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Table of Contents
ORIGINAL ARTICLE
Year : 2021  |  Volume : 70  |  Issue : 1  |  Page : 11-18

Aberrant right subclavian artery: A multi-detector computed tomography study


1 Department of Anatomy, Muzaffarnagar Medical College, Muzaffarnagar, Uttar Pradesh, India
2 Dr. O.P. Gupta Imaging Centre, Meerut, Uttar Pradesh, India

Date of Submission08-Jul-2020
Date of Acceptance08-Feb-2021
Date of Web Publication07-Apr-2021

Correspondence Address:
Dr. C S Ramesh Babu
Muzaffarnagar Medical College, N.H. 58, Opp. Begrajpur Industrial Area, Muzaffarnagar - 251 203, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JASI.JASI_129_20

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  Abstract 


Introduction: Aberrant right subclavian artery (ARSA) arises as the last branch of normally positioned aortic arch and its prevalence estimated as 0.16%–2.0% varies between different ethnic groups. Our aim was to assess the prevalence and associated branching anomalies of ARSA in the Indian participants. Material and Methods: Chest computed tomographic scans of 710 patients were analyzed to study the ARSA and its associated vascular anomalies. Results: We have observed 11 cases (5 in males and 6 in females) of ARSA with an estimated prevalence of 1.54%. In seven cases, there were four branches arising from the arch of aorta in the order of right common carotid, left common carotid, left subclavian, and aberrant right subclavian. In three cases, there were three branches – bicarotid trunk (common trunk of right and left carotids), left subclavian, and the aberrant right subclavian. In one case, there were five branches in the order of right common carotid, left common carotid, left vertebral, left subclavian, and aberrant right subclavian. Only two participants reported mild symptoms of dysphagia. In all the cases, the ARSA had retroesophageal course. Kommerell diverticulum was not observed. ARSA remain asymptomatic in most cases, but its presence should alert the clinician to look for associated vascular and any cardiac anomalies. Discussion and Conclusion: Awareness of the presence of ARSA is crucial for successful outcome of mediastinal, esophageal, and thoracic spine surgeries. Preprocedural computed tomography for the evaluation of aortic arch branching pattern will be beneficial for the successful performance of various surgical and radiological interventions.

Keywords: Arteria lusoria, bicarotid trunk, dysphagia, retroesophageal right subclavian artery, variant aortic arch branching


How to cite this article:
Ramesh Babu C S, Gupta OP, Kumar A. Aberrant right subclavian artery: A multi-detector computed tomography study. J Anat Soc India 2021;70:11-8

How to cite this URL:
Ramesh Babu C S, Gupta OP, Kumar A. Aberrant right subclavian artery: A multi-detector computed tomography study. J Anat Soc India [serial online] 2021 [cited 2021 Apr 22];70:11-8. Available from: https://www.jasi.org.in/text.asp?2021/70/1/11/313154




  Introduction Top


Arch of aorta (AA) passes from right to left arching over the root of the left lung and gives three branches brachiocephalic trunk (BT) which further divides into right common carotid (RCCA) and right subclavian arteries (RSA), left common carotid artery (LCCA), and left subclavian artery (LSA) in that order from right to left. This normal branching pattern is reported to occur in 64.9%–94.3% of population.[1] A recent systematic review and meta-analysis of 23,882 arches from 51 articles by Popieluszko et al.(2018) found normal branching pattern in 80.9% cases (95% confidence interval [CI] 76.3–82.4).[2] Another review of 20,030 cases estimated the prevalence of normal branching pattern as 84.52%.[3] Increased attention is being paid to study variations in the branching pattern of aortic arch since the advent of surgical and radiological interventional procedures on supra-aortic branches. One of the common congenital anomaly of AA is the presence of aberrant right subclavian artery (ARSA), which arises as the last branch of the arch to the left of the spine and generally ascends between the esophagus and spine. In such cases, the BT is absent, and the RCCA arises as the first branch and ARSA as the last branch. Clinicians name it as “Arteria Lusoria.” Although asymptomatic, its presence may cause dysphagia – Dysphagia lusoria. Reported prevalence is 0.16%–2.0% but varies widely between different ethnic groups.[4],[5] This anomaly has a preponderance in females.[6],[7] About 7%–10% of adults with this anomaly develop symptoms such as dysphagia, dyspnoea, retrosternal pain, chronic cough, and weight loss implying that this anomaly remains latent and innocuous in 90%–93% participants. It may be associated with some congenital anomalies such as patent ductus arteriosus, ventricular septal defect, and conotruncal anomalies.[8] The most common vascular anomalies associated with ARSA include bicarotid trunk (BCT) (common origin of both RCCA and LCCA), aortic arch origin of left vertebral artery (LVA), and Kommerell's diverticulum.[6]

