Abstract 15115: Clinical Presentation and Medium-term Outcomes in Anomalous Aortic Origin of the Left Coronary Artery: High-risk Features Beyond Interarterial Course

Abstract

Background: Anomalous aortic origin of the left coronary artery (AAOLCA) confers a rare, but significant, risk of sudden cardiac death (SCD) in children. Surgical intervention is recommended for AAOLCA with an interarterial course, with other subtypes considered benign. We aimed to determine the clinical characteristics and outcomes of AAOLCA in a prospective cohort following a standardized approach. Methods: All patients with AAOLCA <20 years old were prospectively enrolled. Anatomic details were obtained with coronary computerized tomography angiography (CTA). Myocardial functional studies were obtained under provocative stress, including exercise stress test, stress nuclear perfusion imaging, or dobutamine stress cardiac magnetic resonance. Patients were stratified as high-risk if: 1) episode of aborted SCD, 2) interarterial and/or intramural course, or 3) evidence of myocardial ischemia on functional studies. Results: A total of 47 patients (32 male, 68%) with AAOLCA were enrolled, median age 12 [IQR 6.0;15.0] years. Of these, 20 had exertional symptoms (42.5%) and 5 had aborted SCD (10.5%). Thirty-one patients (79%) were classified high-risk, 28% low-risk, and 3 (6%) work-up is ongoing. Of patients with completed work-up, 16/43 (37%) had inducible ischemia and, of these, 2/16 (13%) presented without symptoms and one with aborted SCD as the first symptom. Two patients in our cohort had AAOLCA arising close to the noncoronary sinus, just above the left and noncoronary commissure, resulting in ostial stenosis: one presented with aborted SCD and another with left ventricular dysfunction. To date, 17 patients have undergone repair, median age 10 years [IQR 6.6;15.7]. Median follow-up was 3.9 [IQR 1.3;6.3] years with no significant symptoms and all but 4 patients are cleared from exercise restrictions. Conclusion: AAOLCA may present with diverse anatomic configuration and risk stratification is essential to determine management. AAOLCA from the noncoronary sinus may also present with myocardial ischemia and, thus, be classified as high-risk. A multimodality imaging approach is necessary to adequately risk stratify this population. All patients are alive at last follow-up and the majority free from exercise restriction.