Intrahepatic portal venous shunt
Patient with severe cardiac disease presents with right upper quadrant pain and abnormal liver function
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Abnormal communication between the right portal vein and middle hepatic vein via a bulbous vascular structure (arrows). Distended IVC due to underlying cardiac disease. The patient's symptoms are probably due to hepatic congestion (secondary to right heart strain).
These intrahepatic shunts are rare in the absence of previous hepatic biopsy or underlying cirrhosis. The origin of the shunts is uncertain and presumed to be spontaneous or congenital i.e.persistent embryonic venous anastomoses caused by the failed regression of connections in tributaries of the vitelline vein (precursor of the portal and hepatic veins and portions of the inferior vena cava). The other possibility is rupture of a spontaneous portal vein aneurysm into a hepatic vein. That aetiology is suggested by the appearances in this case.