ELECTRONIC SUBMISSION FOR CONSIDERATION IN THE UNIVERSITY OF TORONTO MEDICAL JOURNAL TITLE: CT Angiographic assessment of common, uncommon and rare aortic arch anatomic variants in 2004 patients AUTHOR NAMES: 1Winnie Fu, MD*, 2Harris Chou, MSc, 3Daryl Bergen, MD, 3 Manraj K.S. Heran, MD AUTHOR AFFLIATIONS: 1 Department of Radiology, University of Calgary, 3330 Hospital Drive NW, Calgary, Alberta T2N 4N1. Address correspondence to Winnie Fu. 1 Leslie Dan Faculty of Pharmacy, University of Toronto, Ontario. 3 Department of Neuroradiology, University of British Columbia, British Columbia. CORRESPONDING AUTHOR EMAIL ADDRESS: winniewinglan.fu@albertahealthservices.ca Abstract Aortic arch anatomic anomalies are not infrequent in our population. The presence of an arch anomaly can be a source of unexpected technical challenge in the catheter angiography and intervention. Today, these anomalies can be better characterized with the non-invasive CT angiography. In our retrospective study, we have analyzed the CT angiography images of 2004 patients with normal arch anatomy, common, uncommon and rare arch anomalies to better assess their incidence. The incidence of these arch anomalies will be helpful on surgical treatment planning of patients who will undergo interventional procedures involving the aortic arch. Keywords: aortic arch, anomalies, CTA, aberrant Introduction Anatomic variants on the origin of the great vessels from the aortic arch have been described in the literature,1,4 however updated data on the incidence of these arch anomalies is sparse. CT angiography (CTA) is rapidly becoming a popular, non-invasive tool for the evaluation of the anatomy of normal aortic arch and these anomalies. We have analyzed a group of patients who underwent CTA studies at our tertiary care hospital. The classic left-sided three vessel aortic arch is common in the population. The first branch is the brachiocephalic artery, which branches into the right subclavian artery and the right common carotid artery (CCA). The second branch is the left CCA, and the last branch is the left subclavian artery. Known minor variations include: a left CCA arising as a common origin of the brachiocephalic trunk, an aberrant right subclavian artery arising as the last branch from the aortic arch and coursing posterior to the esophagus, and right-sided aortic arches. Based on our experience, the presence of an arch anomaly may increase the risks associated with interventional procedures due to the increased technical difficulties of those procedures. CTA allows the presence of an arch anomaly to be detected preoperatively. A better knowledge of the aortic arch anatomy and the incidence of these arch anomalies is important to caution the interventional radiologists and vascular surgeons about the potential technical difficulties to perform vascular procedures and the increased procedural risks. Material and Methods In a retrospective review of 2004 CTA studies performed at our tertiary care hospital (Vancouver General Hospital, BC) from June 2005 to January 2008, cases with origin of the supraortic arteries different from the regular pattern were assessed. Patients of all ages who underwent CTA studies of the aortic arch and supraortic arteries for any reasons were studied, regardless of the previous surgery or therapy to the vessels and the presence of disease or vessel tortuosity. Those studies which failed to achieve acceptable image quality in CTA of the cervical vasculature, for example, inadequate contrast opacification of the vessel lumen or with significant artifacts, were excluded for the purpose of data analysis. For each CTA study, a set of axial images was obtained from the aortic arch through the circle of Willis. The proper interpretation of these CTA images was performed by neuroradiologists and appropriately-trained medical students. The specific objectives of the retrospective study were to evaluate the aortic arch and great vessel anatomy, and assess of the carotid artery bifurcation level and the spatial relationship of external carotid artery to internal carotid artery. In most cases, the axial reconstructed coronal, sagittal and sagittal oblique images provided additional details of anatomic variance. Specific areas of assessment included the left CCA origin either common or arising from the brachiocephalic trunk, left vertebral artery origin from the aortic arch, and aberrant subclavian artery origins. The variables considered in the data analysis were the patient’s gender, age, and incidence of various aortic arch anatomic variants. The results in terms of anatomical variables were presented as percentages and compared with the previous literature reports. Results During the study period, 2004 patients underwent CTA studies of the aortic arch and supraortic arteries at our tertiary care hospital. The average age of our patient population was 59.6 years. 58% was men, while 42% was women. The classic left-sided three vessel aortic arch was seen in 75.5% of our patient population (Figure 1). a b c A B Figure 1. 56-year-old man with classic three vessel aortic arch. A. Axial contrasted enhanced CT image shows the three vessel aortic arch. B. Three-dimensional volume-rendered CT angiogram of aortic arch in oblique coronal plane shows three aupraaortic branches: (a) brachiocephalic artery, (b) left common carotid artery, and (c) left subclavian artery. The second most common arch anomaly occurs when the brachiocephalic and left common carotid arteries share a common trunk from the aortic arch (Figure 2), the occurrence of which was found to be 14.2% (284 cases) in our patient population. The least common arch branching pattern, in which the left common carotid artery originates directly from the brachiocephalic trunk, was present in our 58 cases (2.9%), as shown in Figure 3. A left vertebral artery with a direct origin from the aortic arch between the origins of the left common carotid artery and left subclavian artery arises cranially and enters the left transverse foramen of the fourth cervical vertebra (Figure 4). Of the 2004 patients, 127 cases (6.3%) had the left vertebral artery with a direct origin from the aortic arch. Figure 5 illustrates an aberrant right subclavian artery arising as the last branch from the aortic arch , with a retroesophageal course to supply the right upper limb. The aberrant right subclavian artery was seen in 20 of our 2004 cases (1.0%). A right sided aortic arch lies to the right of midline, with the descending aorta coursing to the right of the spine. Figure 6 shows a right aortic arch with mirror image branching. The right subclavian artery, right common carotid artery and left brachiocephalic trunk originate independently from the arch. 2 cases (0.1%) of right aortic arch with mirror image branching were seen in our study. B A Figure 2. 