Open AccessPortopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview
Author(s) -
Mateo PorresAguilar,
José Altamirano,
Aldo Torre-Delgadillo,
Michael Charlton,
Andrés DuarteRojo
Publication year - 2012
Publication title -
european respiratory review
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.565
H-Index - 66
eISSN - 1600-0617
pISSN - 0905-9180
DOI - 10.1183/09059180.00007211
Subject(s) - hepatopulmonary syndrome , portopulmonary hypertension , medicine , contraindication , liver transplantation , portal hypertension , cardiology , pulmonary hypertension , liver disease , intensive care medicine , cirrhosis , transplantation , pathology , alternative medicine
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.