Young PM, Gerber TC, Williamson EE, et al. Cardiac imaging: part 2, normal, variant, and anomalous configurations of the coronary vasculature. AJR Am J Roentgenol. 2011;197(4):816–826.

Cross-Reference

Cardiac Imaging: The REQUISITES, ed 3, pp 225–228.

Imaging

CT angiography of the coronary arteries shows an anomalous left coronary artery arising from the right sinus of Valsalva (Figs. A–C). The artery courses superiorly through the upper interventricular septum (Fig. B) and emerges to trifurcate into the circumflex, ramus intermedius, and left anterior descending coronary arteries. This patient was unable to undergo stress testing or surgery because of clinical deterioration unrelated to the anomalous coronary artery.

Overview

An ectopic coronary origin is an important anomaly to recognize given the increased risk of sudden cardiac death in certain variants. There are four paths that the anomalous vessel can take to supply its myocardial territory:

1. Retroaortic: This variant is benign and is not associated with an increased risk of sudden cardiac death.

2. Prepulmonic: This is also a benign variant. This vessel has an increased risk of accelerated atherosclerosis and can be injured if the patient undergoes median sternotomy, especially for repair of tetralogy of Fallot.

3. Interarterial: This is the so-called malignant variant in which the artery courses between the aorta and pulmonary artery. Patients are at an increased risk of sudden cardiac death and are usually treated surgically with bypass grafting or reimplantation of the anomalous artery.

4. Septal: This variant is often confused with the interarterial course; however, in the septal path, the artery travels through the myocardium in the proximal interventricular septum. Stress testing helps determine the clinical significance of this lesion. Patients who develop ischemia are treated surgically.