Anomalous Coronary Artery Origins: A Practical Overview for the General Pediatrician

Anomalous aortic origin of a coronary artery (AAOCA) is a rare but potentially life-threatening congenital heart defect, occurring in approximately 0.4–0.8% of the population. While many patients remain asymptomatic, certain variants—particularly anomalous origin of the left coronary artery (AAOLCA) with interarterial or intramural courses—carry an elevated risk of sudden cardiac arrest (SCA), especially during exertion. Primary care providers play a critical role in the early identification and coordination of care for patients with AAOCA. Although routine electrocardiograms are typically normal, exertional chest pain or syncope should prompt referral to pediatric cardiology. Echocardiography is often the first-line diagnostic tool, but further imaging with cardiac computed tomography (CCT) and/or magnetic resonance imaging (MRI) is essential for confirming the diagnosis and risk-stratification. Identifiable high-risk anatomical features include a slit-like ostium, intramural course, and acute angle of take-off. Exercise stress testing is used for risk stratification but has low sensitivity and is recommended to be used with adjunctive testing such as stress perfusion imaging and occasionally cardiac catheterization. Surgical repair is indicated in patients with AAOLCA, symptomatic individuals, or those with demonstrable ischemia. Asymptomatic patients with AAORCA and low-risk features may be followed conservatively. Regardless of surgical status, all patients require lifelong cardiology follow-up, periodic imaging, and individualized sports clearance. Additionally, first-degree relatives may warrant screening due to potential familial clustering. AAOCA impacts quality of life, with patients and families often experiencing emotional distress due to lifestyle restrictions. Primary care providers should monitor for psychosocial concerns and coordinate mental health support as needed. This review aims to equip primary care clinicians with practical knowledge to ensure timely referral, appropriate counseling, and long-term support for children with AAOCA.