Background: The hepatopulmonary syndrome is characterized by a clinical triad consisting of: 1) hepatic disease and/or portal hypertension, 2) intrapulmonary vascular dilatations, and 3) abnormal arterial oxygenations (partial oxygen pressure <70 mm Hg or an alveolar-arterial oxygen gradient >20 mm Hg). Its frequency varies according to the diagnostic methods used.
Objectives: To review the literature about the pathogeny, diagnosis and treatment of the hepatopulmonary syndrome.
Methods: A survey of the more relevant Brazilian and international publications on the hepatopulmonary syndrome in MEDLINE.
Results: The prevalence of the hepatopulmonary syndrome ranges from 4% to 17.5% depending on the diagnostic criteria used. The most commonly associated liver disease is cirrhosis. The alveolar-arterial oxygen gradient seems to be the best parameter for the assessment of the abnormalities of arterial oxygenation. For the detection of intrapulmonary vascular dilatations, contrast echocardiography is the method of choice because it is of easy execution and it can differentiate between the intrapulmonary and intracardiac communications. In the treatment of hepatopulmonary syndrome, it was possible to reproduce and confirm few successful reports using pharmacological therapy and/or intervention radiology. At present, liver transplantation is considered to be the main therapeutic option for these patients, with encouraging results.
Conclusions: The hepatopulmonary syndrome is a frequent disease that requires blood gas measurements for diagnosis. Liver transplantation is the treatment of choice for patients with the syndrome.