The following are key points to remember from this review article about anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva:

1. The true prevalence of AAOCA in the general population remains unknown because few studies have screened patients in the absence of clinical indication for testing. The estimated frequency of anomalous left coronary artery (ALCA) arising from the inappropriate sinus is low as compared with that of anomalous right coronary artery (ARCA) with a weighted prevalence of 0.03% for ALCA as compared with 0.28% for ARCA.
2. Although AAOCA may be diagnosed with echocardiography in young patients with good image quality, experienced operators are required and studies may be limited by spatial resolution. A report from the Congenital Heart Surgeon’s Society Registry suggested limited agreement between institutional and expert transthoracic echocardiogram reports.
3. Coronary computed tomographic angiography (CTA) has an important role in the assessment of coronary artery anomalies. Advantages include rapid scan times and high spatial resolution, which are weighed against disadvantages of radiation exposure and need for iodinated contrast. Appropriate use criteria and guidelines support the use of coronary CTA.
4. Magnetic resonance angiography (MRA) may have a role in assessment of coronary anomalies given lack of radiation exposure for need for contrast. It may be limited by lower spatial resolution, longer scan times, and higher cost as compared with CTA.
5. Intravascular ultrasound (IVUS) offers excellent dynamic imaging, although it may be difficult to engage the coronary in some cases of AAOCA, particularly those with ostial narrowing or an acute takeoff. Additionally, it is necessary to distinguish vessel spasm from true narrowing/compression.
6. Both false-positive and false-negative results limit the value of stress testing. Normal exercise testing prior to sudden cardiac death has been well documented. The absence of ischemia during stress testing cannot be viewed as reassuring.
7. The risk of sudden cardiac death appears highest in young individuals, particularly during or following a period of strenuous exertion, and particularly in those with interarterial ALCA.
8. Studies of adult cohorts with ARCA undergoing conservative therapy have observed a very low mortality (<1%) in 1.3 to 5.6 years of follow-up.
9. Controversy remains regarding the management of patients with interarterial ARCA or ALCA. The risk of sudden cardiac death appears highest among patients with interarterial ALCA and, in selected individuals, the benefits of revascularization likely outweigh the risks of surgery. The authors recommend an individualized approach of risk assessment. Consideration for surgical repair is recommended in those with a concerning history (aborted sudden cardiac death, documented ischemia, exertional syncope, or chest pain) or imaging evidence of proximal vessel narrowing.
10. The authors state that a conservative approach may be reasonable in asymptomatic patients with interarterial ARCA without evidence of ischemia or proximal vessel narrowing.
11. Exercise restriction has been addressed in the 2015 American College of Cardiology/American Heart Association updated scientific statement. In terms of exercise restriction, restriction from all competitive sports is recommended in patients with ALCA while awaiting repair. For ARCA patients without symptoms or a positive exercise test, “permission to compete can be considered after adequate counseling of the athlete and/or the athlete’s parents as to the risk and benefit taking into consideration the uncertainty of a negative stress test.”

Keywords: Athletes, Cardiac Surgical Procedures, Chest Pain, Coronary Vessel Anomalies, Death, Sudden, Cardiac, Diagnostic Imaging, Echocardiography, Exercise Test, Heart Defects, Congenital, Magnetic Resonance Angiography, Myocardial Revascularization, Physical Exertion, Radiation, Risk Assessment, Sinus of Valsalva, Sports, Syncope

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