Controversies Surrounding Coronary Arteries Anomalies in Young Athletes

Coronary artery anomalies (CAAs) include congenital or acquired anomalies that may affect young athletes. Examples of congenital anomalies include anomalous aortic origin of a coronary artery (AAOCA) and anomalous origin of a coronary artery from the pulmonary artery. The main example of acquired CAAs affecting young athletes today is Kawasaki disease, an acquired inflammatory process that targets small vessels, particularly the coronary circulation. For the purpose of this discussion, the focus will be on AAOCA, reportedly the second most common cause of sudden cardiac death (SCD) in young athletes.

The occurrence of SCD generates extreme anxiety in schools, sports organizations, and communities at large, causing it to become a greater societal burden.1 Several factors of AAOCA are unknown, including the exact prevalence, the pathophysiological mechanisms leading to SCD, the actual risk of death for the different types of anatomy, the optimal way to evaluate these patients, and whether or not any treatment strategies decrease the risk of SCD in such patients are unknown. Most of the deaths associated with AAOCA occur unexpectedly in healthy children or young athletes, and may occur immediately after exercise2,3,4 or at rest. An increasing number of children and young adults have been found to have AAOCA on imaging studies performed as part of pre-participation screening or for other incidental reasons, such as the presence of a murmur or an "abnormal" electrocardiogram. The estimated prevalence of AAOCA varies according to the source of data, ranging from 0.06%-0.9% for anomalous right coronary artery (ARCA), to 0.025%-0.15% for anomalous left coronary artery (ALCA).6,9 Despite the available evidence suggesting that ARCA is approximately six times more prevalent than ALCA, the latter seems to be responsible for up to 85% of SCDs related to AAOCA3,4,10 and is thus considered a more lethal anomaly.

The precise mechanism leading to SCD in this condition is yet to be defined, although it is postulated that occlusion or compression of the anomalous vessel during exercise leads to myocardial ischemia and subsequent lethal ventricular arrhythmia (ventricular tachycardia and fibrillation). Morphologic factors that are believed to account for this include abnormal ostium (stenotic, slit-like) of the anomalous coronary artery, a vessel course between the aorta and the pulmonary artery (interarterial), and a vessel course inside the aortic wall (intramural). Clinical presentations are variable, but the first manifestation of AAOCA is often a sudden cardiac event. Why an athlete can exercise intensely for several years without symptoms until the sentinel event occurs remains unknown. Other clinical manifestations that are present in approximately half of patients include the occurrence of chest pain, syncope or near syncope, dizziness, and/or palpitations during exertion.3,4,11 Current reports indicate that the risk of SCD is higher during childhood and early adulthood, with most reported events occurring in patients between 10 and 30 years of age,10,12 abating significantly thereafter. Anecdotal factors have been postulated for such phenomena, pathophysiologic mechanisms, and thus risk stratification,are still lacking in patients diagnosed with AAOCA.

Optimal evaluation of patients with AAOCA is not established but typically includes assessment of exercise performance (stress test), often with myocardial perfusion and tomographic imaging with computerized tomography (CT) or magnetic resonance imaging (MRI). Except in some select cases, cardiac catheterization is rarely utilized when additional information is needed, such as when determining coronary flow reserve and assessing vessel compression throughout the cardiac cycle with intravascular ultrasound.

Stress testing, including myocardial perfusion imaging, has limitations because current published data indicate studies are rarely positive for ischemia, even in those who suffered SCD.3,13 Evaluation using stress testing can often lead to both false positive and false negative results, which limits the utility in AAOCA assessment. Tomographic imaging is not only now considered to be the imaging modality of choice to evaluate AAOCA, but also crucial in determining details of ostial anatomy and the course of the anomalous vessel. Cardiac MRI and CT are the preferred modes of imaging, and the choice between the two varies among institutions.

The management of patients with AAOCA is also challenging given the paucity of data in risk stratification and longitudinal follow-up data. Existing research varies significantly among different centers as recently described by the Congenital Heart Surgeons Society (CHSS).14 Management strategies span from surgery for all, to exercise restriction, to observation without restriction, depending on the institution, vessel anatomy, and vessel course.

Recommending exercise restriction, either comprehensive or selective, for competitive sports is not without controversy. First, exercise restriction would not necessarily prevent the possibility of SCD occurring at rest or with minimal activity. In addition, health care providers should consider the psychological and emotional consequences of restricting exercise in a child or adolescent and the known health consequences of not exercising. Moreover, although most reported series of patients operated on do not have abnormalities identified with stress testing – with and without myocardial perfusion tests postoperatively – a recent series from the Children's Hospital of Philadelphia reported that five out of 24 patients operated on had abnormal stress findings postoperatively,13 suggesting that the value of an operation may be limited. Reports of SCD following successful surgical repair of AAOCA have also been described.15

Figure 1

Figure 1

In light of so many unknowns and a hunger for meaningful data, a multi-institutional AAOCA registry was recently developed by the Congenital Heart Surgeons' Society (CHSS)16,17, and in December of 2012, the first dedicated Coronary Anomalies Program was created at Texas Children's Hospital. The program aims at a standardized approach to the diagnosis, management, and follow-up of these patients. A multidisciplinary team including dedicated cardiologists, surgeons, cardiovascular radiologists, nurses, and research and outcomes program staff developed an algorithm (Figure 1) that has been followed consistently.18 Initial data in 90 patients evaluated and followed thus far show that approximately 45% of patients are symptomatic upon presentation, 4% present with either sudden cardiac arrest or shock, and 43% are asymptomatic. Of these patients, 35% underwent surgical intervention, and all have returned to physical activity with no restrictions postoperatively.19

This remains an obscure area in which many questions are yet to be answered. However, nationwide efforts for collaboration continue to gather meaningful data, with longitudinal follow-up of patients with AAOCA in order to allow better risk stratification and hopefully decrease the occurrence of SCD and unnecessary exercise restriction in these patients.


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