Congenital coronary ostial stenosis and coronary atresia are intrinsic congenital coronary artery anomalies in which there is either an intrinsic narrowing or an absent coronary ostium.
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Epidemiology
Coronary ostial stenosis and atresia are reportedly extremely rare conditions 1,2.
Associations
Conditions associated with congenital coronary atresia include the following 1-5:
- other types of congenital heart disease
- supravalvular aortic stenosis
- pulmonary atresia with intact interventricular septum
- hypoplastic coronary disease
Clinical presentation
Patients usually present in infancy and early childhood with cardiovascular symptoms, but clinical presentations in elderly patients have been described 1-3.
Pathology
In the case of atresia, a fibrous connection between the respective coronary sinus and the blind-ending proximal end of the respective vessels can be seen 3. In the case of atresia of the left main coronary artery, a connection of the proximally blind-ending left anterior descending and circumflex arteries is apparently present 1. In addition, the coronary anomaly usually features prominent collateral vessels between the coronary arteries 2,3.
Radiographic features
Coronary atresia is best demonstrated on coronary CTA or coronary MRA. It can be also suspected in invasive coronary angiography (ICA), if no regular ostium and abundant collateral vascularization with retrograde perfusion from the other coronary artery can be found 1-3.
Radiology report
The radiological report should include a description of the following features:
- ostium and origin of the affected coronary artery
- associated coronary artery anomalies
- coronary collaterals
- any associated congenital heart disease
Treatment and prognosis
Coronary atresia of the left main coronary artery carries a poor prognosis and requires surgical intervention. Adults are usually managed with an internal mammary artery bypass graft, whereas children might profit from an ascending aorta baffle 1.
Differential diagnosis
Conditions that might mimic coronary atresia include the following 2,3:
- anomalous aortic origin of coronary artery
- single coronary artery
- anomalous left coronary artery off the pulmonary artery (ALCAPA)
- absent left main coronary artery (separate origin of LAD and Cx)
- postoperative stenosis/occlusion (e.g. after arterial switch for transposition of the great arteries)
- hypoplastic right coronary artery
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