44-year-old woman with the common origin between brachiocephalic and left common carotid arteries A. Axial contrasted enhanced CT image shows the common origin between brachiocephalic and left common carotid arteries (white arrow). B. Curve planar reformatted CT image shows the common origin between brachiocephalic and left common carotid arteries (white arrow). A B Figure 3. 58-year-old woman with the left common carotid artery arising from the brachiocephalic artery. A. Axial contrasted enhanced CT image shows the left CCA arising from the brachiocephalic artery (white arrow). B. Three-dimensional volume-rendered CT angiogram of aortic arch in oblique coronal plane shows the left CCA arising from the brachiocephalic artery (white arrow). A B Figure 4. 35-year-old man with the left vertebral artery arising directly from the aortic arch. A. Axial contrasted enhanced CT image shows the left vertebral artery arising from the arch (white arrow). B. Three-dimensional volume-rendered CT angiogram of aortic arch in oblique coronal plane shows the left vertebral artery (white arrow) entering left transverse foramen of cervical vertebra. * Figure 5. 28-year-old man with aberrant right subclavian artery (white arrow) coursing behind the esophagus (asterisk) on an axial contrasted enhanced CT image. A B Figure 6. 24-year-old man with the right-sided aortic arch. A. Axial contrasted enhanced CT image shows the right-sided aortic arch(white arrow). B. Three-dimensional volume-rendered CT angiogram of aortic arch in oblique coronal plane shows the right-sided arch (white arrow). Discussion Aortic arch anomalies are not infrequent in the population. The embryologic development of the aortic arch and great vessels is based on a selected involution of particular vascular segments, thereby establishing the final pattern. Aortic arch anomalies result from deviation of this embryologic sequence.7 In our study, CTA has been demonstrated to be highly reliable in the recognition of these common anomalies and detection of rare anatomic variants of the aortic arch. A common origin of the left common carotid artery and brachiocephalic trunk is a relatively frequent anomaly, occurring in 14.2% of our patient population. Such a variation has been reported to occur at an incidence of 13% in the literature.6 However, although similar to the common origin variant, the origin of the left common carotid artery from the brachiocephalic trunk is less common. This arch branching pattern was present in 58 of our 2004 cases (2.9%), compared with the reported incidence of 9%. 6 In 127 of our cases (6.3%), the left vertebral artery arose from the aortic arch, compared with a documented occurrence of 2.4-5.8% in a large autopsy series.8 Another pattern, reported in 0.4-2.0% of the general population3, and seen in 1.0% of our cases, shows the left aortic arch with aberrant right subclavian artery. The mirror image branching arising from the right sided aortic arch is an unusual anomaly, occurring with a frequency of 0.1% in our patient population. Although there are some reports on the right aortic arch, its incidence in general has not been described in the literature. In view of the incidence of the CTA findings of aortic arch anomalies, it is important for the radiologists to recognize the complex anatomic anomalies because of its implications during interventional procedures. It is known that arch anomalies, such as bovine arch in which the left CCA has a common origin with the brachiocephalic artery, increase the technical difficulties of carotid artery stenting procedures, therefore leading to higher risk of neurological complications. 2,5 On the basis of higher procedural risk, a comprehensive anatomic evaluation of the aortic and supraortic circulation should include the CTA assessment, because these variations may be difficult to appreciate on conventional angiography. This is of particular value as the risk assessment and interventional procedure planning can be guided by the findings from CTA. However, it still remains to be explained the role of various arch anomalies in the development of procedural complications. In conclusion, consideration towards pre-procedure CTA’s assessing for aortic arch anomalies should receive more attention. Related issues to be investigated include the identification of the procedural risks associated with various arch anomalies and insights into the mechanisms of procedural complications. Acknowledgements The authors thank Dr Douglas Graeb at Vancouver General Hospital for his invaluable assistance. References 1. Arey JB. Malformations of the aorta and aortic arches. In: Cardiovascular pathology in infants and children. Philadelphia: WB Saunders Company; 1984: 242– 244. 2. Choi HM, Hobson II RW, Goldstein J, Chakhtoura E, Lal BK and Haser PB et al. Technical challenges in a program of carotid artery stenting, J Vasc Surg 2004; 40: 746-751. 3. Felson B, Cohen S and Courter S et al. Anomalous right subclavian artery. Radiology 1950; 54: 340-348. 4. Jaffe RB. Radiographic manifestations of congenital anomalies of the aortic arch. Radiol Clin North Am 1991; 29: 319-334. 5. Lin ST, Trocciola SM, Rhee J, Dayal R, Chaer R and Morrissey NJ et al. 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