{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T15:55:05Z","timestamp":1790870105173,"version":"4.1.0"},"reference-count":30,"publisher":"BMJ","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Heart"],"published-print":{"date-parts":[[2007,5]]},"abstract":"<jats:p>\n                  <jats:bold>Background:<\/jats:bold> Oral bosentan is an established treatment for pulmonary arterial hypertension (PAH).<\/jats:p>\n               <jats:p>\n                  <jats:bold>Objective:<\/jats:bold> To evaluate safety, tolerability, and clinical and haemodynamic effects of bosentan in patients with PAH related to congenital heart disease (CHD).<\/jats:p>\n               <jats:p>\n                  <jats:bold>Patients:<\/jats:bold> 22 patients with CHD related PAH (8 men, 14 women, mean (SD) age 38 (10) years) were treated with oral bosentan (62.5 mg\u00d72\/day for the first 4 weeks and then 125 mg\u00d72\/day).<\/jats:p>\n               <jats:p>\n                  <jats:bold>Main outcome measures:<\/jats:bold> Clinical status, liver enzymes, World Health Organisation (WHO) functional class, resting oxygen saturations and 6-min walk test (6MWT) were assessed at baseline and at 1, 3, 6, and 12 months. Haemodynamic evaluation with cardiac catheterisation was performed at baseline and at 12 month follow-up.<\/jats:p>\n               <jats:p>\n                  <jats:bold>Results:<\/jats:bold> 12 patients had ventricular septal defect, 5 atrioventricular canal, 4 single ventricle, and 1 atrial septal defect. All patients tolerated bosentan well. No major side effects were seen. After a year of treatment, an improvement was seen in WHO functional class (2.5 (0.7) <jats:italic>v<\/jats:italic> 3.1 (0.7); p&lt;0.05), oxygen saturation at rest (87 (6%) <jats:italic>v<\/jats:italic> 81 (9); p&lt;0.001), heart rate at rest (81 (10) <jats:italic>v<\/jats:italic> 87 (14) bpm; p&lt;0.05), distance travelled in the 6MWT (394 (73) <jats:italic>v<\/jats:italic> 320 (108) m; p&lt;0.001), oxygen saturation at the end of the 6MWT (71 (14) <jats:italic>v<\/jats:italic> 63 (17%); p&lt;0.05), Borg index (5.3 (1.8) <jats:italic>v<\/jats:italic> 6.5 (1.3); p&lt;0.001), pulmonary vascular resistances index (14 (9) <jats:italic>v<\/jats:italic> 22 (12) WU m<jats:sup>2<\/jats:sup>; p&lt;0.001), systemic vascular resistances index (23 (11) <jats:italic>v<\/jats:italic> 27 (10) WU.m<jats:sup>2<\/jats:sup>; p&lt;0.01), pulmonary vascular resistances index\/systemic vascular resistances index (0.6 (0.5) <jats:italic>v<\/jats:italic> 0.9 (0.6); p&lt;0.05); pulmonary (4.0 (1.3) <jats:italic>v<\/jats:italic> 2.8 (0.9) l\/min\/m2; p&lt;0.001) and systemic cardiac output (4.2 (1.4) <jats:italic>v<\/jats:italic> 3.4 (1.1) l\/min\/m2; p&lt;0.05).<\/jats:p>\n               <jats:p>\n                  <jats:bold>Conclusions:<\/jats:bold> Bosentan was safe and well tolerated in adults with CHD related PAH during 12 months of treatment. Clinical status, exercise tolerance, and pulmonary haemodynamics improved considerably.<\/jats:p>","DOI":"10.1136\/hrt.2006.097360","type":"journal-article","created":{"date-parts":[[2006,11,29]],"date-time":"2006-11-29T20:24:28Z","timestamp":1164831868000},"page":"621-625","source":"Crossref","is-referenced-by-count":74,"title":["Long term effects of bosentan treatment in adult patients with pulmonary arterial hypertension related to congenital heart disease (Eisenmenger physiology): safety, tolerability, clinical, and haemodynamic effect"],"prefix":"10.1136","volume":"93","author":[{"given":"M","family":"D\u2019Alto","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C D","family":"Vizza","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E","family":"Romeo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R","family":"Badagliacca","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G","family":"Santoro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R","family":"Poscia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B","family":"Sarubbi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Mancone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P","family":"Argiento","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F","family":"Ferrante","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M G","family":"Russo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F","family":"Fedele","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R","family":"Calabr\u00f2","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2006,11,29]]},"reference":[{"key":"2025092001254414000_93.5.621.1","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.15.2.121"},{"key":"2025092001254414000_93.5.621.2","first-page":"755","volume":"46","year":"1958","journal-title":"BMJ"},{"key":"2025092001254414000_93.5.621.3","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra035488"},{"key":"2025092001254414000_93.5.621.4","doi-asserted-by":"publisher","DOI":"10.1053\/euhj.1998.1046"},{"key":"2025092001254414000_93.5.621.5","doi-asserted-by":"publisher","DOI":"10.1016\/j.ehj.2004.09.014"},{"key":"2025092001254414000_93.5.621.6","first-page":"100","volume":"15","year":"1996","journal-title":"J Heart Lung Transplant"},{"key":"2025092001254414000_93.5.621.7","doi-asserted-by":"publisher","DOI":"10.1053\/pcad.2002.127492"},{"key":"2025092001254414000_93.5.621.8","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa012212"},{"key":"2025092001254414000_93.5.621.9","first-page":"81","volume":"43","year":"2004","journal-title":"J Am Coll Cardiol"},{"key":"2025092001254414000_93.5.621.10","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00054804"},{"key":"2025092001254414000_93.5.621.11","doi-asserted-by":"publisher","DOI":"10.1016\/j.cardiores.2003.11.026"},{"key":"2025092001254414000_93.5.621.12","doi-asserted-by":"publisher","DOI":"10.1016\/S0167-5273(99)00062-5"},{"key":"2025092001254414000_93.5.621.13","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.69.3.233"},{"key":"2025092001254414000_93.5.621.14","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(01)06250-X"},{"key":"2025092001254414000_93.5.621.15","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2004.08.025"},{"key":"2025092001254414000_93.5.621.16","first-page":"716","volume":"150","year":"2005","journal-title":"Am Heart J"},{"key":"2025092001254414000_93.5.621.17","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.106.630715"},{"key":"2025092001254414000_93.5.621.18","first-page":"919","volume":"132","year":"1985","journal-title":"Can Med Assoc J"},{"key":"2025092001254414000_93.5.621.19","unstructured":"Grossman W. 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