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Hepatic vascular malformations are common although less frequently symptomatic, but may result in high\u2010output cardiac failure, portal hypertension and biliary ischaemia. Whilst the understanding of the genetic and cell signalling pathways that are the hallmark of hereditary haemorrhagic telangiectasia have been clarified, there remain challenges in therapy for these patients. Only patients with symptomatic hepatic vascular malformations require treatment, with most (63%) responding to first\u2010line medical therapy. For non\u2010responders, bevacizumab is effective in reducing cardiac output in those with heart failure secondary to hepatic vascular malformations as well as other manifestations of the disease. Although liver transplantation is the only curative option, optimal timing is critical. Novel anti\u2010angiogenetic drugs and those that target aberrant cell signalling pathway are being explored.<\/jats:p>","DOI":"10.1111\/liv.16008","type":"journal-article","created":{"date-parts":[[2024,6,7]],"date-time":"2024-06-07T09:00:21Z","timestamp":1717750821000},"page":"2220-2234","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Hepatic manifestations of hereditary haemorrhagic telangiectasia"],"prefix":"10.1111","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0029-9597","authenticated-orcid":false,"given":"Claire","family":"Kelly","sequence":"first","affiliation":[{"name":"Institute of Liver Studies Kings College Hospital  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisabetta","family":"Buscarini","sequence":"additional","affiliation":[{"name":"VASCERN HHT Reference Centre ASST Maggiore Hospital  Crema 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