{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T02:19:32Z","timestamp":1787019572190,"version":"build-2736575974"},"reference-count":25,"publisher":"European Respiratory Society (ERS)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2006,6,15]]},"published-print":{"date-parts":[[2006,9]]},"abstract":"<jats:p>Portopulmonary hypertension (POPH) is regarded as a subtype of pulmonary arterial hypertension (PAH); however, established PAH therapies have not been evaluated for this condition.<\/jats:p>\n                  <jats:p>\n                    The current authors treated 14 patients (four male, 10 female; mean (range) age 55\u2005(39\u201375)\u2005yrs) with moderate (n\u200a=\u200a1) or severe (n\u200a=\u200a13) POPH caused by alcoholic liver disease (n\u200a=\u200a7), chronic viral hepatitis (n\u200a=\u200a3), autoimmune hepatitis (n\u200a=\u200a3), and hepatic manifestation of hereditary haemorrhagic teleangiectasia (n\u200a=\u200a1) with oral sildenafil. Eight patients were newly started on pulmonary vasoactive treatment, while six patients were already on treatment with inhaled prostanoids (iloprost, n\u200a=\u200a5; treprostinil, n\u200a=\u200a1). During treatment with sildenafil, mean\u00b1\n                    <jats:sc>sd<\/jats:sc>\n                    6-min walk distance increased from 312\u00b1111\u2005m to 397\u00b199\u2005m after 3\u2005months, and 407\u00b197\u2005m after 12\u2005months. Mean\u00b1\n                    <jats:sc>sd<\/jats:sc>\n                    pro-brain natriuretic peptide levels decreased from 582\u00b1315\u2005ng\u00b7mL\n                    <jats:sup>-1<\/jats:sup>\n                    to 230\u00b1278\u2005ng\u00b7mL\n                    <jats:sup>-1<\/jats:sup>\n                    , and to 189\u00b1274ng\u00b7mL\n                    <jats:sup>-1<\/jats:sup>\n                    after 3 and 12\u2005months, respectively. Two patients died after 1 and 2\u2005months from liver failure and cardiac failure, respectively. There was a similar response to sildenafil treatment after 3 and 12\u2005months in patients on monotherapy and those on combination therapy.\n                  <\/jats:p>\n                  <jats:p>In conclusion, sildenafil might be effective in monotherapy and in combination therapy with inhaled prostanoids in portopulmonary hypertension, leading to significant improvement by 3\u2005months and sustained response over 12\u2005months.<\/jats:p>","DOI":"10.1183\/09031936.06.00030206","type":"journal-article","created":{"date-parts":[[2006,6,28]],"date-time":"2006-06-28T20:38:48Z","timestamp":1151527128000},"page":"563-567","source":"Crossref","is-referenced-by-count":176,"title":["Sildenafil treatment for portopulmonary hypertension"],"prefix":"10.1183","volume":"28","author":[{"given":"F.","family":"Reichenberger","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Voswinckel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Steveling","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Enke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Kreckel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"Olschewski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Grimminger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W.","family":"Seeger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H. 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