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KMID : 1103720070570050423
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Journal of the Korean Society of Radiology
2007 Volume.57 No. 5 p.423 ~ p.428
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Congenital Absence of the Portal Vein Presentings as Pulmonary Hypertension
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Jun Sur-Young
Lee Whal
Cheon Jung-Eun
Kim Woo-Sun
Kim In-Won
Yeon Kyung-Mo
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Abstract
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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.
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KEYWORD
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Portal vein, Portosystemic shunt, Hypertension, pulmonary, Angiography, CT, Liver
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