Conclusions
Based on the information available to us from the literature, and including our own experience, we are convinced that multimodality imaging is now capable of characterizing and delineating the precise anatomical details of the two sub-sets of individuals born with isomeric atrial appendages. These investigations should now permit the mandatory assessment of the pulmonary venous return required in the setting of right isomerism to unmask concealed pulmonary venous obstruction. In those with left isomerism, such investigations should now delineate the patterns of drainage of the inferior caval and hepatic venous systems. Given the ongoing high mortalities of those requiring a combination of shunting procedures, or functionally univentricular repair, it seems prudent to reassess the conventional strategies for management. In particular, the option of cardiac transplantation should now be systematically evaluated as an alternative pathway of management for those deemed to be at high risk.