Purpose of review: To summarize the pulmonary complications seen in cirrhosis.
Recent findings: The definition of portopulmonary hypertension (POPH) has been refined to exclude cardiac disease. POPH may be treated with a variety of agents; inhaled agents are particularly useful in the peri-transplant period. Hepatopulmonary syndrome (HPS) remains refractory to medical therapy.
Summary: Cirrhosis may be complicated by one of two pulmonary vascular complications, portopulmonary hypertension (POPH) and hepatopulmonary syndrome (HPS). POPH is a syndrome of increased vascular resistance, initiated by pulmonary vascular spasm. HPS is caused by intrapulmonary arteriovenous shunting with resultant hypoxemia. Both conditions are associated with portal hypertension, but are unrelated to the degree of portal hypertension, the nature or severity of the liver disease, and are associated with mortality in excess of the model for end-stage liver disease score. POPH is usually responsive to vasodilators, while HPS remains resistant to therapeutic agents. Both conditions are improved or cured by liver transplantation.