SCA is a rare anomaly and is usually detected incidentally while imaging the heart for other reasons. It can be an isolated anomaly or associated with other congenital cardiac anomalies. Our first case was associated with other congenital cardiac anomalies, whereas the second case showed isolated SCA anomaly. When it is associated with anatomically normal heart, it usually divides into normally formed and distributed coronary arterial branches. But cases associated with other congenital cardiac anomalies have atypical branching patterns [2, 3]. A large study on 126,595 individuals revealed that 0.019% had SCA and 40% of them had associated congenital heart diseases like TOF, truncus arteriosus and transposition of great arteries [2]
In another study, performed over 4,445 patients who underwent coronary CT angiography, 12 patients were diagnosed with a SCA with a prevalence of 0.27%. Of the 12 patients with SCA, only one patient had SCA originating from the left coronary sinus, whereas 11 had SCA originating from the right coronary sinus. Dual LAD variant was identified in four patients. None of the patients had associated congenital cardiac anomalies [9].
Most of the patients with SCA are asymptomatic but some can present with chest pain, myocardial infarction, syncope, ventricular tachycardia and even sudden cardiac death [12].
SCA with an inter-arterial (also called malignant) course has been strongly linked with myocardial ischemia and sudden cardiac death, especially among young athletes. The postulated theories include occlusion due to compression between the aorta and pulmonary artery during physical exertion or presence of a more slit-like orifice which is more prone to get occluded [13, 14].
These patients can also present with symptoms of coronary artery disease. It can be related to increased incidence of atherosclerosis among patients with SCA which can be explained by abnormal origin, long travelling distance, anomalous course and compression between the great vessels which may precipitate endothelial injury and eventually lead to atherosclerosis [15].
Previously conventional angiography was considered the gold standard for evaluation of coronary artery anomalies [7]. With advancements in CT technology, Coronary CTA has emerged as the modality of choice for evaluation of coronary arteries. Due to its high spatial resolution and three-dimensional reconstruction abilities, it provides accurate angiographic information about the origin, course, and termination of coronary anomalies non-invasively [16]. Cardiac magnetic resonance imaging (MRI) is an alternative modality to determine coronary artery anomalies; however, due to low spatial resolution, it is less useful in evaluating the distal coronary system [17].
Having knowledge of the presence of SCA and its course will be helpful in assisting the physician in deciding the treatment strategy and avoiding potential surgical complications. In the majority of asymptomatic individuals without atherosclerotic disease, no invasive intervention is required [12]. Invasive or surgical interventions are recommended for patients with an inter-arterial course of the anomalous artery or symptomatic patients [18]. Surgical procedures include transposition of coronary arteries to appropriate coronary sinus or coronary artery bypass grafting [19]. None of our patient required surgical intervention for SCA although first case got operated for associated cardiac anomalies and preoperative information about pre-pulmonic course of the coronary artery helped the surgeons in deciding the operative plan.
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