Congenital atresia of the right coronary artery ostium
The patient presented with mild atypical chest pain at exertion with equivocal stress ECG findings.
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VR reconstruction of the coronary arteries demonstrated an absent right coronary artery ostium with normal left main origin giving rise to a normal left anterior descending and large sized dominant left circumflex, which extends to the under surface of the heart to give rise to the posterior descending and then continue crossing to the right side reaching the right AV groove to supply the right coronary artery through intercoronary communications (coronary cascades), which demonstrates normal caliber with no focal or diffuse narrow segments at the anastomosis site. No other clear collaterals formations seen supplying the right coronary artery on the current exam.
Congenital atresia of the right coronary artery ostium is exceedingly rare 1. Differentiation between congenital RCA ostial atresia and acquired ostial or proximal RCA occlusion is important. There are a few features that are in favour of a congenital etiology with the most important being the presence of only 1-2 collateral vessels supplying the distal RCA, which demonstrate normal arterial caliber at the anastomosis site. While acquired ostial occlusion will usually demonstrate a dense network of collaterals with narrow caliber at the anastomosis site with RCA 1.
The other important feature which favours the congenital atresia is the absence of significant clinical complaints and absent myocardial scarring along the RCA territories. In acquired ostial occlusion clinical complaints are present, and myocardial scarring secondary to myocardial infarcts is usually present 1.
There is a possibility of this being a case of single coronary artery, i.e. RCA arising from LCX is still possible. Single coronary artery with origin of RCA from LCx has been also reported with very rare incidence of 0–0.035% 2.
- 1. Angelini P. Congenital coronary artery ostial disease: aspectrum of anatomic variants with different pathophysiologies and prognoses. Tex Heart Inst J. 2012;39:55–59.
- 2. Yamanaka O, Hobbs R. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Catheter Cardiovasc Diagn. 1990;21:28–40