Coronary Artery Anomalies

Circulation. 2021 Sep 21;144(12):983-996. doi: 10.1161/CIRCULATIONAHA.121.055347. Epub 2021 Sep 20.


Coronary artery anomalies (CAAs) are a group of congenital conditions characterized by abnormal origin or course of any of the 3 main epicardial coronary arteries. Although CAAs have been identified as a common underlying condition in young athletes with sudden cardiac death, the widespread use of invasive and noninvasive coronary imaging has led to increased recognition of CAAs among adults. CAAS are often discovered as an incidental finding during the diagnostic workup for ischemic heart disease. The clinical correlates and prognostic implication of CAAs remain poorly understood in this context, and guideline-recommended therapeutic choices are supported by a low level of scientific evidence. Several studies have examined whether assessment of CAA-related myocardial ischemia can improve risk stratification in these patients, suggesting that multimodality imaging and functional tests may be key in the management of CAAs. The aim of this review is to outline definitions, classification, and epidemiology of the most relevant CAAs, highlighting recent advances and the potential impact of multimodality evaluation, and to discuss current therapeutic opportunities.

Keywords: coronary vessels; death, sudden, cardiac; heart defects, congenital; myocardial ischemia.

Publication types

  • Review

MeSH terms

  • Coronary Artery Disease / diagnostic imaging*
  • Coronary Artery Disease / etiology
  • Coronary Artery Disease / physiopathology
  • Coronary Vessel Anomalies / complications
  • Coronary Vessel Anomalies / diagnostic imaging*
  • Coronary Vessel Anomalies / physiopathology
  • Coronary Vessels / diagnostic imaging*
  • Coronary Vessels / physiopathology
  • Echocardiography / methods
  • Heart Defects, Congenital / diagnostic imaging
  • Heart Defects, Congenital / physiopathology
  • Humans