TY - JOUR T1 - Portopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW SP - 223 LP - 233 DO - 10.1183/09059180.00007211 VL - 21 IS - 125 AU - Mateo Porres-Aguilar AU - Jose T. Altamirano AU - Aldo Torre-Delgadillo AU - Michael R. Charlton AU - Andres Duarte-Rojo Y1 - 2012/09/01 UR - http://err.ersjournals.com/content/21/125/223.abstract N2 - Liver disease and portal hypertension can be associated with pulmonary vascular complications, including portopulmonary hypertension (POPH), characterised by an elevated mean pulmonary artery pressure secondary to an increased pulmonary vascular resistance, and hepatopulmonary syndrome (HPS), characterised by hypoxaemia due to pulmonary vasodilatation and shunting. Although clear diagnostic guidelines exist for both conditions on the basis of echocardiography, right heart catheterisation and arterial blood gases, there is considerable variation between centres regarding diagnosis and management of these conditions. Awareness of evaluation and management algorithms for POPH and HPS are critical for optimisation of outcomes in patients with these conditions. Key aspects of management of POPH and HPS include identification of patients likely to benefit from liver transplantation (LTx) and management before and after LTx. Although both disorders may improve after LTx, severe forms of POPH represent a contraindication to LTx. Novel approaches to the treatment of POPH and HPS offer new management options that may expand the pool of transplantable patients and improve overall outcomes. ER -