Abstract
Portopulmonary hypertension (POPH) is a severe complication of portal hypertension, which can manifest with or without cirrhosis. The diagnosis is made based on elevated mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) with normal capillary wedge pressure measured during right heart catheterization. Without intervention, POPH carries a very poor prognosis and is characterized by obstruction of pulmonary arterial flow that progresses to right heart failure.
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