Cardiology Unit, ASST Fatebenefratelli Sacco - Luigi Sacco University Hospital, University of Milan, Via G.B. Grassi, 74, 20157, Milan, Italy.
Department of Cardiology, Saint Martin Private Hospital Center, Caen, France.
Pediatr Cardiol. 2021 Dec;42(8):1676-1687. doi: 10.1007/s00246-021-02713-y. Epub 2021 Aug 30.
Coronary artery anomalies (CAAs) are a heterogeneous group of rare congenital diseases whose features and pathophysiological mechanisms are extremely variable, ranging from silent anomalies to sudden cardiac death (SCD) in the most severe cases. Although rare, congenital CAAs confer a high risk of myocardial ischemia and SCD, especially in young, previously "healthy" athletes during or immediately after vigorous exertion. Although some high-risk features that may lead to SCD have been identified, specific pathophysiological mechanisms related to SCD still remain poorly understood. When a CAA is incidentally diagnosed, optimal SCD risk stratification remains challenging, particularly in cases of anomalous aortic origin of a coronary artery arising from the opposite aortic sinus of Valsalva (ACAOS). In recent times, invasive imaging with intravascular ultrasound has gained a role in further identifying high-risk anatomic features; it has been integrated with traditional, non-invasive anatomic imaging evaluations, typically high-quality echocardiography, and cardiac magnetic resonance. Multidisciplinary programs and specific SCD risk scores should be developed in an endeavor to choose the right therapeutic approach, either clinical or interventional/surgical. Intravascular ultrasound is an extremely useful tool to evaluate vessel stenosis, even if prospective studies are still required to further validate this diagnostic strategy. In the present review, we aimed to analyze the pathophysiology and the clinical impact of ACAOS. We also summarized the predominant mechanisms for interference with normal coronary artery function, which might contribute to the onset of life-threatening arrhythmias and SCD.
冠状动脉异常（CAAs）是一组罕见的先天性疾病，其特征和病理生理机制极其多样化，从轻症异常到最严重情况下的心脏性猝死（SCD）不等。尽管罕见，但先天性 CAAs 会导致心肌缺血和 SCD 的风险增加，尤其是在剧烈运动期间或之后，年轻、之前“健康”的运动员中。尽管已经确定了一些可能导致 SCD 的高危特征，但与 SCD 相关的具体病理生理机制仍知之甚少。当偶然诊断出 CAA 时，最佳 SCD 风险分层仍然具有挑战性，尤其是在起源于相反主动脉窦的冠状动脉异常起源于主动脉（ACAOS）的情况下。最近，血管内超声的介入成像在进一步识别高危解剖特征方面发挥了作用；它与传统的、非侵入性的解剖成像评估（通常是高质量的超声心动图和心脏磁共振）相结合。应该制定多学科计划和特定的 SCD 风险评分，以努力选择正确的治疗方法，无论是临床治疗还是介入/手术治疗。血管内超声是评估血管狭窄的极其有用的工具，尽管仍需要前瞻性研究来进一步验证这种诊断策略。在本综述中，我们旨在分析 ACAOS 的病理生理学和临床影响。我们还总结了干扰正常冠状动脉功能的主要机制，这些机制可能导致危及生命的心律失常和 SCD 的发生。
