Hepatopulmonary syndrome

Clin Liver Dis. 2014 May;18(2):407-20. doi: 10.1016/j.cld.2014.01.003. Epub 2014 Feb 25.


The hepatopulmonary syndrome (HPS) is a pulmonary complication of cirrhosis and/or portal hypertension whereby patients develop hypoxemia as a result of alterations in pulmonary microvascular tone and architecture. HPS occurs in up to 30% of patients with cirrhosis. Although the degree of hypoxemia does not reliably correlate with the severity of liver disease, patients with HPS have a higher mortality than do patients with cirrhosis without the disorder. There has been progress into defining the mechanisms that lead to hypoxemia in HPS, but to date there are no therapeutic options for HPS aside from liver transplantation.

Keywords: Contrast echocardiography; Hepatopulmonary syndrome; Hypoxemia; Intrapulmonary vasodilatation; Liver transplantation; Portal hypertension.

Publication types

  • Review

MeSH terms

  • Animals
  • Diagnosis, Differential
  • Disease Models, Animal
  • Hepatopulmonary Syndrome / diagnosis
  • Hepatopulmonary Syndrome / etiology*
  • Hepatopulmonary Syndrome / surgery
  • Humans
  • Hypertension, Portal / complications
  • Hypertension, Portal / physiopathology
  • Hypoxia / etiology
  • Liver Cirrhosis / complications
  • Liver Cirrhosis / physiopathology
  • Liver Transplantation
  • Portasystemic Shunt, Transjugular Intrahepatic
  • Pulmonary Circulation
  • Rats
  • Vasodilation