Cardiac anomalies in the setting of the Abernethy malformation of the portal vein.

We describe a child with tricuspid atresia associated with a porto-systemic shunt and complete absence of the portal vein, the latter known as the Abernethy malformation. As far as we are aware, this association has not previously been reported. We review the various cardiac defects that have been reported in children with the Abernethy malformation, emphasizing the interesting clinical presentations of this rare malformation of the portal venous system.