Repair of atrioventricular canal with double-outlet right ventricle, transposition, or truncus arteriosus

Atrioventricular canal and conotruncal anomalies are a heterogeneous group of lesions presenting unique challenges for surgical repair. These are the establishment of unobstructed pathways from left ventricle (LV) to aorta and from right ventricle (RV) to pulmonary artery, closure of the inlet ventricular septal defect (VSD) and atrial septal defect (ASD) ostium primum, and the avoidance of significant left and right atrioventricular valve (AV) regurgitation. Repair of complete atrioventricular canal (CAVC) with tetralogy of Fallot (TOF) has been most commonly achieved, either using a single-patch or a 2-patch technique. In patients with CAVC with double-outlet right ventricle (DORV) with subaortic VSD extension, the 2-patch repair is not unlike that of CAVC with TOF. However, biventricular repair is most challenging in patients with CAVC and complete origin of the aorta from the RV, as in CAVC with DORV and noncommitted VSD and those with CAVC with transposition of the great arteries (TGA) and LVOTO. The technique of VSD translocation allows anatomic biventricular repair for these particularly challenging patients. The arterial switch operation with CAVC repair can be used for patients with CAVC with DORV with subpulmonary VSD extension and CAVC with TGA without left ventricular outflow tract obstruction. Biventricular repair is achievable in most patients with balanced complete atrioventricular canal and conotruncal anomaly. The extreme heterogeneity of CAVC with conotruncal anomalies requires a highly individual approach that is tailored to the specific constellation of lesions in each patient.