Congenital coronary artery anomalies

Abstract

Despite the low prevalence in the general population, congenital coronary artery anomalies (CAAs) are well recognized as a major cause of sudden cardiac death (SCD). Not all CAAs have the same prognostic impact and each of them should be discussed individually. Apart from anomalous origin of a coronary artery (CA) from the pulmonary artery, the anomalous origin from the wrong aortic sinus, either the left main CA from the right sinus or the right CA from the left sinus, are traditionally considered as the highest risk of SCD. CAAs with an inter-arterial course carry the highest risk of ischaemia, particularly during exercise. Several pathophysiological mechanisms have been postulated, including compression of the anomalous CA between the aorta and the pulmonary artery, the acute angle take-off, the proximal intra-mural aortic course, and a superimposed CA spasm. The diagnostic work-up, including ischaemia provocation test imaging tools, and current management strategies, from sport restriction to surgical intervention, are discussed.