Introduction to anomalous aortic origin of a coronary artery

Congenit Heart Dis. 2017 Sep;12(5):600-602. doi: 10.1111/chd.12497. Epub 2017 Jun 27.


Anomalous aortic origin of a coronary artery (AAOCA) occurs when both coronary arteries arise from the same aortic sinus from a single ostium or two separate ostia. While most coronary anomalies are benign, the two most common subtypes that predispose to sudden cardiac death in the young are interarterial anomalous right coronary artery and interarterial anomalous left coronary artery. Practitioners face many challenges with AAOCA. Diagnosing patients may be difficult because children and adolescents are often asymptomatic and first presentation may be sudden death or sudden cardiac arrest. Risk stratification is also challenging as determining which unique characteristics place the child at highest risk of sudden death has not been adequately delineated and ischemic testing may give false negative results. Last, there is significant variability in decision making regarding management of youth with AAOCA. Future research is needed to help determine the best way to identify at-risk children and which treatment is the safest and most efficacious.

Keywords: anomalous coronary artery; congenital heart disease; congenital heart surgery; pediatrics; sudden death.

Publication types

  • Review

MeSH terms

  • Aorta, Thoracic / abnormalities*
  • Aorta, Thoracic / diagnostic imaging
  • Child
  • Coronary Vessel Anomalies* / complications
  • Coronary Vessel Anomalies* / diagnosis
  • Coronary Vessel Anomalies* / epidemiology
  • Coronary Vessels / diagnostic imaging*
  • Death, Sudden, Cardiac / epidemiology*
  • Death, Sudden, Cardiac / etiology
  • Diagnostic Imaging
  • Exercise Test
  • Global Health
  • Humans
  • Incidence
  • Survival Rate / trends