Abstract
Background: Anomalous Aortic Origin of a Coronary Artery (AAOCA) is a rare congenital heart lesion associated with myocardial ischemia and sudden death in the young. Evidence-based criteria for managing young patients with AAOCA are lacking. The CHSS began multi-center enrollment of patients with AAOCA age ≤ 30 years to develop these criteria. We hypothesized that demographics, symptoms and features of coronary morphology would correlate with whether or not the patient had surgery. Methods: To date, we have enrolled 199 patients from 23 CHSS member institutions (median age at diagnosis= 10.4 years; 64% male). Data was extracted from clinical records. Fifty-three percent were symptomatic at presentation (most commonly chest pain, N=78). AAOCA was diagnosed at autopsy in two patients who presented after sudden death (1 with anomalous left coronary artery [ALCA]; 1 with a single ostium above a commissure). Correlation analysis compared demographics, symptoms, coronary anatomy and management. Results: Imaging reports documented anomalous right coronary artery [ARCA] in 142 patients (74%) and ALCA in 51 patients (26%). Surgery was performed in 106 patients (70 ARCA [66%]; 34 ALCA [32%]; 2 ARCA/ALCA [2%]) at a median age of 12.6 years. Overall, 71% of patients with ARCA vs. 51% with ALCA had surgery. Most surgical patients (96/106) had an intramural anomalous coronary artery (69/72 ARCA; 27/36 ALCA). The correlation of diagnostic reports (N=100) with surgical findings was poor (45%). Surgery correlated with symptoms, older age and intramural ALCA (Table). Conclusions: A high index of suspicion for coronary anomalies should be present when a young patient presents with exertional chest pain, presyncope or syncope. Surgical management of these patients is influenced by patient symptoms and coronary morphology. Future analysis of this expanding cohort will lead to evidence-based protocols to manage the spectrum of risk in this heterogeneous population of young patients.
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