Serial Follow-Up of Two Surgical Strategies for the Repair of Tetralogy of Fallot.

Adverse events following tetralogy of Fallot repair include arrhythmia, reoperation, and death. While limiting the right ventriculotomy mitigates these events over the short and intermediate term, the impact over the long term is unknown. Children requiring tetralogy of Fallot repair were divided into 2 groups: extended right ventriculotomy vs limited right ventriculotomy with transatrial ventricular septal defect closure. Follow-up office ECGs and Holter monitoring, exercise stress tests, and echocardiograms were obtained in the first, second, and third postoperative decades. The primary outcome was cumulative events defined as reoperation, arrhythmia, or death. The extended and limited right ventriculotomy techniques were used in 21 and 17 children, respectively. Cumulative survival was 93.6% at 30 years and was not different between groups. While 10-year (42.9% vs 5.9%, P = 0.02) event rates favored the limited ventriculotomy technique, there were no significant differences at 20 or 25 years. Cox proportional hazard analysis demonstrated that the limited ventriculotomy technique was independently associated with lower events at 10 years (hazard ratio 0.03, 95% confidence interval 0.0016, 0.5697, P = 0.01). The limited ventriculotomy group had greater exercise capacity in the second decade but not the third. Right ventricular end-diastolic diameter Z score was not different at 10 or 20 years, but was significantly smaller in the limited ventriculotomy group at 30 years (5.55 ± 1.69 vs 4.14 ± 0.63, P = 0.03). Limiting the right ventriculotomy during tetralogy of Fallot repair limits 10-year events, improves exercise capacity at 20 years, and attenuates late right ventricular dilation.