{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T03:34:27Z","timestamp":1791516867289,"version":"4.3.4"},"reference-count":22,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2021,3,18]],"date-time":"2021-03-18T00:00:00Z","timestamp":1616025600000},"content-version":"vor","delay-in-days":17,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100005646","name":"Actelion Pharmaceuticals","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005646","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002066","name":"GlaxoSmithKline foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002066","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009947","name":"Merck Sharp and Dohme","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100009947","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Internal Medicine Journal"],"published-print":{"date-parts":[[2021,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Portopulmonary hypertension (PoPH) is a rare condition with poorer survival compared to idiopathic\/familial pulmonary arterial hypertension (IPAH\/FPAH).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>To compare the characteristics, survival, prognostic factors and management of PoPH and IPAH\/FPAH patients and to assess the impact of treatment on survival of PoPH patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Analysis of data of prevalent and incident PoPH patients enrolled in the Spanish registry of PAH (REHAP) from January 1998 to December 2017 and comparison with IPAH\/FPAH patients. Variables analysed: patient and disease (PAH and liver) characteristics, first\u2010line PAH\u2010targeted therapy, causes of death, prognostic factors and survival (according to aetiology and treatment in PoPH patients).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Compared to IPAH\/FPAH patients (\n                      <jats:italic>n<\/jats:italic>\n                      =\u2009678), patients with PoPH (\n                      <jats:italic>n<\/jats:italic>\n                      =\u2009237) were predominantly men, older and had better functional class and higher prevalence of ascites. Haemodynamics were better. Biomarkers for heart failure were worse. Age\u2010 and sex\u2010adjusted 5\u2010year survival rate from diagnosis was 49.3% for PoPH patients and 68.7% for IPAH patients (\n                      <jats:italic>P<\/jats:italic>\n                      &lt;\u20090.001). Treated PoPH had better survival than non\u2010treated. PAH\u2010 and liver\u2010related causes accounted for 30.2% and 24.7% of deaths in PoPH patients. PoPH patients were less likely to receive first\u2010line PAH\u2010targeted therapy and this was associated with greater mortality. Increasing age, worse exercise capacity and ascites were independent prognostic factors of poorer survival; first\u2010line oral monotherapy was associated with improved survival. Eight (3.4%) PoPH patients underwent liver transplantation.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>PoPH patients are undertreated and show poorer survival than IPAH\/FPAH patients. First\u2010line treatment with PAH\u2010targeted therapy was associated with better survival. Presence of ascites was a predictor of mortality.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/imj.14751","type":"journal-article","created":{"date-parts":[[2020,1,14]],"date-time":"2020-01-14T05:52:46Z","timestamp":1578981166000},"page":"355-365","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":56,"title":["Portopulmonary hypertension: prognosis and management in the current treatment era \u2013 results from the REHAP registry"],"prefix":"10.1111","volume":"51","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6534-4977","authenticated-orcid":false,"given":"Maria","family":"Lazaro Salvador","sequence":"first","affiliation":[{"name":"Cardiology Department Hospital Virgen de la Salud  Toledo Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos A.","family":"Quezada Loaiza","sequence":"additional","affiliation":[{"name":"Pneumology Department Hospital Universitario Ram\u00f3n y Cajal  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Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Respiratorias (CIBERES)  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pilar","family":"Escribano\u2010Sub\u00edas","sequence":"additional","affiliation":[{"name":"Pulmonary Hypertension Unit, Cardiology Department Hospital Universitario 12 de Octubre  Madrid Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Cardiovasculares (CIBERCV)  Madrid Spain"},{"name":"Instituto de Salud Carlos III  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"REHAP 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