High-risk anomalous aortic origin of a left coronary artery (L-AAOCA) beyond interarterial course: outcomes in a pediatric cohort with L-AAOCA from the noncoronary aortic sinus

Background Anomalous aortic origin of the left coronary artery (L-AAOCA) is a leading cause of sudden cardiac death (SCD) in the young, especially with an interarterial course. L-AAOCA arising from the non-coronary sinus (NS) with a juxtacommissural origin is less well characterized and its risk profile remains incompletely defined. Purpose We aim to report the clinical presentation, evaluation, management and outcomes in L-AAOCA/NS, juxtacommissural in a pediatric cohort. Methods Single institution cohort study of patients <21 years with L-AAOCA/NS, juxtacommissural evaluated following a standardized approach. Coronary anatomy was assessed on retrospective ECG-gated CTA while functional assessment included EST and nuclear stress perfusion imaging (sNPI) or dobutamine stress CMR in patients ≥6 years. Invasive intracoronary flow assessment (iFR, FFR) on cardiac catheterization was performed in selected cases if concerns with symptoms and negative non-invasive studies. For surgical patients, repeat studies were performed at 3 months postoperatively. High-risk features included exertional symptoms, aborted SCD, intramural (IM) course, slit-like ostium, or inducible myocardial ischemia. Results In total, 20 patients (55% male), median age of 10 years (IQR 4.4 - 4.2) presenting with L-AAOCA/NS, juxtacommissural between 12/2012 - 7/2025 were included, compring 15% of all patients presenting with L-AAOCA. Of these, 6/20 (30%) had exertional chest pain or syncope, 1/20 (5%) presented with cardiogenic shock and 1/20 (5%) had aborted SCD (Figure). CTA was performed in 18/20 (90%); 8/20 (40%) had an origin adjacent to L/NS commissure with none having an IM course defined on CTA. EST was performed in 15/20 (75%) patients and was abnormal in 2/15 (13%). Stress perfusion testing was completed in 13/20 (65%) patients and 4/13 (31%) were positive for ischemia. Abnormal iFR/FFR was present in 1 of 2 cases studied. Surgery was indicated and performed in 7/20 (35%) patients, median IM length 2 mm (IQR 1-3): 2/7 (29%) reimplantations, 2/7 (29%) unroofings, 2/7 (29%) osteoplasty, and 1/7 (14%) neo-ostium formation. One patient denied recommended cardiac catheterization, thus recommendation for surgery could not be provided. Post-operative tests indicated resolution of pre-operative inducible ischemia (invasive/non-invasive) in all 5 patients. As of last follow-up (median 3.6 years, IQR 2.3 – 8.1), all patients were alive 20/20 (100%) and all engage in physical activity including recreational 11/20 (55%), school organized 5/20 (25%), and club level 4/20 (20%). Conclusion In this pediatric cohort of L-AAOCA/NS, juxtacommissural, 10% presented with aborted SCD/cardiogenic shock and 1/3 had evidence of inducible ischemia, suggesting a less benign AAOCA subtype in some patients. Following repair, there was resolution of inducible ischemia with excellent post-surgical medium to long-term outcomes with successful return to physical activity.