Purpose of review: To discuss the advances in the understanding of the pathophysiology of experimental and human hepatopulmonary syndrome (HPS) and in the management of HPS, particularly regarding liver transplantation.
Recent findings: Advances have been made in defining the pathophysiology of HPS in experimental models as well as in human disease, including the role of endothelin-1, pulmonary monocytes, and angiogenesis. Additionally, the implications of the presence of HPS as it relates to prioritizing patients for liver transplantation and posttransplant outcomes will also be reviewed.
Summary: Mechanisms of disease continue to be defined in HPS, providing potential targets for pharmacologic intervention. Outcomes after liver transplantation are also becoming clearer, including the management of HPS with severe hypoxemia.