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J Korean Radiol Soc. 2007 Nov;57(5):423-428. English. Case Report. https://doi.org/10.3348/jkrs.2007.57.5.423
Jun S , Lee W , Cheon JE , Kim WS , Kim IO , Yeon KM .
Department of Radiology, Seoul National University Hospital, Korea. leew@radiol.snu.ac.kr
Abstract

Congenital absence of the portal vein (CAPV) is a rare malformation in which intestinal and splenic venous flow bypasses the liver and drains directly into the systemic circulation via a congenital portosystemic shunt. We describe two cases of CAPV presenting as pulmonary hypertension that were initially suspected as primary pulmonary hypertension. However, subsequent ultrasonography and CT detected the absence of a portal vein and the presence of a portosystemic shunt. Pulmonary hypertension is a recognized complication of liver disease and portal hypertension. However, these two cases illustrate that CAPV may result in pulmonary hypertension without liver disease or portal hypertension.

Copyright © 2019. Korean Association of Medical Journal Editors.