Evolution of surgical management for anomalous aortic origin of a coronary artery (AAOCA): broadening the understanding of a complex lesion

Background Anomalous aortic origin of a coronary artery (AAOCA) is a leading cause of sudden cardiac death (SCD) in the young with variable strategies for risk stratification and outcomes. Purpose We report the clinical presentation and surgical outcomes of patients with AAOCA repair at our institution after the inception of our Coronary Artery Anomalies Program (CAAP) following a standardized approach. Methods The medical records of patients who underwent surgical repair of an AAOCA between December 2012 and March 2025 were queried for clinical data, operative details, and postoperative outcomes. All patients followed a standardized evaluation algorithm and the management strategy was discussed in the CAAP multidisciplinary meeting. Descriptive statistics were used to report clinical characteristics and outcomes. Results A total of 113 patients underwent surgical repair of an AAOCA. 51 (45%) presented with exertional chest pain or syncope, 54 (48%) were asymptomatic or had non-exertional symptoms, and 8 (7%) presented with aborted SCD. A total of 60/113 (53%) patients had evidence of ischemia prior to surgery (aborted SCD, ischemic ST/T-wave changes on exercise stress testing, inducible perfusion defects on stress perfusion imaging, severely reduced ventricular function, or impaired intracoronary flow on cardiac catheterization). Surgeries included repair of a right (R)-AAOCA in 70 patients (62%), left (L)-AAOCA in 34 (30%), and intraseptal L-AAOCA (L-AAOCA/IS) in 9 (8%). Surgical procedures included 72 unroofings, 26 translocation/reimplantations, 9 transconal unroofings, 3 ostioplasties, 2 side-to-side anastomoses, and 1 neo-ostium creation. The proportion of patients with objective evidence of ischemia guiding surgical decision-making increased over the study period (Figure 1). Perioperative complications occurred in 10/113 (8.8%) patients, excluding readmissions and patients who were critically ill preoperatively; these included 5 (4.4%) post-pericardiotomy syndrome, 1 (0.9%) pleural effusion, 1 (0.9%) pneumothorax, 1 (0.9%) surgical site infection, 1 (0.9%) anaphylactic reaction, and 1 (0.9%) requiring reoperation for immediate postoperative myocardial ischemia. Over a median follow-up of 4.2 years (IQR 1.7–7.4), re-operations occurred in 4 (3.5%) patients: 2 heart transplantations, 1 reoperation for coronary ischemia requiring CABG, and 1 LCA re-implantation. Freedom from re-operation at 10 years was 95.9%. There was 1 (0.9%) late mortality (11 years after operation), and 110/113 (97.3%) patients have been cleared to unrestricted exercise. Conclusions Surgical management of AAOCA is safe and effective with good medium-to-long-term outcomes and most patients return to full activity. Defining precise morphologic features, ongoing refinement of objective ischemia assessment and multidisciplinary approach has impacted operative planning overtime and is likely to impact long-term outcomes in this complex lesion.