Congenital Absence of the Portal Vein Presenting as Pulmonary Hypertension

malformation in which intestinal and splenic venous flow bypasses the liver and drains directly into the systemic circulation via a congenital portosystemic shunt. Variable associated anomalies and clinical manifestations caused by the disrupted enterohepatic circulation in CAPV have been reported, including congenital cardiac anomalies, skeletal and visceral anomalies, hepatic neoplasms, liver dysfunction, hemorrhoidal bleeding and portosystemic encephalopathy (1, 2). We describe two cases of CAPV presenting as pulmonary hypertension, in which primary pulmonary hypertension was initially suspected. However, subsequent ultrasonography and CT detected the absence of the portal vein and the presence of a portosystemic shunt. To the best of our knowledge, only three cases of pulmonary hypertension associated with CAPV have been reported since 1974 (3). 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 and that CAPV can be diagnosed with noninvasive imaging techniques such as Doppler ultrasonography or CT angiography.