Portopulmonary hypertension in United Kingdom: A contemporary, national, observational study

Background We studied portopulmonary hypertension (PoPH) in an incident population. Aims To analyse the clinical characteristics, pulmonary haemodynamics and management of PoPH patients presenting between 2001 and 2010. Methods National database and case notes source review. Results One hundred and thirty-one patients with PoPH were identified from the UK national database and full validated data was achieved in 76 (35 females). Mean age at diagnosis was 53±13 years. 77% presented in WHO functional class III. There were 67 cirrhotic patients with causes including alcohol (42), hepatitis C (7), autoimmune (5), and primary biliary cirrhosis (4). The spread of Child-Pugh severity was: A 28, B 32 and C 7. Non-cirrhotic (9/76) causes included portal vein thrombosis (4/9), idiopathic portal hypertension (1), Budd-Chiari syndrome (1), and chronic veno-occlusive disease (1). Haemodynamic measurements at presentation showed a mean pulmonary arterial pressure of 49±10mmHg, cardiac index 2.9±1.0L/min/m2and pulmonary vascular resistance 7.6±3.6 Wood units. There was no association between severity of cirrhotic disease and haemodynamic abnormality apart from right atrial pressure between Child-Pugh A and B (9 vs 15mmHg; P<0.05). Treatment at last evaluation consisted of monotherapy in 72%, including phosphodiesterase-5 inhibitors (51%), endothelin receptor antagonists (13%), prostacyclins (7%), and combination therapy in 11%. Conclusion PoPH presents in cirrhotic and non-cirrhotic causes of portal hypertension. There was no correlation between the severity of liver disease and severity of PoPH. The commonest cause in United Kingdom is alcoholic cirrhosis.