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.
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Terminology
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.
Epidemiology
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.
Associations
Anomalous aortic origin of a coronary artery has been associated with the following conditions 1,3:
Clinical presentation
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.
Complications
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:
ventricular arrhythmias
Pathology
Subtypes
Anomalous aortic origins of coronary arteries can be subdivided according to the origin and the affected coronary artery and its course:
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anomalous coronary artery
left main coronary artery arising from the right coronary sinus
left anterior descending artery arising from the right coronary sinus
circumflex artery arising from the right coronary sinus
right coronary artery arising from the left coronary sinus
left or right coronary artery arising from the non-coronary sinus
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origin
separate ostia (remote, adjacent)
single ostium (bifurcation within/outside aortic wall)
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course
intramural/interarterial
retrocardiac
wrapping around the apex
Radiographic features
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.
Coronary angiography
Invasive coronary angiography (ICA) may particularly aid if coronary stenosis is suspected or intravascular ultrasound (IVUS) or a flow quantification is needed 1.
CT
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.
MRI
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.
Nuclear medicine
A nuclear stress test might be performed for the assessment of myocardial ischemia 1.
Radiology report
The radiological report should include a description of the following features 1-6:
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ostium and origin of the anomalous coronary artery
slit-like, fish-mouth-shaped orifice
acute angle takeoff
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the course of the anomalous coronary artery
intramural/interarterial (long/short intramural segment)
transseptal/subpulmonary
other associated coronary artery anomalies (e.g. coronary hypoplasia, coronary atresia)
coronary artery disease and stenoses
any associated congenital heart disease
MRI
In addition to the above features:
regional wall-motion abnormalities
Treatment and prognosis
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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