Echocardiography and Coronary CT Angiography Imaging of Variations in Coronary Anatomy and Coronary Abnormalities in Athletic Children: Detection of Coronary Abnormalities that Create a Risk for Sudden Death

Abstract

In a recent publication, Maron et al. reported the causes of sudden death in athletes from data collected in the US National Registry of Sudden Death in Athletes at the Minneapolis Heart Institute Registry. It is not surprising that in this study, cardiovascular disease is reported as the most common cause of sudden death in athletes (56%). The most frequently encountered cardiac pathology was hypertrophic cardiomyopathy (36% of the population who died of cardiac disease). Coronary artery anomalies of wrong sinus origin were next in frequency (17%). Less common causes attributed to coronary pathology were Kawasaki disease, origin of the left coronary artery from the pulmonary artery, atherosclerotic coronary artery disease, and myocardial bridging of the left anterior descending artery. Echocardiographic imaging along with color and pulsed-wave Doppler has been widely employed to visualize the anatomy and function of the heart and proves to be a valuable instrument in the identification of coronary artery abnormalities. Moreover, coronary CT angiography provides additional confirmatory information. This article will discuss the scope, importance, and implications of echocardiographic and coronary CT angiography imaging of the major coronary anomalies and abnormalities in young athletes who are at risk of sudden death and who otherwise have a structurally normal heart.

Computed Tomography Imaging in Patients with Congenital Heart Disease Part I: Rationale and Utility. An Expert Consensus Document of the Society of Cardiovascular Computed Tomography (SCCT)

“…118-120 Coronary CTA in patients of all ages with coronary anomalies is well described and has superior accuracy for this indication because of the ability to simultaneously visualize the coronary arteries as well as the great vessels. 117, 119,[121][122][123][124][125][126][127] CMR has been shown to be useful for congenital coronary anomalies in older children and adolescents, but is less useful in the youngest patients because image quality is 108,109 Thoracic Vasculature Abnormalities (Pulmonary/systemic venous abnormalities,…”

The Management of Athletes with Congenital Heart Disease

“…The changes in the coronary arteries vary from transient dilation/ectasia to substantial enlargement with aneurysm formation and later with areas of stenosis, leading to chest pain (angina), myocardial ischemia/infarction, ischemic cardiomyopathy, and sudden death. [52][53][54] Here once again is the importance of the increased awareness to not only the occurrence of symptoms during exertion but also the past medical history of the athletes. Even those patients with no coronary artery involvement demonstrated in the acute phase may experience endothelial dysfunction (the inner layer of the vessel wall) and may be at increased risk of development of premature coronary artery disease in the future.…”

Coronary Artery Imaging in Children

“…Transthoracic 2-dimensional echocardiography along with color and pulsed-wave Doppler is widely used as the primary imaging modality of coronary arteries in children (5). However, echocardiography is often limited by operator dependency, poor acoustic window especially in older or postoperative children, and intrinsic diagnostic pitfalls (Fig.…”

“…1). For instance, an echo free space between the left atrial appendage and periaortic tissue may give a false impression of the left coronary artery normally arising from the left aortic sinus of Valsalva in children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) (5). As a result, confirmatory imaging, such as CT or MRI, should be performed if echocardiographic findings are equivocal or negative in patients with high clinical suspicion of coronary artery abnormalities.…”

“…ALCAPA usually presents with congestive heart failure in infants and should be distinguished from dilated cardiomyopathy at this age (30). Because echocardiographic findings may be often inconclusive (5), CT or MRI can be used for identifying ALCAPA and the extent of myocardial infarction (Fig. 1).…”