Pulmonary hypertension for the non-specialist

Pulmonary hypertension is a rare and complex disease that arises from a wide range of underlying conditions. Therapeutic options have expanded enormously over the last two decades resulting in major improvements in prognosis for some patients. As a consequence, it is vital that the disease is recognised early and referred on to specialist centres for further investigation and definitive diagnosis to improve prognosis in this life-altering condition.

Pulmonary hypertension and hepatic encephalopathy: lethal complications of Rendu-Osler-Weber disease

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.