폐동맥 고혈압으로 발현한 선천성 간문맥 형성부전의 증례 보고 = Congenital Absence of the Portal Vein Presenting as Pulmonary Hypertension

다국어 초록 (Multilingual 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.

국문 초록 (Abstract)

선천성 간문맥형성부전은 문맥혈류가 간을 우회하여 문맥-체정맥 단락을통해 대정맥으로 직접 배출되는 드문 기형이다. 저자들은 일차성 폐동맥고혈압으로 진단되었다가 초음파 및 전산화단층촬영을 통해 문맥의 형성부전 및 선천성 문맥-체정맥 단락을 비침습적으로 발견해 냄으로써, 이로 인한 이차성 폐동맥 고혈압이 발생하였음을 밝힐 수 있었던 증례를 2예 보고하고자 한다.

참고문헌 (Reference)

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