Anomalous Left Main Coronary Artery: Case Series of Different Courses and Literature Review

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. 1. Background Coronary artery anomalies remain an important cause of debilitating cardiac symptoms and in some instances sudden cardiac death. Anomalies are most often classified into abnormalities of origin, distribution, and association with fistulae when present. The precise prevalence of coronary artery anomalies is not well defined. A study completed by Yamanaka and Hobbs in 1990 found the overall incidence of coronary artery anomalies in more than 120,000 patients undergoing coronary angiography to be 1.3% [1]. In a retrospective review of the Department of Defense data from 1977 to 2001, nontraumatic sudden death occurred in 126 military recruits out of over 6 million cases reviewed. Of these patients, the cause of death was identified as a coronary artery anomaly in 39 patients with 21 of those having an anomalous coronary artery origin. All 21 of these patients were found to have an anomalous origination of the left coronary artery from