Coarctation of inferior vena cava in situs inversus totalis

A woman aged 28 years with situs inversus totalis presented with chronic Budd-Chiari syndrome with symptoms of 3 years' duration. The left sided inferior vena cava was occluded below the diaphragm above a patent morphologic right hepatic vein. A dorsal cavoatrial bypass was attempted but abandoned due to bleeding from extensive collaterals. The occurrence of coarctation of the inferior vena cava in situs inversus totalis lends support to the view that it is a congenital condition and occurs due to disturbance in fusion of the hepatic segment of the inferior vena cava and the hepatocardiac channel.