A 17-Year Old Hockey Player With Aortic Abnormalities: Dissecting the Decisions Affecting Aortopathies and Sport Participation

A 17-year-old male competitive hockey player presents for evaluation after detection of an enlarged aorta. He underwent screening for aortic disease because his father had an aortic dissection and emergent aortic surgery at 23 years old. Through genetic testing, his father was subsequently found to have an ACTA2 mutation.

A transthoracic echocardiogram was performed demonstrating a tricuspid aortic valve without stenosis or regurgitation, normal left ventricular dimensions, left ventricular ejection fraction 60% and an aortic root diameter 3.47 cm measured at the sinus of Valsalva (patient height 172 cm, weight 67 kg, calculated Z score 2.28). Imaging of the aorta was additionally notable for a loss of the normal aortic contour and no discernible sinotubular junction. The ascending aorta was enlarged and measured 3.93 cm.

Figure 1

A chest magnetic resonance angiography (MRA) performed 15 months prior showed mild dilation of the aortic root (3.3 cm x 3.3 cm), measured at the sinus of Valsalva, and mild dilation of the ascending aorta (3.7 cm x 3.4 cm).

On physical examination, there were no physical stigmata to suggest a systemic connective tissue disorder. He was asymptomatic and reported no chest discomfort or shortness of breath at rest or with exertion.

What is the next step in the evaluation of this patient and what are your recommendations for sports participation?

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