Congenital Coronary Artery Anomalies: Three Cases and Brief Review of the Literature

Abstract

Coronary artery anomalies (CAAs) are congenital vascular defects which can remain hidden and asymptomatic over the complete life course of an individual. They are defined as deviations from the normal coronary anatomy regarding the arterial origin, course, or both. Their incidence varies from 1.3% to 5.64% in coronary angiography cohorts, and they can be detected as incidental findings. In certain cases, CAAs can be hemodynamically significant and unfortunately can be proven lethal. Their link with sudden cardiac death, especially in otherwise healthy competitive athletes, is well established, but their prognostic significance, range of symptoms, and pathophysiology remain to be further elucidated. Here, along with a brief review of related literature, we present a series of three cases: one case of an anomalous origin of the right coronary artery (RCA) from the left coronary sinus, one case of a split RCA originating from the left coronary sinus, and one case of a dual left anterior descending (LAD) artery system.

Figure 1

CT angiography of the aorta revealing an anomalous origin of the RCA (depicted with an “∗”). The RCA exhibits an interarterial course between the aorta and the pulmonary artery (PA).

Figure 2

Coronary angiography confirming the anomalous aortic origin of the RCA from the left coronary sinus. LM: left main.

Figure 3

CT coronary angiography with volume-rendered 3D reconstruction of the images, revealing the interarterial course of the RCA (pointed with the arrow) between the aorta and the pulmonary artery (PA).

Figure 4

(a, b) Coronary angiography (right anterior oblique projection) revealing the split RCA (marked as RCA1 and RCA2) with an anomalous origin from the left coronary sinus. The RCA and LCA were outlined simultaneously using the JL4 catheter placed at the left coronary sinus. LCx: left circumflex artery; OM: obtuse marginal artery; LAD: left anterior descending (artery).

Figure 5

CT angiography of the aorta revealing a short LMCA (a) which then splits into two distinct branches (pointed with arrows of different color) descending toward the apex in the anterior interventricular groove. The short LAD is pointed with blue arrows and the long LAD with red arrows.

Figure 6

(a, b) Coronary angiogram: right anterior oblique caudal projection (a) and left anterior oblique caudal “spider” projection (b). A very short LMCA, best visualized in (b), gives rise to the LCx and a common LAD trunk which we denote as LAD-P. The LAD-P bifurcates into two arteries, S-LAD, giving rise to long septal perforators, and L-LAD reaching the apex, giving rise to shorter perforating arteries and diagonals. LAD-P: left anterior descending proper; L-LAD: long LAD; S-LAD: short LAD; LCx: left circumflex artery; S: septal perforating branches.