ABSTRACT
This chapter focuses on the pulmonary circulation in patients with cirrhosis and portal hypertension. A wide spectrum of pulmonary abnormalities have been reported in patients with liver disease. The prevalence, pathogenesis, pathophysiology, clinical manifestations, and current management of pulmonary hypertension and pulmonary arterial oxygen desaturation will be discussed. Endotoxins are normally cleared by both Kupffer cells and hepatocytes, but in portal systemic shunting, these substances reach the pulmonary circulation and may play a role in the pathogenesis of portapulmonary hypertension. Plexiform lesions are nonspecific and occur in primary pulmonary hypertension and pulmonary hypertension secondary to chronic liver disease, congenital heart disease, and chronic hypoxia. Hypoxemia occurring in association with liver disease may be the consequence of specific pathologic entities that affect both liver and lung or due to pulmonary functional-anatomical changes that occur in relation to any form of severe chronic liver disease.