Different types of aortic arch branching pattern associated with ARSA have been reported in the literature which is classified into three types by Adachi-Williams. In Type I or Type G, four branches arise from the arch in the order of RCCA, LCCA, LSA, and ARSA [Figure 1]a. In Type II or Type CG, five branches arise from the arch in the order of RCCA, LCCA, LVA, LSA, and ARSA [Figure 1]b. In Type III or Type H, three branched pattern is seen with BCT, LSA followed by ARSA [Figure 1]c.[5]
Figure 1: Schematic figure showing Adachi-William's classification of aberrant right subclavian artery. (a) Type I (Type-G) Four branched pattern in the sequence of RCCA, left common carotid artery, left subclavian artery and Aberrant right subclavian artery. (b) Type II (Type CG) Similar to Type I but with additional branch left vertebral artery arising in between the origin of left common carotid artery and left subclavian artery. (c) Type III (Type H) with three branches– the bicarotid trunk (common trunk of both common carotids), left subclavian artery and aberrant right subclavian artery

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Our aim was to assess the prevalence and associated branching anomalies of ARSA in Indian participants by contrast-enhanced multi-detector computed tomography (CT). Most of the Indian studies are cadaveric studies and case reports. Only few radiological reports are available. Hence, the present, retrospective, observational study was undertaken.


  Material and Methods Top


This retrospective observational study was done in 710 participants (males – 435 and females – 275) who underwent contrast-enhanced CT chest examination for various suspected lung and mediastinal pathologies during the period September 2015 to March 2018. The scans of the patients with malignancies likely to distort the anatomy of aorta and supra-aortic branches, poorly enhanced scans, and those with thoracic aortic disease were excluded leaving 710 participants suitable for the study. The imaging center routinely obtains informed consent before contrast injection. Volume rendered images and maximum intensity projection were obtained from the axial scans. The presence of ARSA, its course and relationship with esophagus, and associated vascular anomalies was noted.

Results

In 11 participants (males – 5 and females – 6) ARSA was noted arising as the last branch of the AA (1.54%; 11/710). Type I (Type G) which can be described as an isolated ARSA was observed in seven participants (males – 2 and females – 5) (0.98%; 7/710) [Figure 2]. Although overall incidence did not show much gender difference, the isolated ARSA has a predominance in females. In three participants (males-2; female-1) Type III (Type H) with BCT, LSA and ARSA was noted (0.42%; 3/710) [Figure 3] and [Figure 4]. In a male subject Type II (Type CG) was seen with the sequence of RCCA, LCCA, LVA, LSA, and ARSA (0.14%; 1/710) [Figure 5]. In all cases, ARSA had a retroesophageal course crossing the spine obliquely from left to right [Figure 6]. Kommerell's diverticulum was not observed in our study. In one case, the ARSA made an indentation in the esophagus and trachea [Figure 7]. Only two patients reported mild symptoms of dysphagia.
Figure 2: (a) Volume rendered image showing Type I Aberrant right subclavian artery with four branches from aortic arch. Note the absence of brachiocephalic trunk and the direct arch origin of right common carotid (RCCA) as the first branch. (b) Axial image showing the aberrant right subclavian artery passing to the right behind the esophagus. SVC: Superior vena cava

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Figure 3: Volume rendered image showing Type III Aberrant right subclavian artery with the Bicarotid trunk as the first branch followed by left subclavian artery and aberrant right subclavian artery

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Figure 4: Axial image showing Type III Aberrant right subclavian artery with bicarotid trunk, left subclavian artery and aberrant right subclavian artery sequence. Note the position of aberrant right subclavian artery between the esophagus and the vertebral body. SVC: Superior vena cava, LBCV: Left brachiocephalic vein

