An enormous shunt between the portal and hepatic veins associated with multiple coronary artery fistulas.

A 9-year-old boy with a rare combination of multiple coronary artery fistulas and congenital shunt between the portal and hepatic veins (portal-systemic shunt) is presented. The most likely pathogenesis for the portal-systemic shunt in this case was persistence of the ductus venosus as a bypass tract of the portal vein. This shunt is considered as one cause of cardiomegaly and dilatation of the hepatic vein in this case, and careful follow-up is mandatory because this shunt could induce portal-systemic encephalopathy. There are a few reports of an anomalous portal venous connection to the hepatic vein as a result of abnormal portal embryogenesis. We experienced a unique case of congenital portal-systemic shunt associated with coronary artery fistulas. The present paper reports angiographic findings in this case and the etiological importance of the congenital portal-systemic shunt.