**Proximal Anomalous Connections of Coronary Arteries in Adults**

Pierre Aubry1, Xavier Halna du Fretay2, Patrick A. Calvert1,3, Patrick Dupouy4, Fabien Hyafil5, Jean-Pierre Laissy6 and Jean-Michel Juliard1 *1Department of Cardiology, Bichat Hospital, Paris 2Department of Cardiology, Foch Hospital, Suresnes 3Department of Cardiology, Papworth Hospital* 

> *NHS Foundation Trust, Cambridge, 4Department of Interventional Cardiology and*

*Cardiovascular Imaging, Hôpital Privé d'Antony, Antony 5Department of Nuclear Medicine, Bichat Hospital, Paris 6 Department of Radiology, Bichat Hospital, Paris,* 

*1,2,4,5,6France* 

*3United Kingdom* 

#### **1. Introduction**

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Isolated proximal **ANO**malous connections of **COR**onary 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.

Proximal Anomalous Connections of Coronary Arteries in Adults 185

Fig. 1. Axial cross-sectional computed tomography views showing normal origin of the right

Cardiologists and radiologists should be aware of the normal origin and anatomical variants (Angelini, 2007) of the coronary arteries (table 1) in order to make an accurate diagnosis of

**Normal connections**  Left coronary Left main dividing into LAD and CX coronary arteries

**Anatomical variants**  Left coronary Separate origin of LAD and CX coronary arteries in left sinus

Level of connection Up to 10 mm above the level of the sinotubular junction

Table 1. Normal connections and anatomical variants of the coronary arteries. CX:

Right coronary Separate origin of conus artery in right sinus

coronary artery (arrow) and left coronary artery (arrow head).

Right artery Single ostium Site of left connection In mid-left sinus Site of right connection In mid-right sinus Level of connection Upper half of sinus

Site of left connection Close to the right sinus

Site of right connection Close to the non-coronary sinus

Ostium shape Circular Angulation with aorta 45 to 90° Initial course to aorta Extramural

Ostium shape Ovoid Angulation with aorta 90 to 135°

circumflex, LAD: left anterior descending.

ANOCOR.

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.
