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portopulmonary hypertension

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By Jason Mann No BS pulmonary critical care fellow
Michael J Krowka, Michael B Fallon, Steven M Kawut, Valentin Fuhrmann, Julie K Heimbach, Michael A E Ramsay, Olivier Sitbon, Ronald J Sokol
Two distinct pulmonary vascular disorders, hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) may occur as a consequence of hepatic parenchymal or vascular abnormalities. HPS and POPH have major clinical implications for liver transplantation. A European Respiratory Society Task Force on Pulmonary-Hepatic Disorders convened in 2002 to standardize the diagnosis and guide management of these disorders. These International Liver Transplant Society diagnostic and management guidelines are based on that task force consensus and should continue to evolve as clinical experience dictates...
July 2016: Transplantation
Michael J Krowka
Portopulmonary hypertension (POPH) refers to the presence of pulmonary arterial hypertension (PAH) in patients with portal hypertension. Pulmonary hypertension in patients with liver disease or portal hypertension can be due to multiple mechanisms, including hyperdynamic (high-flow) state, increased pulmonary venous congestion, and vascular constriction or obstruction of the pulmonary arterial bed. Vascular obstruction to pulmonary arterial flow, reflected by increased pulmonary vascular resistance (PVR), is a key parameter that defines POPH...
February 2012: Seminars in Respiratory and Critical Care Medicine
Rodrigo Cartin-Ceba, Michael J Krowka
Portopulmonary hypertension (POPH) is the presence of pulmonary arterial hypertension in patients with portal hypertension. Among liver transplant (LT) candidates, reported incidence rates of POPH range from 4.5% to 8.5%. In patients with LT, intraoperative death and immediate post-LT mortality are feared clinical events when transplantation is attempted in the setting of untreated, moderate to severe POPH; therefore, POPH precludes LT unless the mean pulmonary artery pressure can be reduced to a safe level and right ventricular function optimized...
May 2014: Clinics in Liver Disease
Zeenat Safdar, Sonja Bartolome, Norman Sussman
Portopulmonary hypertension (POPH) is a serious complication of cirrhosis that is associated with mortality beyond that predicted by the Model for End-Stage Liver Disease (MELD) score. Increased pulmonary vascular resistance (PVR) may be initiated by pulmonary vasoconstriction, altered levels of circulating mediators, or shear stress, and can eventually lead to the classic vascular remodeling (plexiform lesion) that characterizes POPH. Portal hypertension is a prerequisite for the diagnosis of POPH, although the severity of pulmonary hypertension is unrelated to the severity of portal hypertension or the nature or severity of liver disease...
August 2012: Liver Transplantation
Ki Tae Suk
Portal hypertension is a severe consequence of chronic liver diseases and is responsible for the main clinical complications of liver cirrhosis. Hepatic venous pressure gradient (HVPG) measurement is the best available method to evaluate the presence and severity of portal hypertension. Clinically significant portal hypertension is defined as an increase in HVPG to >10 mmHg. In this condition, the complications of portal hypertension might begin to appear. HVPG measurement is increasingly used in the clinical fields, and the HVPG is a robust surrogate marker in many clinical applications such as diagnosis, risk stratification, identification of patients with hepatocellular carcinoma who are candidates for liver resection, monitoring of the efficacy of medical treatment, and assessment of progression of portal hypertension...
March 2014: Clinical and Molecular Hepatology
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