A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt

作者：Yi Jeong Eun; Jung Hae Ok^*; Youn Ho Joong; Choi Jong Young; Chun Ho Jong; Lee Jae Young

来源：Journal of Korean Medical Science, 2014, 29(4): 604-608.

DOI：10.3346/jkms.2014.29.4.604

• Congenital Extrahepatic Portocaval Shunt
• Pulmonary Arterial Hypertension
• Heart Septal Defect, Ventricular
• Multifocal Nodular Regenerative Hyperplasia of the Liver

Congenital 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 mu g/day) with improved condition and walking test. Physicians should note that congenital portocaval shunt may cause pulmonary hypertension.

• 出版日期2014-4