Sindrome hepatopulmonar

Abstract

Hepatopulmonary syndrome (HPS) was first recognized in 1884 by Fluckiger, however it was until 1977 tat Kennedy and Knudson considered it a syndrome. It consists of hepatic failure, pulmonary vasodilation and hypoxemia. The causes of this clinical entity are acute or chronic hepatic failure. The endothelium-derived relaxing factor is the main cause for pulmonary vascular disorders. The clinical spectrum includes symptoms produced by hepatic failure per se, such as ascitis, jaundice, esophageal varices, palmar erythema, stomach bleeding and those produced by the pulmonary component such as orthodeoxia, platypnea, digital clubbing and cyanosis. Hypoxemia mechanisms in HPS are ventilation/perfusion inequality, shunts and diffusion limitation. Diagnosis can be based on various studies, such as chest X-rays, lung perfusion scan, two-dimensional contrast echocardiography and pulmonary arteriography. To date there is no completely effective treatment, however, liver transplantation has been considered as the best choice, though others like spring-coil embolization and pharmacological therapies can be used.