Anomalous coronary arteries and the risk of adverse cardiac events

An anomalous coronary artery from the opposite sinus of Valsalva (ACAOS) is a rare inherited cardiac condition with a prevalence of around 1% in the general population. ACAOS is associated with sudden cardiac death in young athletes and therefore its exclusion by means of cardiac imaging is crucial in young symptomatic individuals. With the increasing use of noninvasive imaging modalities to exclude coronary artery disease (CAD) in the middle-aged and older population, physicians are faced with an increased absolute number of patients with the incidental finding of ACAOS. However, it seems that in the middle-aged population the intrinsic risk of ACAOS moves into the background compared with the gradually increasing risk associated with CAD. The assessment of high-risk anatomical features, such as slit-like ostium, acute take-off angle, interarterial course, intramural course with elliptical vessel shape and proximal narrowing of the anomalous vessel is a cornerstone in the evaluation of patients with ACAOS. Further, downstream testing in order to evaluate haemodynamic relevance by means of perfusion imaging of anatomic high-risk features in ACAOS is critical. Perfusion can be tested with single-photon emission computed tomography (SPECT) or positron-emission tomography (PET), allowing fusion with coronary computed tomography angiography and enabling differentiation of perfusion defects from an anomalous coronary artery and from possible concomitant CAD. As to date no randomised prospective trials are available, the therapeutic approach to sports restriction and/or surgery is mainly based on experts’ opinions and recommendations with a low level of evidence, and is usually chosen on an individual case-by-case basis. A presumed prognostic benefit from surgical repair and sports restriction should be carefully balanced against the risk of surgery and possible impairment to quality of life, and patient management should be chosen only after considering symptoms, age, sports behaviour, and imaging information.