{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T23:22:47Z","timestamp":1776381767915,"version":"3.51.2"},"reference-count":6,"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>Hereditary haemorrhagic telangiectasia (HHT) is a disorder of arteriovenous malformations and telangiectases. In rare cases affected individuals may develop typical pulmonary arterial hypertension (PAH). Vasodilator therapy has not been recommended because of a potential increase in arteriovenous shunt volume.<\/jats:p><jats:p>This report is on two patients with severe HHT\u2010associated PAH who were treated with bosentan, an oral endothelin ET<jats:sub>A<\/jats:sub>\/ET<jats:sub>B<\/jats:sub> receptor antagonist. After 1\u00a0year, symptomatic and functional improvements were confirmed by haemodynamic evaluation demonstrating a significant decrease of mean pulmonary artery pressures and an increase in cardiac index, without evidence for an increase in effective shunt volume.<\/jats:p>","DOI":"10.1111\/j.1365-2362.2006.01683.x","type":"journal-article","created":{"date-parts":[[2006,8,2]],"date-time":"2006-08-02T23:14:53Z","timestamp":1154560493000},"page":"71-72","source":"Crossref","is-referenced-by-count":25,"title":["Bosentan therapy for pulmonary arterial hypertension associated with hereditary haemorrhagic telangiectasia"],"prefix":"10.1111","volume":"36","author":[{"given":"D.","family":"Bonderman","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Nowotny","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N.","family":"Skoro\u2010Sajer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Adlbrecht","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"I. M.","family":"Lang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,8,2]]},"reference":[{"key":"e_1_2_5_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2004.02.028"},{"key":"e_1_2_5_3_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200110-028PP"},{"key":"e_1_2_5_4_2","doi-asserted-by":"publisher","DOI":"10.1586\/14779072.2.2.175"},{"key":"e_1_2_5_5_2","doi-asserted-by":"publisher","DOI":"10.1080\/07853890410018925"},{"key":"e_1_2_5_6_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa012212"},{"key":"e_1_2_5_7_2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00054804"}],"container-title":["European Journal of Clinical Investigation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1365-2362.2006.01683.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1365-2362.2006.01683.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,27]],"date-time":"2023-09-27T08:57:46Z","timestamp":1695805066000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1365-2362.2006.01683.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,8,2]]},"references-count":6,"journal-issue":{"issue":"s3","published-print":{"date-parts":[[2006,9]]}},"alternative-id":["10.1111\/j.1365-2362.2006.01683.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1365-2362.2006.01683.x","archive":["Portico"],"relation":{},"ISSN":["0014-2972","1365-2362"],"issn-type":[{"value":"0014-2972","type":"print"},{"value":"1365-2362","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,8,2]]}}}