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Figure 5: Axial image showing Type II aberrant right subclavian artery with five branched pattern and presence of left vertebral artery between left common carotid artery and left subclavian artery. Aberrant right subclavian artery is passing to the right behind esophagus

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Figure 6: Coronal section showing the oblique course of aberrant right subclavian artery (arrow) in front of the spine to reach the right side

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Figure 7: Sagittal section in bone window showing aberrant right subclavian artery causing an indentation (arrow) in the esophagus and trachea

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  Discussion Top


ARSA, also named as retroesophageal right subclavian artery, arteria lusoria, arteria subclavia dextra lusoria, is a more frequently encountered congenital anomaly of aortic arch predominantly observed in females. Its presence is generally detected incidentally in cadaveric and autopsy studies, barium esophagogram, upper GI endoscopy, and many radiological modalities (such as conventional angiography, CT and magnetic resonance (MR) angiography, endoscopic ultrasound, endobronchial ultrasound, and transesophageal echocardiography). Developmentally, the aortic arch and its branches develop from pharyngeal arch arteries which appear symmetrically in the pharyngeal arches. Normally, the AA develops from left aortic sac, left fourth arch artery, and part of left dorsal aorta. Right subclavian artery develops from right fourth arch artery, proximal part of right dorsal aorta, and right seventh intersegmental artery. The part of right dorsal aorta distal to seventh intersegmental artery normally involutes. Abnormal involution of the right fourth arch artery and proximal right dorsal aorta and persistence of distal part of right dorsal aorta gives rise to ARSA as the last branch of AA [Figure 8].
Figure 8: Schematic diagram showing the developmental basis of aberrant right subclavian artery. (a) Normal development of right subclavian artery from right fourth arch artery, proximal right dorsal aorta and right seventh intersegmental artery. The distal part of right dorsal aorta normally involutes. Left fourth arch artery along with left dorsal aorta forms arch of aorta and left seventh intersegmental artery develops into left subclavian artery. Parts which disappear are shown in dotted lines. (b) Aberrant right subclavian artery develops due to abnormal involution of right fourth arch artery and proximal right dorsal aorta and persistence of distal right dorsal aorta. The common carotid arteries develop from proximal part of respective third arch arteries

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A recent systematic review and meta-analysis of 51 articles comprising 23,882 arches indicated that overall incidence of ARSA is 0.7% (95% CI is 0.2%–1.5%). If only imaging studies are included (32 articles, 20141 cases), the incidence is 0.9% (95% CI is 0.2–1.8), and in cadaveric studies (20 articles, 3740 cases), the incidence is 1.0% (95% CI 0.0–3.3).[2] Another systematic review analyzing 20,030 cases reported an incidence of 0.91% (183/20,030) out of which 176 cases had isolated ARSA and seven cases were accompanied by secondary arteries from the arch.[3] The present study reports a prevalence of 1.54% (11/710), which is within the range estimated by meta-analysis. The prevalence of ARSA exhibits racial differences and country-wise prevalence by radiological studies is given in [Table 1].
Table 1: Incidence of aberrant right subclavian artery in radiological studies

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Natsis et al. in their study on 267 Greek cadavers reported a prevalence of 2.2% (6/267), and in one cadaver, the ARSA had a course between the trachea and esophagus.[36] Reviewing 15 cadaveric studies initially, they estimated the incidence of ARSA as ranging from 0.2% to 13.3% but later modified it to 1.2% (range 0.19%–2.5%) after exclusion of four studies.[36],[37] Polednak using meta-analysis estimated the unweighted prevalence as 1.23% and pooled prevalence as 1.30% (95% CI 0.86%–1.82%).[38] Extracting data from the published reports Molz and Burri found a prevalence of 0.7% (593 ARSAs out of 68,049 autopsy cases) and 2.3% (517/22,201 patients).[7] Country-wise prevalence of ARSA in cadaveric studies is given in [Table 2].
Table 2: Incidence of aberrant right subclavian artery in cadaveric studies globally

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ARSA also shows gender differences in its prevalence as suggested by published literature. Polguj et al. analyzing 141 published reports suggested that gender distribution was 55.3% in females and 44.7% in males.[6] It is also suggested that isolated ARSA shows 58%–75% female predominance.[7] In our study also isolated ARSA has a female predominance (5 female versus 2 male).

