Congenital Absence of the Portal Vein Presenting as Pulmonary Hypertension.
10.3348/jkrs.2007.57.5.423
- Author:
Suryoung JUN
1
;
Whal LEE
;
Jung Eun CHEON
;
Woo Sun KIM
;
In One KIM
;
Kyung Mo YEON
Author Information
1. Department of Radiology, Seoul National University Hospital, Korea. leew@radiol.snu.ac.kr
- Publication Type:Case Report
- Keywords:
Portal vein;
Portosystemic shunt;
Hypertension, pulmonary;
Angiography, CT;
Liver
- MeSH:
Hypertension, Portal;
Hypertension, Pulmonary*;
Liver;
Liver Diseases;
Portal Vein*;
Portasystemic Shunt, Surgical;
Ultrasonography
- From:Journal of the Korean Radiological Society
2007;57(5):423-428
- CountryRepublic of Korea
- Language:English
-
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.
