J Korean Radiol Soc.  2007 Nov;57(5):423-428. 10.3348/jkrs.2007.57.5.423.

Congenital Absence of the Portal Vein Presenting as Pulmonary Hypertension

• Jun S
• Lee W
• Cheon JE
• Kim WS
• Kim IO
• Yeon KM

• KMID: 2103898
• DOI: http://doi.org/10.3348/jkrs.2007.57.5.423

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.

Keyword

Portal vein; Portosystemic shunt; Hypertension, pulmonary; Angiography, CT; Liver

MeSH Terms

Hypertension, Portal
Hypertension, Pulmonary*
Liver
Liver Diseases
Portal Vein*
Portasystemic Shunt, Surgical
Ultrasonography