Primary pulmonary hypertension presenting as portal hypertension
Rosenberg, L.S.; Silverman, A.; Strain, J.E.
American Journal of Gastroenterology 71(4): 427-431
1979
ISSN/ISBN: 0002-9270 PMID: 453136 Document Number: 142071
A two and a half-year old child is described who presented with signs of portal hypertension (hematemesis, hepatosplenomegaly, ascites). Her subsequent work-up revealed that the "pressure-head" originated within the pulmonary arterial bed. Indeed, severe changes of primary pulmonary hypertension were found at autopsy. What is unique about this case is the absence of cardiopulmonary symptoms prior to the development of suprahepatic portal hypertension. In addition, the pulmonary disease developed in the absence of underlying chronic hepatic disease or extrahepatic portal vein thrombosis which, reportedly, can lead to pulmonary hypertension.