Anomalous aortic origin of a coronary artery: learning from the past to make advances in the future

Purpose of review To review anomalous aortic origin of a coronary artery (AAOCA) anatomy, prevalence, mechanism and risk of ischemia, presentation, evaluation, management, and future directions. Recent findings Although most anatomic variants of AAOCA are benign, a small number are associated with increased risk of sudden death. A complete evaluation, including the use of advanced noninvasive imaging and provocative testing should be performed on nearly every patient with AAOCA. On the basis of recent studies, the ischemic risk appears to be greatest with a left anomalous coronary artery but an anomalous right coronary artery is not benign. Other risk factors include: a left anomalous coronary with an intramural course, high take-off, or slit-like orifice, and a right anomalous coronary with a longer intramural course. Exercise restriction is rarely recommended. Management primarily consists of nonoperative care, or surgical repair in those who are symptomatic or who have high-risk variants. Surgery itself continues to evolve; however, it is not benign, with a higher than expected chance of morbidity. Summary Advances have been made over the past decade regarding management of patients with AAOCA; however, the mechanism of ischemia and ability to predict risk is still incompletely understood. Management decisions should be based on anatomy, results of investigations, and shared decision-making with patients and their families. Surgery may be recommended for those at higher risk and should be done at centers experienced in AAOCA surgery. Future research should be collaborative in order to share experiences and insights to help advance our understanding of risk and ultimately to improve patient management.