Coronary artery anomalies (CCAs), a complex of defects featuring congenital incorrect origination of the coronary arteries from the aorta or the pulmonary artery, are rare and may present clinically with chest, pain, dyspnea, arrhythmias, syncope or sudden cardiac death (SCD). CAAs are a persistent challenge ...
Coronary artery anomalies (CCAs), a complex of defects featuring congenital incorrect origination of the coronary arteries from the aorta or the pulmonary artery, are rare and may present clinically with chest, pain, dyspnea, arrhythmias, syncope or sudden cardiac death (SCD). CAAs are a persistent challenge in cardiology, starting with differentiating patients with CAAs of favorable versus poor prognosis, especially in view of sports activities. The identification and correct description of the anomaly and risk stratification of individual patients with CAAs is delicate, but crucial. Based on autopsy studies, in sports-related sudden cardiac death, CAAs were found to be associated with adverse cardiovascular events during competitive athletes. It is still unclear which level of screening imaging should be applied to young individuals to effectively prevent SCD related to some CAAs. Moreover, at present treatment strategies are mainly based on expert's opinions, while controlled randomized trials assessing outcomes of different therapeutic strategies are lacking.
On the other hand, with the increasing use of invasive- and non-invasive imaging to rule out coronary artery atherosclerotic disease in middle-aged and older population, we are facing increased recognition of asymptomatic CAAs. Whether older individuals with coincidentally detected CAAs are at the same risk as young individuals is still unclear. Non-invasive anatomic imaging is complementary to invasive imaging and helps to further delineate high-risk anatomic features. Using downstream functional noninvasive perfusion imaging or intravascular coronary imaging can potentially assess ischemia induced by dynamic lumen compression of CAAs during increased volume load and tachycardia. Implementing opportune clinical and imaging information can effectively guide clinical recommendations (periodic observation, surgical correction, percutaneous interventional correction, medication, sports management) on these patients.
Multicenter registries may help adapting current recommendations by accumulating large databases.
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