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a syndrome consisting of liver disease (usually cirrhosis), hypoxemia, and the presence of intrapulmonary vascular dilatations; notable for the common presence of orthodeoxia and platypnea.
hepatopulmonary syndromeA condition in which hypoxemia due to intrapulmonary shunting and/or a V/Q mismatch develops in a Pt with liver cirrhosis; usually there is no apparent parenchymal lung disease, but Pts may have orthodeoxia, an unusual finding of ↑ hypoxemia with a change from the supine to the erect position; the pathogenesis of HPS is uncertain but may be due to an ↑ production of endogenous nitric oxide; shunting of HPS may respond to IV methylene blue
hepatopulmonary syndrome(hĕp″ă-tō-pŭl′mō-năr″ē) [″ + L. pulmo, lung]
A combination of liver disease, decreased arterial oxygen concentration, and dilatation of the blood vessels of the lung. Clinically the patient may have signs and symptoms of liver disease, including gastrointestinal bleeding, esophageal varices, ascites, palmar erythema, and splenomegaly. Pulmonary signs include clubbing of the fingers, cyanosis, dyspnea, and decreased arterial oxygen concentration while in an upright position (orthodeoxia).