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Pulmonary hypertension accompanying portal hypertension is a rare cause of plexogenic pulmonary arteriopathy, which etiopathogenesis is still unknown. The case report presented is that of a young man who underwent surgery at age 8 for ruptured esophageal varices associated with portal thrombosis, and who later developed pulmonary hypertension which led to death at age 21. Hepatic filtration shunt and similarity of this syndrome to pulmonary hypertension subsequent to ingestion of the appetite depressant Aminorex suggest the hypothesis that pressor substances, not detoxified by the liver, are released into the pulmonary circulation.
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