Congenital coronary artery anomalies

Last revised by Craig Hacking on 29 Jan 2024

Congenital coronary artery anomalies (CCAAs) are not common, found only in ~1% (range 0.1-2%) of patients 1,3.

The major anomalies are due to abnormal course, abnormal origin or a combination of both.

The most important finding to look for is the "malignant" course of the anomalous coronary artery, i.e. does the artery run between big pulsating objects - right ventricular outflow tract / pulmonary artery on one side and aorta on the other? Increased pulsations during or following exercise can sometimes compress the coronary artery enough to diminish blood flow to the myocardium, which can cause a re-entry phenomenon in the myocardium and sudden cardiac death due to ventricular fibrillation or sustained ventricular tachycardia.

Interarterial "malignant" course is most commonly identified in the right coronary artery, classically presenting in young adults or teenagers with sudden cardiac death. It is estimated that around a quarter of sudden cardiac deaths in this population are caused by malignant coronary artery course.

An intramural course of a coronary artery (known as myocardial bridging) is another lesion which may be hemodynamically significant and present with exertional angina. Usually, it occurs at mid-LAD. It is important to describe the location (LAD segment relative to branch vessels), length, and depth of bridging in your report.

Of course, all anomalous coronary artery anatomy should be described in the radiology report, as this may be used to guide catheter angiography by the interventional cardiologist 7.

Related pathology

A vast number of different coronary artery abnormalities have been described and are discussed separately (see related articles). 

Variant anatomy is described in each of the separate vessel articles and can be group into variants of origin, course, branching, and distribution.

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