Outcomes of a protocolized approach for surgical unroofing of intramural anomalous aortic origin of coronary artery in children and adults

Objective: Management of anomalous aortic origin of coronary arteries has been variable, and limited data are available on early and late outcomes. Methods: We report a single institution's experience with a protocolized approach to 148 consecutive patients who underwent surgical unroofing of intramural anomalous aortic origin of coronary arteries (June 2003 to December 2020). The management algorithm included preoperative and postoperative cross-sectional and echocardiographic imaging, exercise testing, and a standardized operative technique. Results: The median age of the cohort was 44.4 years (range, 4 months to 83 years); 130 patients had an anomalous right coronary artery, and 19 patients had an anomalous left coronary artery. Surgical unroofing was an isolated procedure in 118 patients (80%) and a concomitant procedure in 30 patients (20.3%). There were 2 (1%) early deaths; both were reoperations for aortic root or valve replacement. Over a median follow-up of 9.5 (interquartile range, 5-12.3) years, there were 5 late deaths, 3 due to noncardiac causes and 2 due to unknown cause. Late survival after anomalous aortic origin of coronary arteries repair at 10 and 15 years was 94.5% and 94.5%, respectively. There were no early or late deaths in the pediatric cohort with a median follow-up of 10.9 years (interquartile range, 6.9-12.1). At a median clinical follow-up of 3.9 years (interquartile range, 1.1-9.5), 36 patients had chest pain but none with evidence of ischemia related to the unroofing. Conclusions: Surgical unroofing of anomalous aortic origin of coronary arteries can be performed safely with low early mortality, even in the setting of concomitant procedures. Late survival is excellent, with the vast majority being symptom free.