{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,25]],"date-time":"2025-10-25T18:53:31Z","timestamp":1761418411353},"reference-count":12,"publisher":"Wiley","issue":"s3","license":[{"start":{"date-parts":[[2006,8,2]],"date-time":"2006-08-02T00:00:00Z","timestamp":1154476800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur J Clin Investigation"],"published-print":{"date-parts":[[2006,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:bold>Background\u2002<\/jats:bold> Bosentan, a dual endothelin receptor antagonist, has been used for symptomatic improvement of patients with idiopathic pulmonary arterial hypertension (PAH) and specific types of secondary (e.g. scleroderma and human immunodeficiency virus infection) PAH, but its efficacy in patients with congenital heart disease and chronic thromboembolic pulmonary hypertension is still under evaluation. In this study two patients are presented, one with common atrioventricular canal and pulmonary hypertension and the other with recurrent pulmonary thromboembolism; both improved significantly after bosentan administration.<\/jats:p><jats:p><jats:bold>Materials and methods\u2002<\/jats:bold> The first patient was a 38\u2010year\u2010old female with trisomy\u00a021, common atrioventricular canal and pulmonary hypertension (Eisenmenger syndrome). The second patient was a 57\u2010year\u2010old male, who was on anticoagulant therapy owing to an episode of pulmonary embolism that had been diagnosed 3\u00a0years earlier. Recurrent pulmonary thromboembolism was considered the most probable diagnosis. Chest spiral computed tomography examination excluded pulmonary endarterectomy as a therapeutic approach. At admission, the two patients were on New York Heart Association class\u00a0IV, diagnosed by progressive rest dyspnea. Bosentan was administered to patients 1 and 2 for 6 and 9\u00a0months, respectively (62\u00b75\u00a0mg b.i.d. for 4\u00a0weeks, then 125\u00a0mg b.i.d.).<\/jats:p><jats:p><jats:bold>Results\u2002<\/jats:bold> Bosentan therapy resulted in significant clinical improvement in both patients. No hepatic dysfunction nor any other side\u2010effect was observed.<\/jats:p><jats:p><jats:bold>Conclusions\u2002<\/jats:bold> Bosentan could be an effective therapy for pulmonary hypertension owing to congenital heart disease and chronic thromboembolic disease.<\/jats:p>","DOI":"10.1111\/j.1365-2362.2006.01682.x","type":"journal-article","created":{"date-parts":[[2006,8,2]],"date-time":"2006-08-02T23:14:53Z","timestamp":1154560493000},"page":"39-43","source":"Crossref","is-referenced-by-count":5,"title":["Bosentan in Eisenmenger syndrome and chronic thromboembolic pulmonary hypertension"],"prefix":"10.1111","volume":"36","author":[{"given":"S.","family":"Kourouklis","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Christopoulos","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K.","family":"Liagkas","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"Paradellis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Z. 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