Risk stratification and real-world management of pediatric AAORCA: discordance between anatomical features and clinical symptoms

Abstract

Background Anomalous aortic origin of the right coronary artery (AAORCA) is increasingly identified in children, yet risk stratification remains controversial. This study aims to identify key anatomical predictors of clinical symptoms and evaluate real-world management patterns in a large pediatric cohort. Methods We conducted a single-center retrospective cohort study in pediatric patients ≤18 years diagnosed with AAORCA between November 2021 and December 2025. Clinical presentation, imaging findings, management strategies, and follow-up outcomes were analyzed. Integrated anatomical features were derived using a predefined hierarchical approach incorporating surgical findings, CTA, and echocardiography. Multivariable logistic regression analysis was performed to identify factors associated with symptomatic presentation. Results A total of 151 patients were included (mean age 7.40 ± 4.87 years; 60.9% male), of whom 51.7% presented with symptoms. High-risk anatomical features were frequently identified but showed incomplete concordance with clinical symptoms. In multivariable analysis, patient age was independently associated with symptom presentation. Although ostial stenosis showed a stronger unadjusted association with symptoms than other anatomical features, no anatomical variable remained independently associated with symptom status after adjustment. A real-world decision pathway revealed substantial heterogeneity in management strategies among patients with high-risk anatomical findings. No sudden cardiac death or major adverse cardiovascular events were observed during available follow-up. Conclusion Pediatric AAORCA demonstrates marked heterogeneity in clinical presentation, anatomical features, and management strategies. Clinical symptoms show incomplete concordance with high-risk anatomical features, highlighting the complexity of risk stratification in children. Among the evaluated clinical and anatomical characteristics, patient developmental age emerged as a strong independent predictor of symptoms, whereas ostial stenosis demonstrated the strongest unadjusted association with symptom status; however no anatomical variable remained independently associated with symptoms after multivariable adjustment. Real-world management strategies varied substantially and reflected individualized decision-making in the setting of limited pediatric evidence. Future studies may benefit from phenotype-oriented rather than anatomy-only risk classification frameworks.