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Physical examination, liver biopsy, clinical imaging and genetic testing disproved hereditary haemorrhagic telangiectasia (HHT) and liver cirrhosis. Bevacizumab (BVZ) was initiated on the basis of experience from treating vascular malformations in HHT patients. The patient has not shown signs of GI bleeding since the initiation of BVZ. Genetic testing detected a mutation in the <jats:italic>EPHB4<\/jats:italic> gene of previously unknown significance, but a connection with vascular malformations has been suggested in the literature. Collectively, this case calls for considering hepatic vascular malformations in patients with non-cirrhotic portal hypertension.<\/jats:p>","DOI":"10.1136\/bcr-2024-264654","type":"journal-article","created":{"date-parts":[[2025,4,7]],"date-time":"2025-04-07T16:20:34Z","timestamp":1744042834000},"page":"e264654","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Decompensated liver failure due to portal hypertension as a result of hepatic arteriovenous malformations"],"prefix":"10.1136","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-2562-127X","authenticated-orcid":false,"given":"Pernille Darre","family":"Haahr","sequence":"first","affiliation":[{"name":"Gastroenterology and Hepatology, Odense Universitetshospital, Odense, Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gustav Bang","family":"Harvald","sequence":"additional","affiliation":[{"name":"Gastroenterology and Hepatology, Odense Universitetshospital, Odense, Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0890-4728","authenticated-orcid":false,"given":"Annette Dam","family":"Fialla","sequence":"additional","affiliation":[{"name":"Gastroenterology and Hepatology, Odense Universitetshospital, Odense, Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2025,4,7]]},"reference":[{"key":"2025082722482778000_18.4.e264654.1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(21)01374-X"},{"key":"2025082722482778000_18.4.e264654.2","doi-asserted-by":"publisher","DOI":"10.1016\/s2468-1253(19)30349-8"},{"key":"2025082722482778000_18.4.e264654.3","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2018.09.014"},{"key":"2025082722482778000_18.4.e264654.4","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2015.07.040"},{"key":"2025082722482778000_18.4.e264654.5","doi-asserted-by":"crossref","unstructured":"Lautt WW . 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