Hepatopulmonary syndrome: An update

Abstract

Hepatopulmonary syndrome (HPS) is characterized by defects in oxygenation caused by intra-pulmonary vasodilation occurring because of chronic liver disease, portal hypertension, or congenital portosystemic shunts. Clinical implications of portal hypertension are very well-known, however, awareness of its effect on multiple organs such as the lungs are less known. The presence of HPS in chronic liver disease is associated with increased mortality. Medical therapies available for HPS have not been proven effective and definitive treatment for HPS is mainly liver transplantation (LT). LT improves mortality for patients with HPS drastically. This article provides a review on the definition, clinical presentation, diagnosis, and management of HPS.

Keywords: Hepatopulmonary syndrome; Chronic liver disease; Hypoxemia; Intrapulmonary vasodilatation; Liver failure

Core Tip: Hepatopulmonary syndrome (HPS) is a progressive disease, the presence of which in cirrhotic patients worsens their prognosis. Patients with HPS have an increase rate of mortality compared to those without HPS when matched for severity of liver disease, age, sex, and liver transplantation (LT). HPS should be identified in all patients with chronic liver disease and supportive management should be provided until definitive treatment, e.g., LT could be done.