Proximal Anomalous Connections of Coronary Arteries in Adults
Aubry P, Halna du Fretay X, Calvert PA, Dupouy P, Hyafil F, Laissy JP and Juliard JM.
Intech. 2012. In: Rao PS, editor. Congenital heart disease: Selected Aspects: 181-230.
Isolated proximal ANOmalous connections of CORonary arteries (ANOCOR) are not rare with an angiographic prevalence and tomographic prevalence of 0.5% and 1.3% respectively, in adult populations. The diagnosis of ANOCOR is sometimes fortuitous in adults undergoing a coronary angiography to detect a coronary artery disease (CAD). The absence of diagnosis in young people may have severe consequences with sudden death occurring frequently during intensive exertion. The prognosis depends mainly on the initial course of the ectopic coronary vessel. Preaortic course with intramural segment is recognized as high risk for sudden death in children or young adults. Management of ANOCOR with intramural course may be difficult in patients >35 years of age and with no evidence of myocardial ischemia. The mechanisms of life-threatening cardiac events are still not well understood even if anatomical risk factors are identified. Fortunately, most cases of ANOCOR are simply incidental anatomical findings. Multidetector computed tomography (CT) is recognized as the best imaging technique for identifying ANOCOR. Intravascular ultrasonography (IVUS) may help to quantify the severity of high-risk ANOCOR. In the ACC/AHA 2008 guidelines for the management of adults with congenital heart disease, surgical repair is the treatment of choice for high-risk ANOCOR (Warnes et al. 2008). However, this therapeutic management is based on little solid data with limited long-term follow-up. Percutaneous coronary intervention (PCI) has been proposed in some ANOCOR. The limited experience of most angiographers in detection of ANOCOR may explain non infrequent misdiagnoses with erroneous interpretations of ANOCOR. Large-scale prospective multicenter studies are needed to improve screening and imaging strategies and to better define the treatment of these potentially lethal congenital coronary abnormalities. Today, prospective registries are ongoing in France and North and South America with the goal of assessing the natural history of ANOCOR, as well as the long-term impact of surgical repair or PCI. The present review will focus on recent imaging modalities allowing us to revisit previous concepts and definitions.
