Occurence & Clinical Presentation

AAOCA has a low prevalence in the general population, estimated as 0.06-0.9% for anomalous right coronary artery (AAORCA) and 0.025-0.15% for anomalous left coronary artery (AAOLCA). Boys are three times more likely than girls to have AAOCA.

AAOCA from the opposite sinus of Valsalva accounts for 14-17% of all cardiac-related deaths in youth. AAOLCA is considered more lethal than AAORCA, being responsible for up to 85% of SCDs related to AAOCA. Most cardiac events appear to occur in individuals between 10-30 years of age. Yearly occurrence of SCD among young athletes varies widely, according to several reports and depending on the population studies, estimated between 1 in 43,800 and 1 in 200,000. Most SCDs from AAOCA occur during or just after physical exertion.

Sports commonly associated with SCD include the following, among others:

  • Basketball
  • Soccer
  • Track and field
  • Swimming
  • Cross-country running
  • Football


AAOCA is a congenital heart condition. Congenital is defined as present at birth.  As with most congenital heart defects, a specific cause cannot be identified and may be multifactorial.

Clinical Presentation – Signs & Symptoms

About half of patients with AAOCA present without symptoms and the diagnosis is suspected on cardiac imaging (heart ultrasound or echocardiogram) performed for another reason. However, among those who do have signs and symptoms, common ones are:

  • Chest pain during or immediately following exertion
  • Heart palpitations during or immediately following exertion
  • Shortness of breath/difficulty breathing during or immediately following exertion
  • Dizziness during or immediately following exertion
  • Fainting during or immediately following exertion
  • Cardiogenic shock
  • SCA (sudden cardiac arrest)
  • SCD (sudden cardiac death)