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.