One of the vascular anomaly most commonly associated with ARSA is the presence of BCT, present in 19.2% (27/141) cases with ARSA[6] This is classified as Type III (Type H) and its estimated prevalence is <0.05%.[47] We have observed this type in three participants (Male 2; Female-1; 0.42%). Natsis et al. reported two cases of this variant, both in males, in a study involving 72 Greek cadavers (2.78%; 2/72) and reviewed the earlier literature.[48] The combination of BCT with ARSA is mainly reported as case reports.[49],[50],[51] Gluncic and Marusic reported the presence of BCT, common trunk of left subclavian and left vertebral followed by ARSA in a female cadaver.[52] In another case report, the sequence of LSA as first branch, then BCT followed by ARSA was observed in a female patient.[53] It must be noted that the sequence of RSA, BCT, and LSA is also observed as a variant branching pattern of aortic arch.

Another rare vascular anomaly associated with ARSA is the five-branched pattern with RCCA, LCCA, LVA, LSA, and ARSA classified as Type II (Type CG) observed in 1 male patient (0.14%). This type was found in two cases (2/2370) by Wang et al.[29] and two cases (2/3460) by Choi et al.[34] In a cadaveric case report, an accessory LVA was found to arise directly from the aortic arch proximal to ARSA and the normal LVA from LSA was hypoplastic.[54] MR angiography of a female patient revealed a very rare branching pattern with BCT, LVA, LSA, and ARSA, and in this case, the right vertebral artery arose from RCCA.[55] Tsai et al. investigated the anomalies of vertebral and common carotid arteries in 102 patients with ARSA. They reported vertebral artery anomalies in 15.7% cases (16/102 patients) and common carotid trunk (BCT) in 20.6% cases (21/102 patients). They also noted that in 84.3% (86/102 patients) cases both vertebral arteries had normal origin from corresponding subclavian arteries.[56] In a most recent review, Plotkin et al. analyzed the data of 312 patients having aberrant subclavian arteries (both right and left) and studied the associated vascular anomalies and aortic pathology. The study included 281 ARSA patients and 31 aberrant LSA (ALSA) patients. In ARSA group the LVA had direct arch origin in 9 patients only and subclavian artery origin in 271 cases. On the contrary, in ALSA group, the LVA arose from LSA in all cases.[57]

Kommerell diverticulum (KD) appears as a dilatation at the origin of the ARSA and coexist in 14.9% cases.[6] In our study, none of the 11 patients exhibited KD. It was observed that KD was more common in men with aberrant subclavian arteries and was more frequently associated with ALSA in comparison to ARSA.[57]

Clinical implications

Awareness of the presence of ARSA is crucial for successful outcome of mediastinal, esophageal, and thoracic spine surgeries. Association with Kommerell's diverticulum and aneurysm of the diverticulum is a high-risk feature. The presence of this variant makes right radial artery approach for cardiac catheterization extremely difficult and unsuccessful. Manipulation of endoscopes and other instruments through the esophagus may damage this aberrant vessel leading to catastrophic bleeding. Pathological formation of arterio-esophageal fistula leads to life-threatening upper GI bleeding. This anomaly is associated with nonrecurrent inferior laryngeal nerve whose presence can complicate thyroid, parathyroid, and lower neck surgeries. May be associated with Di George syndrome, Trisomy-21 and some congenital cardiac anomalies such as tetralogy of Fallot and conotruncal anomalies. It has also been suggested that the presence of aberrant subclavian artery is an anatomical marker for 22.q11 deletion syndrome.


  Conclusion Top


Recent application of various endovascular, surgical, neurological, and radiological interventional procedures has kindled interest to evaluate branching pattern variations of aortic arch. ARSA is generally detected incidentally and its presence should alert the clinician to look for any associated vascular and cardiac anomalies. Multi-detector CT is the modality of choice as it accurately depicts vascular anomalies and can be used before planning any interventions.

Acknowledgment

The authors acknowledge the technical assistance rendered by Mr. Sushil Kumar of Muzaffarnagar Medical College, Muzaffarnagar and Mr. Pran Prakash of Jaswant Rai Superspeciality Hospital, Meerut in preparation of images.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]
 
 
    Tables

  [Table 1], [Table 2]



 

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Abstract
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