title: Hepatopulmonary syndrome: update on pathogenesis and clinical features.
abstract: Hepatopulmonary syndrome (HPS) is a serious vascular complication of liver disease that occurs in 5-32% of patients with cirrhosis. The presence of HPS markedly increases mortality. No effective medical therapies are currently available and liver transplantation is the only established treatment option for HPS. The definition and diagnosis of HPS are established by the presence of a triad of liver disease with intrapulmonary vascular dilation that causes abnormal arterial gas exchange. Experimental biliary cirrhosis induced by common bile duct ligation in the rat reproduces the pulmonary vascular and gas exchange abnormalities of human HPS and serves as a pertinent animal model. Pulmonary microvascular dilation and angiogenesis are two central pathogenic features that drive abnormal pulmonary gas exchange in experimental HPS, and thus might underlie HPS in humans. Defining the mechanisms involved in the microvascular alterations of HPS has the potential to lead to effective medical therapies. This Review focuses on the current understanding of the pathogenesis, clinical features and management of HPS.
authors: Division of Gastroenterology, Hepatology and Nutrition, Department of Internal Medicine, The University of Texas Health Science Center at Houston, 6431 Fannin Street, MSB 4.234, Houston, TX 77030-1501, USA.
authors: Junlan
authors: J
authors: Zhang
authors: Michael B
authors: MB
authors: Fallon
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D020065:Hepatopulmonary Syndrome / Q000209:etiology* / Q000503:physiopathology*; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000503:physiopathology; D008168:Lung / Q000098:blood supply* / Q000503:physiopathology*; D055806:Microvessels / Q000503:physiopathology; D009389:Neovascularization, Pathologic / Q000503:physiopathology; D011659:Pulmonary Gas Exchange / Q000502:physiology; D051381:Rats; D014664:Vasodilation / Q000502:physiology