{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,3,2]],"date-time":"2025-03-02T05:40:54Z","timestamp":1740894054169,"version":"3.38.0"},"reference-count":19,"publisher":"SAGE Publications","issue":"3","license":[{"start":{"date-parts":[[2014,4,30]],"date-time":"2014-04-30T00:00:00Z","timestamp":1398816000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Ther Adv Respir Dis"],"published-print":{"date-parts":[[2014,6]]},"abstract":"<jats:sec><jats:title>Introduction:<\/jats:title><jats:p> Ambrisentan is an oral selective endothelin receptor antagonist licensed for use in pulmonary arterial hypertension (PAH). There are few data on clinical use and long-term tolerability in a wider range of patients with pulmonary hypertension (PH). <\/jats:p><\/jats:sec><jats:sec><jats:title>Methods:<\/jats:title><jats:p> All patients treated with ambrisentan over a 4-year period were identified. Baseline characteristics, liver function test (LFT) results and World Health Organization (WHO) functional class were retrieved from hospital databases. <\/jats:p><\/jats:sec><jats:sec><jats:title>Results:<\/jats:title><jats:p> 272 patients received ambrisentan between March 2009 and June 2013 (32% idiopathic PAH, 36% connective tissue disease PAH, 11% congenital heart disease PAH, 6% portopulmonary hypertension, 1% HIV PAH, 4% PH in association with lung disease, 8% chronic thromboembolic PH and 2% PH in association with sarcoidosis). 33.5% of patients received ambrisentan as monotherapy and 12% received ambrisentan as their initial PH therapy. 18% stopped treatment due to side effects and 12% stopped due to lack of efficacy. Oedema was the most common side effect leading to cessation of therapy, which occurred in 7% of patients. 57% of patients who discontinued ambrisentan due to side effects also discontinued other PAH therapies due to side effects previously or subsequently. Ambrisentan was discontinued in two (&lt;1%) patients due to abnormal LFTs. The 3-year survival in congenital heart disease PAH, idiopathic PAH and systemic sclerosis-associated PAH was 80%, 62%, and 38%, respectively ( p = 0.003). Survival was superior in patients in whom WHO functional class improved in response to therapy. <\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion:<\/jats:title><jats:p> Ambrisentan is used as an initial therapy and as monotherapy in a minority of patients in a large UK PH referral centre. Discontinuation due to side effects, and especially oedema, was higher than reported in previous studies while discontinuation due to abnormal LFTs was very uncommon. A majority of patients who discontinued therapy due to side effects also previously or subsequently discontinued other PAH therapies. Improvement in WHO functional class was associated with superior survival. <\/jats:p><\/jats:sec>","DOI":"10.1177\/1753465814532304","type":"journal-article","created":{"date-parts":[[2014,5,2]],"date-time":"2014-05-02T01:44:38Z","timestamp":1398995078000},"page":"71-77","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":11,"title":["Ambrisentan therapy in pulmonary hypertension: clinical use and tolerability in a referral centre"],"prefix":"10.1177","volume":"8","author":[{"given":"Robin","family":"Condliffe","sequence":"first","affiliation":[{"name":"Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield S10 2JF, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Charlie A.","family":"Elliot","sequence":"additional","affiliation":[{"name":"Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Judith","family":"Hurdman","sequence":"additional","affiliation":[{"name":"Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ian","family":"Sabroe","sequence":"additional","affiliation":[{"name":"Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital and Department of Infection and Immunity, University of Sheffield, Sheffield, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Catherine","family":"Billings","sequence":"additional","affiliation":[{"name":"Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David G.","family":"Kiely","sequence":"additional","affiliation":[{"name":"Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Neil","family":"Hamilton","sequence":"additional","affiliation":[{"name":"Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, 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