Single-center clinical analysis of anomalous aortic origin of a coronary artery (AAOCA) in children: a multimodal imaging approach to risk stratification

This study aimed to analyze the clinical characteristics of pediatric patients with Anomalous Aortic Origin of a Coronary Artery (AAOCA), explore the application of multimodal imaging in risk stratification, and establish individualized diagnosis and treatment protocols for this population. A retrospective observational study was performed on children diagnosed with AAOCA at Wuhan Children’s Hospital between January 2015 and April 2026. Clinical manifestations, multimodal imaging findings, therapeutic strategies, and long-term outcomes were comprehensively analyzed. A total of 58 AAOCA patients confirmed by coronary computed tomography angiography (CCTA) were enrolled. Initial transthoracic echocardiography (TTE) yielded false-negative results in 24 patients (41.4%). Twenty-seven patients underwent single-photon emission computed tomography myocardial perfusion imaging (SPECT-MPI, MPS), among whom 6 had normal perfusion and 21 showed perfusion defects. Seven patients with positive MPS findings received surgical correction, including 2 patients with L-AAOCA( 1 coronary angioplasty and 1 coronary unroofing) and 5 patients with R-AAOCA; all treated with coronary unroofing. No patients with negative MPS results underwent surgery, indicating that negative MPS findings perhaps provide limited valuable evidence to avoid unnecessary coronary revascularization. Two patients developed massive postoperative pericardial effusion and recovered after pericardiotomy and drainage; the remaining 5 surgical patients experienced no perioperative complications. All surgical patients were discharged after full recovery and received 3 months of aspirin antiplatelet therapy. Non-surgical patients were instructed to restrict strenuous physical activity and followed up for 3 months to 3 years. Follow-up CCTA or TTE revealed normal coronary ostia without stenosis in all patients. Clinical manifestations of pediatric AAOCA vary widely across age groups, ranging from asymptomatic presentation to sudden cardiac arrest (SCA), leading to marked discrepancies in risk evaluation and therapeutic decision-making. Multimodal imaging including TTE, CCTA and SPECT-MPI facilitates risk stratification and guides personalized clinical management of children with AAOCA.