Anatomically corrective repair of complete atrioventricular septal defectsand major cardiac anomalies

Background. Although satisfactory results of corrective surgery for atriove ntricular septal defects (AVSDs) with complex lesions such as double-outlet right ventricle (DORV) or atrial isomerism have been reported in recent ye ars, the optimal surgical options for isomerism hearts is still a controver sial issue. Methods. We performed anatomically corrective repair on 13 children with ba lanced forms of complete AVSDs and associated major cardiac anomalies. Eigh t of the 13 patients had atrial isomerism (right in 1, left in 7), 5 of who m had DORV. Four others had DORV with trisomy 21, and 1 had tetralogy of Fa llot. Atrial septation for isomerism with the placement of an additional pr osthesis was performed on 4 patients. Results. Two hospital deaths and one late death occurred only in the isomer ism group. Three reoperations were required solely in the left isomerism gr oup: one replacement of the valved external conduit concomitantly with reco nstruction of the left ventricular outflow obstruction, one mitral valve re placement 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 contr oversial issue. (C) 2001 by The Society of Thoracic Surgeons.