Long-Term Outcome of Patients With Complete Atrioventricular Septal Defect Combined With the Tetralogy of Fallot: Staged Repair Is Not Inferior to Primary Repair

Background: Primary repair is the preferred strategy for surgical treatment of complete atrioventricular septal defect combined with the tetralogy of Fallot. However, a staged approach may be preferable for very small or cyanotic infants. The long-term outcomes of infants undergoing staged vs primary repair were compared.

Methods: Data from 47 patients with complete atrioventricular septal defect combined with the tetralogy of Fallot who were operated on at our institution between 1974 and 2013 were reviewed. The study end points were all-cause death and reoperation. The patients were classified into two groups: staged repair (SR) and primary repair (PR). The indications for staged repair were cyanosis and young age.

Results: There were 22 SR and 25 PR patients. The SR patients were younger at the time of the initial operation (p = 0.001), and were more frequently cyanotic (21 SR vs 5 PR patients, p = 0.003). The 10-year survival after repair of the SR and PR patients was 78% ± 11% and 83% ± 8%, respectively (p = 0.8). No risk factors for death were identified. The 10-year freedom from reoperation for atrioventricular valve regurgitation of SR and PR patients after repair was 73% ± 12% and 71% ± 10%, respectively (p = 0.5). At least moderate atrioventricular valve regurgitation before repair was the only risk factor for reoperation (p = 0.01).

Conclusions: Cyanotic and very young children with complete atrioventricular septal defect combined with the tetralogy of Fallot who require urgent treatment have long-term outcomes after staged repair similar to those of patients who undergo primary repair. Preoperative atrioventricular valve regurgitation is associated with increased risk for reoperation over the long-term.