Background: Atrioventricular septal defect (AVSD) is a congenital heart disease and usually accompanied by wide range of cardiac and extracardiac congenital anomalies. A concomitant portal vein fibrosis anomaly with AVSD and also suffered from esophageal varices bleeding due to portal hypertension has never been reported.
Case Report: A 15 years old junior high school girl suffered from hematemesis due to variceal bleeding. A history of AVSD during her neonatus period was obtained from her father (a neurologist). The first episode of variceal bleeding was happened at 14 months old three days after AVSD closure surgery and treated with endoscopic variceal sclerotherapy. The variceal bleeding
never recurr until age 15 years old. She has no history of clinical signs of infection during neonatus . Recent liver function test was normal and hepatitis viral serological examinations, HBsAg and Anti-HCV, were negative. Ultrasound study at her teen-age showed normal liver with very small diameter and wall thickening of the portal vein, low portal flow velocity
(9.2 cm/s), splenomegaly and also cavernous transformation of the portal vein. Recent endoscopy examination revealed moderate enlargement of the esophageal varices and endoscopic variceal ligation was performed to prevent further episodes of bleeding. Echocardiography showed normal wall motion and ejection fraction without signs of pulmonary hypertension.
Conclusions: To our knowledge this is the first report of variceal bleeding due to extrahepatic portal hypertension in a patient with portal vein fibrosis and history of atrioventricular septal defect in which these anomalies were concomitantly congenital in origin.

Keywords: Heart Septal Defects, Hypertension, Portal, Portal Vein