Repair of Tetralogy of Fallot with Anomalous Coronary Arteries Coursing Across the Obstructed Right Ventricular Outflow Tract

Anomalous coronary artery (ACA) anatomy occurs in 2–9% of patients with tetralogy of Fallot (TOF), in which the left anterior descending coronary artery (LAD) originates from the right coronary artery (RCA) crossing the right ventricular outflow tract. The purpose of this study was to review our results of repair for TOF with ACA. Between 1978 and 2001, 43 ACA patients (mean age, 4.8 years; range, 5 months–41 years) underwent repair for TC. The ACA anatomy was classified as the single LAD from the RCA (n = 20), a significant conal branch (dual LAD) from the RCA (n = 13), paired anterior descending arteries originating from the left and right coronary arteries (n = 7), and single RCA from the LAD (n = 3). In cases in which the anomalous LAD crossed the obstructed infundibulum, thinning or coring of the endocardium was done. Patch infundibuloplasty was performed in 39 patients, with 10 needing separate RV and pulmonary artery patches, and the pulmonary valve was preserved. Nine patients had the addition of a monocusp to a transannular incision. Two patients had a main pulmonary arterioplasty alone. There was one early (2.3%) and no late deaths. Mean early and late postoperative gradients were 21.5 ± 10.5 mmHg (4 patients had ≥30 mmHg) and 27.1 ± 13.7 mmHg (5 patients had >30 mmHg; p = 0.12), respectively. There were four reoperations during a mean follow-up of 4.8 years (range, 6 months to 18 years). Actuarial freedom from reoperation was 90% at 5, 10, and 15 years. At the latest follow-up, all patients were in NYHA functional class I. TOF repair for an ACA can be performed without disturbing the native coronary anatomy and without the use of conduits in most cases. Outcomes are similar to those of other patients with TOF. The presence of ACA does not impose increased risk after this surgical strategy.