Aortic Root Disease in Athletes: Aortic Root Dilation, Anomalous Coronary Artery, Bicuspid Aortic Valve, and Marfan’s Syndrome

Two professional athletes in the U.S. National Basketball Association required surgery for aortic root dilation in 2012. These cases have attracted attention in sports medicine to the importance of aortic root disease in athletes. In addition to aortic root dilation, other forms of aortic disease include anomalous coronary artery, bicuspid aortic valve, and Marfan's syndrome. In this review, electronic database literature searches were performed using the terms "aortic root" and "athletes." The literature search produced 122 manuscripts. Of these, 22 were on aortic root dilation, 21 on anomalous coronary arteries, 12 on bicuspid aortic valves, and 8 on Marfan's syndrome. Aortic root dilation is a condition involving pathologic dilation of the aortic root, which can lead to life-threatening sequelae. Prevalence of the condition among athletes and higher risk athletes in particular sports needs to be better delineated. Normative parameters for aortic root diameter in the general population are proportionate to anthropomorphic variables, but this has not been validated for athletes at the extremes of anthropomorphic indices. Although echocardiography is the favored screening modality, computed tomography (CT) and cardiac magnetic resonance imaging (MRI) are also used for diagnosis and surgical planning. Medical management has utilized beta-blockers, with more recent use of angiotensin-converting enzyme (ACE) inhibitors, angiotensin II receptor blockers (ARBs), and statins. Indications for surgery are based on comorbidities, degree of dilation, and rate of progression. Management decisions for aortic root dilation in athletes are nuanced and will benefit from the development of evidence-based guidelines. Anomalous coronary artery is another form of aortic disease with relevance in athletes. Diagnosis has traditionally been through cardiac catheterization, but more recently has included evaluation with echocardiography, multislice CT, and MRI. Athletes with this condition should be restricted from participation in competitive sports, but can be cleared for participation 6 months after surgical repair. Bicuspid aortic valve is another form of aortic root disease with significance in athletes. Although echocardiography has traditionally been used for diagnosis, CT and MRI have proven more sensitive and specific. Management of bicuspid aortic valve consists of surveillance through echocardiography, medical therapy with beta-blockers and ARBs, and surgery. Guidelines for sports participation are based on the presence of aortic stenosis, aortic regurgitation, and aortic root dilation. Marfan's syndrome is a genetic disorder with a number of cardiac manifestations including aortic root dilation, aneurysm, and dissection. Medical management involves beta-blockers and ARBs. Thresholds for surgical management differ from the general population. With regard to sports participation, the most important consideration is early detection. Athletes with the stigmata of Marfan's syndrome or with family history should be tested. Further research should determine whether more aggressive screening is warranted in sports with taller athletes. Athletes with Marfan's syndrome should be restricted from activities involving collision and heavy contact, avoid isometric exercise, and only participate in activities with low intensity, low dynamic, and low static components. In summary, many forms of aortic root disease afflict athletes and need to be appreciated by sports medicine practitioners because of their potential to lead to tragic but preventable deaths in an otherwise healthy population.