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. © 2014 Royal College of Physicians of Edinburgh.