The hepatopulmonary syndrome (HPS) is an important and often under-recognized vascular complication of cirrhosis and portal hypertension characterized by pulmonary vascular dilatation, which results in hypoxemia. This syndrome is identified in as many as 20% of patients who are evaluated for orthotopic liver transplantation (OLT), and it has recently been found to increase mortality in affected patients, particularly when hypoxemia is severe. Currently, OLT is the only therapy established to reverse intrapulmonary vasodilatation, although postoperative mortality is increased in patients with severe hypoxemia. No randomized controlled trials of pharmacologic therapies have been undertaken, but supplemental oxygen improves oxygenation. Data derived from case reports, small studies, and experimental models suggest that pharmacologic therapies may be effective. In cirrhotic patients with HPS, particularly those with moderate hypoxemia (PaO2 < 60 mmHg), OLT should be considered prior to the development of severe deoxygenation. Supplemental oxygen should be given to patients with a PaO2 < 60 mmHg or those with exercise oxygen desaturation. For those patients with mild hypoxemia or those who are not OLT candidates, a trial of pharmacologic treatment may be considered.
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