{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T11:22:01Z","timestamp":1779276121471,"version":"3.51.4"},"reference-count":6,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2019,12,1]],"date-time":"2019-12-01T00:00:00Z","timestamp":1575158400000},"content-version":"vor","delay-in-days":61,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2019,10,1]],"date-time":"2019-10-01T00:00:00Z","timestamp":1569888000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2019,10]]},"abstract":"<jats:p>Hereditary haemorrhagic telangiectasia or Rendu\u2010Osler\u2010Weber syndrome is a systemic vascular disease with autosomal dominant inheritance, mucocutaneous telangiectasia, and repeated nasal bleeding due to vascular abnormalities. Hereditary haemorrhagic telangiectasia may occasionally lead to complications, including arteriovenous malformations and pulmonary hypertension. We present a case of a 52\u2010year\u2010old female patient with hereditary haemorrhagic telangiectasia who was referred to our hospital for treatment of pulmonary hypertension. She had been diagnosed with hereditary haemorrhagic telangiectasia during adolescence and was being followed up. Six months prior to presentation, she had undergone coil embolization for pulmonary haemorrhage due to pulmonary arteriovenous malformations. She was in World Health Organization functional class IV, with a mean of pulmonary arterial pressure of 38\u2009mmHg, a pulmonary capillary wedge pressure of 10\u2009mmHg, and a right atrial pressure of 22\u2009mmHg. A contrast\u2010enhanced computed tomography angiography showed large arteriovenous malformations in the liver. Right heart catheterization revealed an increase in oxygen saturation in the inferior vena cava between the supra\u2010 and infra\u2010hepatic veins, low pulmonary vascular resistance, and high right atrial pressure. Hence, she was diagnosed with hereditary haemorrhagic telangiectasia with pulmonary hypertension due to major arteriovenous shunt resulting from arteriovenous malformations in the liver. Therefore, we considered liver transplantation as an essential treatment option. She underwent cadaveric liver transplantation after a year resulting in dramatic haemodynamic improvement to World Health Organization functional class I. Liver transplantation is a promising treatment in patients with hereditary haemorrhagic telangiectasia and pulmonary hypertension resulting from arteriovenous shunt caused by arteriovenous malformations in the liver.<\/jats:p>","DOI":"10.1177\/2045894019896677","type":"journal-article","created":{"date-parts":[[2019,12,5]],"date-time":"2019-12-05T10:53:52Z","timestamp":1575543232000},"page":"1-4","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":5,"title":["Liver transplantation in a patient with hereditary haemorrhagic telangiectasia and pulmonary hypertension"],"prefix":"10.1002","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6645-2075","authenticated-orcid":false,"given":"Kentaro","family":"Ejiri","sequence":"first","affiliation":[{"name":"Department of Cardiovascular Medicine Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences Okayama City Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9392-7117","authenticated-orcid":false,"given":"Satoshi","family":"Akagi","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences Okayama City Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kazufumi","family":"Nakamura","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences Okayama City Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naofumi","family":"Amioka","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences Okayama City Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keishi","family":"Ichikawa","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences Okayama City Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takahito","family":"Yagi","sequence":"additional","affiliation":[{"name":"Department of Hepato\u2010Biliary\u2010Pancreatic Surgery Okayama University Hospital Okayama City Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroshi","family":"Ito","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences Okayama City Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2019,12]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2012.250"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00061308"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.sla.0000247258.35406.a4"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199510053331407"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200009283431305"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1053\/jlts.2000.6356"}],"container-title":["Pulmonary Circulation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1177\/2045894019896677","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1177\/2045894019896677","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1177\/2045894019896677","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,28]],"date-time":"2023-08-28T15:56:42Z","timestamp":1693238202000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1177\/2045894019896677"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,10]]},"references-count":6,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2019,10]]}},"alternative-id":["10.1177\/2045894019896677"],"URL":"https:\/\/doi.org\/10.1177\/2045894019896677","archive":["Portico"],"relation":{},"ISSN":["2045-8940","2045-8940"],"issn-type":[{"value":"2045-8940","type":"print"},{"value":"2045-8940","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,10]]}}}