Long-term Outcomes of Transatrial-Transpulmonary Repair of Tetralogy of Fallot With Anomalous Coronary Arteries

Repair of tetralogy of Fallot (TOF) can be complicated by the presence of an anomalous coronary artery (ACA) crossing the right ventricular outflow tract (RVOT). This study sought to evaluate the late outcomes of a policy of transatrial-transpulmonary repair for this condition. The transatrial-transpulmonary approach was used in 864 consecutive TOF repairs between 1993 and 2018 at a single institution, of which 55 (6%) patients had an ACA. Nineteen (35%,19/55) patients underwent prior palliation. Late survival and freedom from reoperations were compared with the general cohort of 809 patients who underwent complete repair during the same period. Early mortality was 2% (1/55). Median follow-up was 15.6 years. Late mortality was 6% (3/54). Absence of a preoperative diagnosis of ACA was not a risk factor for worse outcomes in terms of late re-interventions, acute coronary syndrome, residual RVOT gradient, and late mortality. Survival was 91% (95% confidence interval [CI]: 77–96%) at 20 years and was comparable to the general TOF cohort (95%, 95% CI: 90–98%, P = 0.12). Actuarial freedom from any re-intervention was 46% (95% CI: 27–62%) at 20 years, which was also comparable to the general cohort (31%, 95% CI: 20–42%, P = 0.19). The presence of an ACA does not appear to affect late survival or re-intervention rates in patients undergoing transatrial-transpulmonary repair of TOF.