Abstract
Congenital extrahepatic portosystemic shunts (ECPSS) are rare developmental anomalies in which a variable portion of the portal blood bypasses the liver and is shunted in the systemic circulation via one or more aberrant vessels. We present a clinical case of a 70-year-old man, who was referred to the Cardiology Department because of exertional dyspnea, fatigue, and feeling of heaviness and pressure behind the sternum. MDCT of the aorta was performed and an aberrant vessel was discovered with communication with the left iliac vein on one side and superior mesenteric and splenic veins on the other. The portal vein was hypoplastic. The radiologic findings were suggestive of malformation of Abernethy. The ECPSS can be classified into 2 main groups (with complete and partial shunting). The patients have different clinical presentation. Some of them are completely asymptomatic while in others the shunt can manifest even before birth as fetal growth retardation or in the early neonatal period with neonatal cholestasis and galactosemia. Common complications are hepatic encephalopathy and hepatopulmonary syndrome and there is a wide variety of concomitant abnormalities. The imaging modalities play a crucial role in the diagnosis, classification, follow-up and the proper choice of therapeutic management in patients with ECPSS.