Working Diagnosis:
Anomalous aortic origin of the right coronary artery (AAOCA) from the left sinus of Valsalva with an intramural segment and interarterial course

Treatment:
Possible treatment options include coronary bypass grafting, reimplantation, or an unroofing procedure.

Outcome:
The patient underwent a successful unroofing procedure to allow perfusion of the right coronary artery along the length of the intramural segment. This process allowed flow from the native right sinus of Valsalva, effectively widening the os and eliminating the potential for compression since her AAOCA had a large intramural and interarterial course.

Author's Comments:
AAOCA's are estimated to be present in approximately 1% of the overall population but are proportionately more common in athletes with sudden cardiac death (SCD). The pathophysiology of AAOCA is incompletely understood, but when SCD occurs secondary to AAOCA, it is caused by insufficient coronary blood flow, often occurring during high intensity exercise. There is a lack of consensus on management of asymptomatic right AAOCA: however, surgery is warranted for symptomatic right AAOCA's. According to the AHA/ACC 2015 guidelines, an athlete with a successful surgical repair of an anomalous origin from the wrong sinus may participate in sport of any kind 3 months after surgery if the patient remains free of symptoms and an exercise stress test shows no evidence of ischemia or cardiac arrhythmias. As such, this patient was cleared four months post-operatively and returned to sport after completing a gradual exercise progression. Since rejoining her team, the patient was in the starting lineup for each game without further symptoms.

Editor's Comments:
The takeaway point of this case is the imperative pursuit of a definitive diagnosis when a patient presents with chest pain, dizziness, shortness of breath, heart palpitations, or loss of consciousness during exertion. While sudden cardiac death often occurs without prior warning symptoms or signs, some etiologies of SCD may present initially with non-fatal symptoms/signs. Any patient who presents with unexplained symptoms/signs during exertion warrants further evaluation by a cardiologist for further work-up.

References:
Cheezum, M. K., et al. (2017). Anomalous Aortic Origin of a Coronary Artery From the Inappropriate Sinus of Valsalva. Journal of the American College of Cardiology 69(12): 1592-1608.

Maron, B. J., et al. (2015). Eligibility and Disqualification Recommendations for Competitive Athletes with Cardiovascular Abnormalities: Preamble, Principles, and General Considerations: A Scientific Statement From the American Heart Association and American College of Cardiology. Journal of the American College of Cardiology 66(21): 2343-2349.

Schubert, S. A. and I. L. Kron (2016). Surgical Unroofing for Anomalous Aortic Origin of Coronary Arteries. Operative Techniques in Thoracic and Cardiovascular Surgery 21(3): 162-177.

Hainline, B., et al. (2016). Interassociation Consensus Statement on Cardiovascular Care of College Student-Athletes; Journal of the American College of Cardiology 67(25): 2981-2995.

Villa, A. D., et al. (2016). Coronary artery anomalies overview: The normal and the abnormal. World journal of radiology 8(6): 537-555.

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