Patients with complete atrioventricular canal defect and aortic arch obstruction represent a particular challenge for management. The incidence is rare, so surgical experience is limited. A reasonable treatment option for newborns and young infants with competent atrioventricular valves is the staged approach, with the arch obstruction repaired first, followed at an appropriate interval by repair of the complete atrioventricular canal defect. If there is a significant degree of atrioventricular valve regurgitation, the primary single-stage correction of both aortic arch obstruction and the intracardiac malformation should be undertaken, irrespective of age. It remains to be seen whether this surgical strategy can be adopted for the entire spectrum of atrioventricular canal defect associated with arch obstruction.
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