Anomalous aortic origin of the coronary artery (AAOCA) with an inter-arterial±intra-mural course is rare anomaly that carries a high risk of ischemic event and even sudden death. Unroofing of the intramural course had been adopted as the gold standard. However, there are anatomical forms where the alternative techniques require to be considered.

We reviewed our cohort between 1999 and 2019. Results 38 patients underwent surgical intervention. Median age was (Mean age was 42.5±2.7 years). Twenty-eight patients (74%) had right AAOCA, 9(23%) left AAOCA and one (3.2%) both left and right AAOCA. One patient had a left coronary artery coursing within the subpulmonary muscular conus. Twenty-nine (76%) patients presented with symptoms, 9 (23%) patients were asymptomatic. Symptoms were chest pain for 24 patients, syncope or lipothymia for 15 patients, 1 patient had a cardiac arrest during exercise and for 8 patients the diagnosis was made by chance. Four patients had a positive ischemic test. Repair techniques included 27 unroofing procedures (67.7%) with an associated translocation of the pulmonary artery for 10 patients in early experience. For 6 patients the unroofing was not-feasible because of the absence of an intra-mural distinct segment or was intraoperatively judged not inappropriate and had a reimplantation of the AAOCA. Four patients had a coronary artery bypass grafting (CABG) and three translocation of the pulmonary artery. There was no early or late mortality. CT scan was performed in 31 cases showed a patent coronary artery ostium. Seventeen patients underwent postoperative ischemia testing with no evidence of ischemia.

Surgical correction in AAOCA is mandatory for symptomatic and asymptomatic patients with evidence of myocardial ischemia under stress or restricted coronary artery segment. Surgical unroofing remains the gold standard but is not appropriated in all particular forms: alternative techniques should be considered in the latter. Surgical results are promising.