleid","sequence":"additional","affiliation":[{"name":"Department of Biostatistics, Epidemiology &amp; Scientific Computing King Faisal Specialist Hospital &amp; Research Center 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