Congenital absence of the circumflex artery

Congenital absence of the circumflex artery describes the lack of development of the circumflex artery within the atrioventricular groove.

Epidemiology

Congenital absence of the circumflex artery is a very rare coronary artery anomaly.

Associations

Congenital absence of the circumflex artery is usually associated with a superdominant right coronary artery ^1-4.

Clinical presentation

The condition is considered benign and might be incidentally discovered in imaging studies. However, cases presenting with angina or palpitations have been reported ^1-4.

Complications

The condition can lead to the following conditions ^1-4:

• myocardial ischemia

• arrhythmia

Radiographic features

Congenital absence of the circumflex artery can be depicted with coronary CTA, coronary MRA or invasive coronary angiography by its absence in the left atrioventricular groove and a superdominant right coronary artery supplying the inferolateral wall from the opposite side after crossing the crux of the heart.

Radiology report

The radiology report should include a description of the following features:

• absence of the circumflex artery within the left atrioventricular groove

• a superdominant right coronary artery with its course and branch anatomy

• left main coronary artery origin and variant anatomy

• left anterior descending artery with course branches and segments

• coronary artery disease and stenoses 

Treatment and prognosis

The condition is considered benign and does not require any treatment in the absence of symptoms ^1-4.

Differential diagnosis

Conditions that might mimic congenital absence of the circumflex artery ^1,2:

• hypoplastic circumflex artery

• ostial total occlusion of the circumflex artery