Anomalous left main coronary artery: case series of different courses and literature review

Abstract

Background. Congenital anomalies of the coronary arteries are a cause of sudden cardiac death. Of the known anatomic variants, anomalous origination of a coronary artery from an opposite sinus of Valsalva (ACAOS) remains the main focus of debate. Case Series. We present three cases, all presenting to our facility within one week's time, of patients with newly discovered anomalous origination of the left coronary artery from the right sinus of Valsalva (L-ACAOS). All patients underwent cardiac computed tomography for evaluation of coronary anatomy along with other forms of functional testing. Despite the high risk nature of two of the anomalies, the patients are being treated medically without recurrence of symptoms. Summary. After review of the literature, we have found that the risk of sudden cardiac death in patients with congenital coronary anomalies, even among variants considered the highest risk, may be overestimated. In addition, the exact prevalence of coronary anomalies in the general population is currently underestimated. A national coronary artery anomaly registry based on cardiac computed tomography and invasive coronary angiography data would be helpful in advancing our understanding of these cardiac peculiarities. The true prevalence of congenital coronary anomalies and overall risk of sudden cardiac death in this population are not well known. Surgical intervention remains the mainstay of therapy in certain patients though recent investigations into the pathophysiology of these abnormalities have shown that the risk of surgery may outweigh the minimal reduction in risk of sudden cardiac death.

Figure 1

Cardiac computed tomography showing anomalous left main off of the right sinus of Valsalva and subsequent intraseptal course.

Figure 2

Left anterior oblique view of left main coronary artery with an anomalous origin off the right sinus of Valsalva with intraseptal course.

Figure 3

Right anterior oblique-cranial view of left main coronary artery with an anomalous origin off the right sinus of Valsalva with interarterial course.

Figure 4

Cardiac computed tomography images of anomalous left main coronary artery off of the right sinus of Valsalva with a course between the pulmonary artery (PA) and aorta (AO).

Figure 5

Three dimensional reconstruction exhibiting the interarterial course of the left main coronary artery as presented in Case 2.

Figure 6

Cardiac computed tomography and three dimensional reconstruction showing an anomalous left main coronary artery (LMCA) off of the right sinus of Valsalva assuming a posterior course around the aorta (AO).