• J. Korean Med. Sci. · Apr 2014

    Case Reports

    A case of pulmonary arterial hypertension associated with congenital extrahepatic portocaval shunt.

    • Jeong-Eun Yi, Hae-Ok Jung, Ho-Joong Youn, Jong Young Choi, Ho Jong Chun, and Jae Young Lee.
    • Division of Cardiology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
    • J. Korean Med. Sci. 2014 Apr 1; 29 (4): 604-8.

    AbstractCongenital extrahepatic portocaval shunt (CEPS) is a rare anomaly of the mesenteric vasculature in which the intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava, the left hepatic vein or the left renal vein. This uncommon disease is frequently associated with other malformations and mainly affects females. Here we report a case of pulmonary arterial hypertension associated with CEPS (Abernethy type 1b shunt) in a 20-yr-old man who was incidentally diagnosed during evaluation of multiple nodules of the liver. The patient was treated by inhalation of iloprost (40 µg/day) with improved condition and walking test. Physicians should note that congenital portocaval shunt may cause pulmonary hypertension.

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