The aortic arch (AA) branches vascularize the vital structures in the head, neck and upper limbs, including the brain [1, 7]. The anatomy of AA has become a well-accepted research topic due to many reasons, such as the significant clinical implications of the perfusion territory and the geographical variation in branching pattern [7, 14, 18, 19]. As a pioneer study, this study has evaluated the branching pattern of AA in a selected Sri Lankan population (using contrast-enhanced CT scans of the thorax) and compared the AA anatomy with available data from other communities. Ninety per cent (90%) of the study population was found to have the standard (Type 1) aortic arch branching pattern with brachiocephalic trunk (BCT), left common carotid (LCCA), and left subclavian (LSA) arteries arising from the AA. Type 2 AA (bovine variant) with a common origin for BCT and LCCA was the most common variant (4.6%) observed. Type 4, 5 or 7 arches were absent in the study group. Despite minor variations among males and females, no gender variation was appreciated in the branching pattern of AA.
Further re-instating the geographical distribution in AA anatomy, the AA anatomy of the index population was closely followed by that of the nearest geographical region - the Indian population [15–17]. However, few Indian studies have reported much higher prevalence rates in AA variations (36.5%; n = 52 [8]; 36.67% n = 30; [19]. Anyhow, the small sample size of these cadaveric studies needs to be considered seriously upon generalizing the findings [8, 19]. Additionally, the global prevalence of AA variations calculated from pooled data was higher than reported by the current study. Compared to the global prevalence, previous studies have also reported a low prevalence of AA variations for Asian, European and North American populations [7]. In contrast, arch variations have been reported more frequently in African (65.2%) and South American (69.5%) populations [7, 20].
Although seven types of AA have been described previously, only four types were found in the study population [7]; Type 4, 5 and 7 arches were not reported in the study group. The current study agrees with many previous studies by reporting Type 2 AA as the most prevalent arch variant [4, 7, 8, 12, 14, 15, 19–21]. However, Qiu et al. (n = 120) in a cadaveric study have reported type 3 AA (left vertebral artery directly arising from the AA; 7.5%) as the most prevalent anomaly [11]. However, the global prevalence of type 3 arch was as low as 2.9% (95% CI, 1.7–4.3) [7]. Type 7 arch (right side aortic arch) was the least common AA variant reported globally (0.2%) as well as for Asians (0.2%) [7].
As described earlier, type 2 AA was the most prevalent variant for many populations [7, 14–16, 19–21]. The prevalence of type 2 AA for the study population (4.6%) was lower than the global values (13.6%). In some geographical regions, such as Africa and South America, type 2 AA was found in nearly one-fourth of the population [7]. Importantly, type 2 AA has shown a positive association with aortic dilatation and dissection. Also, in aortic dissection, the prognosis of patients with type 2 AA was poor [7, 14]. Increased flow velocity in AA due to fewer branches, as seen in the type 2 arch, is the postulated pathophysiology for increased morbidity and mortality in aortic dissection [7]. Another major clinical significance in the AA variant would be the potential surgical complication during thoracic surgeries, mainly if the variant were not diagnosed previously [7]. Thus, even 4.6% of prevalence rates reported in the study population warrants serious consideration.
Type 6 arch (AA with right aberrant subclavian artery) is also an arch variant with significant clinical implications. The prevalence of type 6 AA reported for the study population (1.8%) was higher than the global prevalence (0.7%. 0.2–1.5) [7]. The lowest prevalence of type 6 AA was reported in South Americans (0.2%; 95% CI. 0.0–4.6) and the highest in the Africans (1.4%; 95% CI. 0.0–6.2). Notably, the prevalence of type 6 AA among the male population of the current study has reached 2.8%. In common with many other AA anomalies, the patients with type 6 AA are often asymptomatic. However, the retro-oesophageal pathway of the right subclavian artery can occasionally produce symptoms such as dyspnea and dysphagia. Dyspnea and dysphagia are respectively due to the tracheal and oesophageal compression by the aberrant artery. These symptoms have been reported approximately in 10% of patients with type 6 AA [7, 22]. The symptomatic incidence of type 6 AA has shown a positive association with a vascular diverticulum called Kommerell diverticulum, a bulbous dilatation at the origin of the right subclavian artery [22]. The Kommerell diverticulum - an embryological remnant of dorsal AA - is reported only in 15% − 30% of type 6 arches [20]. Type 6 arch is also associated with congenital cardiovascular diseases such as coarctation of the aorta, persistent ductus arteriosus, ventricular septal defects, carotid and vertebral artery abnormalities [20]. Anyhow, none of our study participants with type 6 AA was symptomatic, nor with a Kommerell diverticulum or congenital cardiac anomalies.
Type 7 arch (right-sided aortic arch), a rare congenital AA abnormality, is frequently associated with many congenital cardiovascular abnormalities such as Tetralogy of Fallot, pulmonary stenosis with septal defects, tricuspid atresia and truncus arteriosus [2, 3, 18]. Both congenital cardiovascular abnormalities and type 7 AA are aberrations of normal development during early fetal development. In this period, six pairs of AA are formed to perfuse the pharyngeal arches. The left fourth arch usually persists to adult life as the AA, while the fourth right arch disappears. Very rarely, vice versa occurs, resulting in right-sided AA [2, 3]. In addition to associated anomalies, the type 7 arch itself is with critical vascular abnormalities such as compression or kinking of the aorta, causing significant vascular stenosis [18]. Therefore, despite being a rare arch anomaly, type 7 AA is of considerable clinical significance.
The male preponderance in AA variations observed in this study agrees with Keet et al. [20]. Keet et al. have described a significant gender variation (p = 0.025) in a study with a predominantly male sample (70% males). Anyhow, male predominance identified in this study was not statically significant (p = 0.605). Several other studies have also reported insignificant gender influence on AA variations. Interestingly, some have reported a gender influence, particularly on specific AA variants, such as on type 6 AA [20, 23, 24].
In addition to direct clinical implications, the anatomy of AA exerts a substantial impact on angiographic and vascular intervention procedures. The branching pattern of the AA influences the choice of the angiographic catheter. Inappropriate angiographic catheter usage is known to increase the procedural time and the contrast dose used for the procedure [4, 5]. The procedural time is directly proportionate to the patient radiation dose and indirectly related to the carcinogenic risk [25]. Notably, the injected contrast dose is positively related to the incidence of contrast-induced adverse reactions and nephropathy [5]. It has been stated that the carotid stenting procedure is riskier and complicated in patients with type 2 AA, for whom alternative angiographic techniques, such as the brachial artery approach, is more appropriate than a trans-femoral approach [7]. All the above facts highlight the importance of understanding the prevalence of AA's anatomical variations for a better patient outcome in angiographic and surgical procedures.
This study provides a step forward understanding of the prevalence of AA variants for a Sri Lankan population. The internal integrity of the study is assured, considering the reliability of contrast-enhanced CT with multiplanar reformat on delineating vascular anatomy [1, 9]. Since the study sample has obtained from two separate geographical regions from Sri Lanka, the findings can be generalized to the Sri Lankan population to a considerable limit. However, results could be more widely generalized in a study sample representing all geographical regions.