Objective: The purpose of this study was to review our results with an approach of early primary repair for tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries, using several techniques to minimize the use of a conduit.
Methods: Twenty consecutive neonates and infants with anomalous coronary arteries crossing an obstructed right ventricular outflow tract underwent primary repair. Median age was 5.5 months and mean weight 6.22 kg. The anomalous coronary arteries included the left anterior descending from the right coronary artery (n = 10), the right coronary artery from the left anterior descending (n = 1), the left anterior descending from the right sinus (n = 1), and a significant conal branch from the right coronary artery (n = 7) or left anterior descending (n = 1). Two neonates had pulmonary atresia. The right ventricular outflow tract was reconstructed without a conduit in 18 patients, including those with pulmonary atresia. Surgical techniques included main pulmonary artery translocation in 4 patients, transannular repair under a mobilized left anterior descending coronary artery in 2 patients, and displaced ventriculotomy with subcoronary suture lines in 8 patients. In 4 patients the right ventricular outflow tract was repaired via the ventriculotomy and/or pulmonary arteriotomy. A homograft was used as the sole right ventricle-pulmonary artery connection in 1 patient and in another a homograft was added to a hypoplastic native pathway.
Results: There have been no early or late deaths. The right ventricular/left ventricular pressure ratio within 48 hours of the operation was 0.47 +/- 0.10. There were 2 reoperations at 8 and 11 years after the operation, during a mean follow-up of 5.2 years (1-11.3 years).
Conclusions: Primary repair of tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries can be done in neonates and infants with excellent results. Alternative surgical techniques for right ventricular outflow tract reconstruction, such as main pulmonary artery translocation, can avoid the use of a conduit in most patients.