[Hepatic cirrhosis and primary pulmonary hypertension. A case report (author's transl)].

The present report deals with the clinical history and autopsy findings of a 41 year-old male patient with hepatic cirrhosis, surgical splenorenal shunt and severe pulmonary hypertension. Since the original description of Mantz and Craige several series have been reported, both in children and in adults, of the association between pulmonary hypertension and liver disease and/or portal hypertension. However, the type of hepatic alteration associated to pulmonary hypertension and the mechanisms responsible for this association remain unclarified. In the present case prominent plexiform and/or angiomatoid lesions of the pulmonary vasculature were observed, prompting the authors to speculate on the possibility that non-identified vasoconstrictor substances might contribute to the production of morphological lesions in the arterio-venous pulmonary shunts.