Journal
CHEST
Volume 123, Issue 2, Pages 562-576Publisher
ELSEVIER
DOI: 10.1378/chest.123.2.562
Keywords
endothelin; liver; nitric oxide; portal hypertension; portopulmonary hypertension; prostacyclin; pulmonary hypertension; serotonin; transplant
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Pulmonary involvement is common in patients with portal hypertension and can manifest in diverse manners. Changes in pulmonary arterial resistance, manifesting either as the hepatopulmonary. syndrome or portopulmonary hypertension (PPHTN), have been increasingly recognized in these patients in recent years. This review summarizes the clinicopathologic features, diagnostic criteria, as well as the latest concepts in the pathogenesis and management of PPHTN, which is defined as an elevated pulmonary artery pressure in the setting of an increased pulmonary vascular resistance and a normal wedge pressure in a patient with portal hypertension.
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