title: Pulmonary hypertension and hepatic encephalopathy: lethal complications of Rendu-Osler-Weber disease.
abstract: Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterised by epistaxis, mucocutaneous telangiectasia with systemic manifestations due to visceral telangiectasia and arterio-venous malformations (AVMs). We describe unusual complications of HHT in a 68-year-old male who developed high-output cardiac failure with pulmonary hypertension in combination with hepatic encephalopathy due to hepatic AVMs. This case shows the importance of a multi-system approach to HHT and considers the treatment of its hepatic complications.
authors.affiliation: TJ Ford Prince of Wales Hospital Sydney NSW 2031 Australia. Email t.ford@unsw.edu.au.
authors.forename: T J
authors.initials: TJ
authors.lastname: Ford
authors.forename: M W
authors.initials: MW
authors.lastname: Fong
authors.forename: B C
authors.initials: BC
authors.lastname: Cheah
authors.forename: C
authors.initials: C
authors.lastname: Alexopolous
mesh_terms: D000368:Aged; D017809:Fatal Outcome; D006501:Hepatic Encephalopathy / Q000000981:diagnostic imaging / Q000209:etiology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000209:etiology*; D008297:Male; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*; D014463:Ultrasonography
keywords: hepatic arteriovenous malformation; hepatic encephalopathy; hereditary haemorrhagic telangiectasia; high-output cardiac failure; pulmonary hypertension