Background: Although satisfactory results of corrective surgery for atrioventricular septal defects (AVSDs) with complex lesions such as double-outlet right ventricle (DORV) or atrial isomerism have been reported in recent years, the optimal surgical options for isomerism hearts is still a controversial issue.
Methods: We performed anatomically corrective repair on 13 children with balanced forms of complete AVSDs and associated major cardiac anomalies. Eight of the 13 patients had atrial isomerism (right in 1, left in 7), 5 of whom had DORV. Four others had DORV with trisomy 21, and 1 had tetralogy of Fallot. Atrial septation for isomerism with the placement of an additional prosthesis was performed on 4 patients.
Results: Two hospital deaths and one late death occurred only in the isomerism group. Three reoperations were required solely in the left isomerism group: one replacement of the valved external conduit concomitantly with reconstruction of the left ventricular outflow obstruction, one mitral valve replacement for severe regurgitation of left atrioventricular valve, and one relief of progressing left ventricular outflow obstruction.
Conclusions: The results of anatomically corrective surgery for AVSDs with major associated cardiac anomalies in the nonisomerism group were excellent. The optimal surgical options for isomerism heart, however, remain a controversial issue.