title: Liver transplantation for hepatic arteriovenous malformation in hereditary haemorrhagic telangiectasia.
abstract: Symptomatic hepatic involvement is a rare complication of hereditary hemorrhagic telangiectasia and most commonly consists of fibrosis or cirrhosis. We describe a 33-year-old woman in whom multiple hepatic arteriovenous malformations led to high output cardiac failure and liver failure due to biliary necrosis with refractory biliary sepsis, requiring orthotopic liver transplantation. Hepatic arteriovenous malformations were the first manifestation of the disease and a similar asymptomatic hepatic tumour was subsequently detected in her 60-year-old father who also had the classical cutaneous stigmata of the syndrome. Unrecognised genetic factors may determine the clinical spectrum of hereditary haemorrhagic telangiectasia including the hepatic manifestations.
authors.affiliation: Department of Medicine, Clinical School of Medicine, University of Cambridge, Addenbrooke's NHS Trust, UK.
authors.forename: T
authors.initials: T
authors.lastname: Bauer
authors.forename: P
authors.initials: P
authors.lastname: Britton
authors.forename: D
authors.initials: D
authors.lastname: Lomas
authors.forename: D G
authors.initials: DG
authors.lastname: Wight
authors.forename: P J
authors.initials: PJ
authors.lastname: Friend
authors.forename: G J
authors.initials: GJ
authors.lastname: Alexander
mesh_terms: D000328:Adult; D001165:Arteriovenous Malformations / Q000601:surgery*; D005260:Female; D006801:Humans; D008099:Liver / Q000098:blood supply*; D017093:Liver Failure / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation; D010375:Pedigree; D018805:Sepsis / Q000209:etiology*; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*