Background: There may be some consensus about the optimal time for total repair in patients with TOF. However, in patients with symptomatic TOF or pulmonary atresia in early infancy, there are controversies on performing early total repair or palliation. We have tried to do one-stage total repair in this subset since May, 2004. We reviewed the outcomes of early surgical interventions and also our strategy. Method: Between Jan. 1997 and Dec. 2006, 80 consecutive patients with TOF (n = 31) or PA (pulmonary atresia) VSD (n = 49) underwent surgical treatment at age of 90 days or less. 42 patients initially underwent shunt operations and 38 underwent one-stage total repair (operation at 38 ± 23 days vs. 44 ± 26 days, respectively). We divided the patients into 2 groups before (group A, n = 55 (38 PA VSD; 17 TOF)) or after (group B, n = 25 (11 PA VSD; 14 TOF)) May, 2004. Results: In group A, only 4 patients (11%) among PA VSD patients and 10 (59%) of TOF patients had one-stage total repair. There were 6 hospital deaths (11%) and 4 deaths (8%) before total repair. In group B, all the patients except one TOF patient underwent one-stage total repair. There were no hospital deaths in this group. In group A, 31 patients (76%) underwent total repair at 17 ± 10 months after initial shunt operations. All 68 survivors undergoing total repair (43 in group A; 25 in group B) were followed up for 51 ± 33 months. There were 3 late deaths (2 in group A; 1 in group B). Catheter and/or surgical interventions after total repair were needed in 33 patients (49%), all of which were related with complicated RVOT. Surgical interventions were required in 10 patients (24%) of group A and 6 (24%) of group B and all the patients except 2 had a PA VSD anatomy. Conclusions: Our strategy of one-stage total repair of TOF or PA VSD in early infancy shows good early and mid-term outcomes. However, many patients, especially with PA VSD require reinterventions after total repair and should be carefully followed up to evaluate RVOT problems.
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