Proposed Standardized Nomenclature for Anomalous Aortic Origin of a Coronary Artery

Increasing use of advanced noninvasive imaging has provided great anatomic and morphologic details to characterize congenital coronary artery anomalies. Anomalous aortic origin of a coronary artery (AAOCA) is a subtype that may lead to myocardial ischemia with variable clinical manifestations across the lifespan. Management decision making in AAOCA is hindered by anatomic variants of differing physiologic consequences. Several systems of nomenclature have been used that lack uniformity in potentially high-risk morphologic features. The ICAAC (International Coronary Artery Anomalies Collaborative), a group of experts in coronary artery anomalies, proposes a standardized nomenclature to describe anatomy and morphologic features of AAOCA using the Delphi methodology. This system is agnostic of risk and intended to unify the descriptors of AAOCA within the scientific community. A common language will allow definition of high-risk morphologic features inducing myocardial ischemia while fostering collaborative efforts to improve risk stratification in AAOCA across ages, ultimately guiding evidence-based management decision making.