Anomalous aortic origin of coronary artery

Last revised by Arlene Campos on 28 Aug 2024

Anomalous aortic origin of coronary artery (AAOCA) refers to a congenital coronary artery anomaly in which a coronary artery arises from a different coronary sinus.

Anomalous origin of the coronary artery arising from the opposite sinus (ACAOS) is a narrower definition and refers to either the left coronary artery arising from the right coronary sinus or vice versa.

Anomalous aortic origin of the right coronary artery is more common than a left coronary artery arising from the right coronary cusp, but the latter is more dangerous 1,2. The prevalence of an anomalous aortic origin of the left coronary artery has been estimated up to 0.15% and it is a cause of sudden cardiac death especially in patients <35 years of age 1-4.

Anomalous aortic origin of a coronary artery has been associated with the following conditions 1,3:

An anomalous aortic origin of the coronary artery can present with angina, palpitations, dyspnea dizziness, syncope or might be found incidentally, on invasive coronary angiography (ICA),  coronary CTA or coronary MRA. In particular, an anomalous aortic origin of the left coronary artery arising from the right coronary is more commonly associated with cardiovascular symptoms and might present with sudden cardiac death usually closely associated with exercise 1-5.

The most dangerous and rightly feared complication of an anomalous aortic origin of a coronary artery is sudden cardiac death. Other complications include 1,3-5:

Anomalous aortic origins of coronary arteries can be subdivided according to the origin and the affected coronary artery and its course:

An evaluation of the proximal course and the relationship to the aortopulmonary root-anatomy of an anomalous aortic origin of a coronary artery is recommended and can be done with coronary MRA, invasive coronary angiography (ICA) or preferably by coronary CTA 1-5.

Stress-testing methods do not seem to significantly correlate with the prognosis and clinical symptoms in case of an anomalous aortic origin of the left coronary artery 1,3 and are not helpful on vasodilator stress 3. Nevertheless, imaging evaluation of physically stress-induced myocardial ischemia has been recommended for clinical decision-making 2.

Invasive coronary angiography (ICA) may particularly aid if coronary stenosis is suspected or intravascular ultrasound (IVUS) or a flow quantification is needed 1.

Coronary CTA is the preferential method for visualization and description of the origin and course of coronary arteries as well as the relationship of the anomalous vessel to the other cardiac structures.

Coronary MRA can be alternatively used for the depiction of origin and proximal course and its relationship to the aortic root and the main pulmonary artery 1 and has been proposed for screening athletes and military recruits due to the lack of radiation exposure 3. It can be conducted with respiratory-gated 3D imaging (e.g. native 3D-SSFP wholeheart and/or 3D-mDixon after gadolinium contrast).

In addition, this might be combined with perfusion imaging and myocardial viability protocol 1.

Vasodilator stress testing (e.g. with adenosine) does not provoke myocardial ischemia in this situation.

A nuclear stress test might be performed for the assessment of myocardial ischemia 1.

The radiological report should include a description of the following features 1-6:

In addition to the above features:

Patient management is still controversial, with limited evidence-base 9, and depends on symptoms, the affected coronary artery and the anomalous course. Prepulmonic and retroaortic courses are considered benign and not of hemodynamic relevance.  An interarterial and especially an intramural course is considered malignant and is associated with an increased risk of sudden cardiac death 1-3,6.

Recommendations have been given by the American Heart Association (AHA), the American College of Cardiology (ACC) and the European Society of Cardiology (ESC) in which surgery has been recommended in the setting of myocardial ischemia attributable to an anomalous aortic origin of either left or right coronary artery (AAOCA) 1,2.

Surgery has been felt as reasonable in the case of ventricular arrhythmias in any type of anomalous aortic origin of coronary artery 1. In older patients with anomalous aortic origin of the left coronary artery from the right coronary sinus, detailed anatomical, dynamic and functional assessment can help direct patients either to surgery or to medical management 9.

Surgery is not recommended in patients with an anomalous aortic origin of the right coronary artery and no signs of myocardial ischemia 2.

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