Clinical Presentation and Medium-Term Outcomes in Anomalous Aortic Origin of a Coronary Artery with High Take-Off

Introduction: Anomalous aortic origin of a coronary artery (AAOCA) confers risk of sudden death in the young. Paucity of data exists in AAOCA with high take-off (ht-AAOCA) from the aorta. Hypothesis: Patients with ht-AAOCA may be at risk of inducible myocardial ischemia and surgery might be indicated in a small subset with good medium-term outcomes. Methods: Patients with ht-AAOCA, defined as origin at the sinotubular junction and above by CTA, <25 years old were prospectively enrolled. Myocardial functional studies were obtained under provocative stress: exercise stress test, stress nuclear perfusion imaging, dobutamine stress cardiac MRI, or cardiac catherization (in select patients). These studies were repeated 3 months post-operatively (post-op) in surgical patients. Results: A total of 71 patients (45 male, 63%) with ht-AAOCA were enrolled, median age 15 [IQR 12; 19] years. ht-AAOCA of the right (ht-AAORCA) was seen in 66/71 patients (93%): 15 (23%) had exertional symptoms, 13 (20%) had nonexertional symptoms, 14 (21%) had both, 24 (37%) were asymptomatic. Ht-AAOCA of the left (ht-AAOLCA) was seen in 5/71 patients (7%), only 1 (20%) had exertional symptoms. In ht-AAORCA patients, 50/66 (76%) had abnormal ostia and 6/66 (9%) juxtacommissural origin, as compared to 3/5 (60%) and 2/5 (40%), respectively, in ht-AAOLCA patients. Average intramural course in ht-AAORCA was 4.46 mm and 2.36 mm in ht-AAOLCA. Abnormal functional testing corresponding to the affected territory was seen in 11/71 (15%) ht-AAOCA patients. Surgery was recommended for 18/71 (25%) patients due to concerning symptoms, coronary anatomy, or abnormal functional testing. Of these, 13/18 (72%) underwent coronary artery unroofing (in 12) or reimplantation (in 1), 3 declined surgery, 2 pending decision. All surgical patients have returned to unrestricted exercise after reassuring post-op studies, but 1/18 (6%) awaits completion. At median follow up of 1.8 years (range 0.1-8.9), all patients are alive and well, 93% are exercising with no restrictions. Conclusion: Although ht-AAOCA appears benign in most patients, 15% shows inducible myocardial ischemia. Surgery may be indicated in a subset of patients, with overall excellent medium-term outcomes and freedom from exercise restrictions.