Living donor liver transplantation for congenital absence of the portal vein in a child with cardiac failure

Congenital absence of the portal vein (CAPV) requires liver transplantation when encephalopathy develops. However, transplantation has technical difficulties because no collateral circulation exists except for the portosystemic shunt. Ligating the shunt will cause disastrous mesenteric venous congestion. We report a 19-month-old female infant with CAPV, who had portosystemic encephalopathy and cardiac failure, and underwent living donor liver transplantation with a partial clamp technique using a vein graft. This is the first case of successful liver transplantation for CAPV with cardiac failure.