Hepatopulmonary syndrome: Update on pathogenesis and clinical features

Junlan Zhang, Michael B. Fallon

Research output: Contribution to journalReview article

57 Scopus citations

Abstract

Hepatopulmonary syndrome (HPS) is a serious vascular complication of liver disease that occurs in 5-32% of patients with cirrhosis. The presence of HPS markedly increases mortality. No effective medical therapies are currently available and liver transplantation is the only established treatment option for HPS. The definition and diagnosis of HPS are established by the presence of a triad of liver disease with intrapulmonary vascular dilation that causes abnormal arterial gas exchange. Experimental biliary cirrhosis induced by common bile duct ligation in the rat reproduces the pulmonary vascular and gas exchange abnormalities of human HPS and serves as a pertinent animal model. Pulmonary microvascular dilation and angiogenesis are two central pathogenic features that drive abnormal pulmonary gas exchange in experimental HPS, and thus might underlie HPS in humans. Defining the mechanisms involved in the microvascular alterations of HPS has the potential to lead to effective medical therapies. This Review focuses on the current understanding of the pathogenesis, clinical features and management of HPS.

Original languageEnglish (US)
Pages (from-to)539-549
Number of pages11
JournalNature Reviews Gastroenterology and Hepatology
Volume9
Issue number9
DOIs
StatePublished - Sep 1 2012
Externally publishedYes

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology

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