Abstract
Coronary artery anomalies (CAA) represent a heterogeneous group of congenital disorders of the arterial coronary circulation, defined by an anomalous origin of the coronary ostium and/or vessel course. Of particular interest are anomalous coronary arteries originating from the opposite sinus of Valsalva (ACAOS). The interarterial variants (with the anomalous vessel situated between the great arteries) are historically called “malignant,” based on an anticipated higher risk for myocardial ischemia and sudden cardiac death (SCD), especially affecting young patients during strenuous physical activity. However, the interarterial course itself may not be the predominant cause of ischemia, but rather represents a surrogate for other ischemia-associated anatomical high-risk features. As the exact pathophysiology of ACAOS is not well-understood, there is a lack of evidence-based guidelines addressing optimal diagnostic work-up, downstream testing, sports counseling, and therapeutic options in patients with ACAOS. Therefore, treating physicians are often left with uncertainty regarding the clinical management of affected patients. This review focuses on the pathophysiologic consequences of ACAOS on myocardial ischemia and discusses the concept of the interplay between fixed and dynamic coronary stenosis. Further, we discuss the advantages and limitations of the different diagnostic modalities and give an outlook by highlighting the gaps of knowledge in the assessment of such anomalies.
Highlights
Coronary artery anomalies (CAA) represent a heterogeneous group of congenital disorders of the arterial coronary circulation, hallmarked by the anomalous origin of the coronary ostium, vessel course, and/or unusual number [1]
Of particular interest are anomalous coronary arteries with the origin of the anomalous vessel from the opposite sinus of Valsalva (ACAOS), especially if they follow an interarterial course between the great arteries
Autopsy series showed that ACAOS were in up to one-third the underlying cause of sports-related sudden cardiac death (SCD) in young military recruits in the United States (L-ACAOS more frequently than R-ACAOS) [9, 12, 13]
Summary
Coronary artery anomalies (CAA) represent a heterogeneous group of congenital disorders of the arterial coronary circulation, hallmarked by the anomalous origin of the coronary ostium, vessel course, and/or unusual number [1]. Of particular interest are anomalous coronary arteries with the origin of the anomalous vessel from the opposite sinus of Valsalva (ACAOS), especially if they follow an interarterial course between the great arteries (i.e., aorta and pulmonary artery) This rare congenital abnormality has a prevalence of 0.26% in the general population (0.03% for left coronary ACAOS; L-ACAOS, 0.23% for right coronary ACAOS; R-ACAOS) [5, 6]. Management strategies in the middle-aged and elderly group is even less well-established compared to young individuals, and range from strict sports restriction and/or revascularization to watchful waiting [14, 16,17,18, 23] (see Table 1 for a summary of available recommendations) The latter strategy (i.e., watchful waiting) is supported by growing evidence for possibly decreasing hemodynamic relevance of the ACAOS above a certain age [24], when symptomatic CAD becomes more prevalent [25]. We will discuss advantages and limitations of the different diagnostic modalities and provide an outlook by highlighting the gaps of knowledge in the evaluation of ACAOS patients
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