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Portopulmonary hypertension (POPH) refers to the presence of
pulmonary arterial hypertension (PAH) in patients with portal hypertension.
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Among patients with portal hypertension, reported incidence rates of POPH
range from 2 to 9%.
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Long-term survival in cases of POPH is poor.
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Pulmonary hypertension in patients with liver disease or portal hypertension
can be due to multiple mechanisms:
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► hyperdynamic (high-flow) state
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► increased pulmonary venous congestion
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► vascular constriction or obstruction of the pulmonary arterial bed
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► hyperdynamic (high-flow) state
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Vascular obstruction to pulmonary arterial flow, reflected by increased
pulmonary vascular resistance (PVR), is a key parameter that defines POPH
Driving Pressure and Survival in the Acute Respiratory Distress Syndrome Marcelo B.P. Amato, M.D., Maureen O. Meade, M.D., Arthur S. Slutsky, M.D., Laurent Brochard, M.D., Eduardo L.V. Costa, M.D., David A. Schoenfeld, Ph.D., Thomas E. Stewart, M.D., Matthias Briel, M.D., Daniel Talmor, M.D., M.P.H., Alain Mercat, M.D., Jean-Christophe M. Richard, M.D., Carlos R.R. Carvalho, M.D., and Roy G. Brower, M.D. N Engl J Med 2015; 372:747-755 February 19, 2015 DOI: 10.1056/NEJMsa1410639 BACKGROUND Mechanical-ventilation strategies that use lower end-inspiratory (plateau) airway pressures, lower tidal volumes (V T ), and higher positive end-expiratory pressures (PEEPs) can improve survival in patients with the acute respiratory distress syndrome (ARDS), but the relative importance of each of these components is uncertain. Because respiratory-system compliance (C RS ) is strongly related to the volume of aerated remaining functional lung during disease (termed functional lung size)...
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