--- title: "Aberrant Right Subclavian Artery" docid: "a20702fe-5409-44a5-a804-cb071023bade" authors: - key: "770e1d77-2287-436e-910b-48232afc7842" value: "Prabhakar Rajiah, MBBS, MD, FACR, FRCR, FACC, FAHA, FSCCT" - key: "e915766e-8102-46e4-a33e-c83f8ae12f29" value: "Harold Goerne, MD" breadcrumbs: - name: "Cardiac" slug: "cardiac" treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39" - name: "Diagnosis" slug: "diagnosis" treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121" - name: "Aorta" slug: "aorta" treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7" - name: "Aberrant Right Subclavian Artery" slug: "aberrant-right-subclavian-artery" treeNodeId: null category: "Cardiac" documentVersionId: "69195035-5819-4996-b27d-b107c62d7c60" imageCount: 12 lastUpdated: "01/28/25" pageDescription: "Aberrant Right Subclavian Artery" pageKeywords: "Cardiac, Diagnosis, Aorta, Aberrant Right Subclavian Artery" pageTitle: "Aberrant Right Subclavian Artery | STATdx" enhancedTitle: "Aberrant Right Subclavian Artery" type: "DX" references: true breadcrumbs: - "Cardiac" - "Diagnosis" - "Aorta" - "Aberrant Right Subclavian Artery" --- # KEY FACTS - ## Terminology - Aberrant right subclavian artery (ARSA) as last branch of left-sided aortic arch, isthmus, or proximal descending aorta - Course: Retroesophageal 80%; intertracheoesophageal 15%; pretracheal 5% - ## Imaging - Barium esophagogram shows persistent esophageal narrowing at level of extrinsic compression with oblique course - CTA or MRA help to understand origin and course of supraaortic trunks - No vascular ring formed by left aortic arch and ARSA - Loose vascular ring if ARSA originates from Kommerell diverticulum - Complete vascular ring if ARSA with circumflex right descending thoracic aorta with right ductus - ## Top Differential Diagnoses - Major aortopulmonary collateral arteries (MAPCAs) - Right arch with aberrant left subclavian artery - Retroesophageal diverticulum - ## Pathology - Regression of right 4th arch between right subclavian and right common carotid arteries, including right ductus arteriosus - ## Clinical Issues - Most common congenital aortic arch abnormality (0.5-2.0%) - 90-95% asymptomatic; incidental imaging finding - Adults: Dysphagia (lusoria), dyspnea, back pain - Infants: Cough, stridor, aspiration pneumonia - Surgery for symptoms, aneurysm, or large diverticulum # TERMINOLOGY - ## Abbreviations - Aberrant right subclavian artery (ARSA) - ## Synonyms - Lusoria artery (arteria lusoria) - Incomplete vascular ring - ## Definitions - Aberrant origin of right subclavian artery as last branch of left-sided aortic arch or proximal descending aorta - Normally RSA originates from brachiocephalic artery # IMAGING - ## General Features - ### Best diagnostic clue - ARSA as last branch of left-sided aortic arch or proximal descending aorta - Retroesophageal/retrotracheal course of ARSA - ### Location - ARSA takeoff from distal aortic arch, isthmus, or proximal descending aorta - Course: Retroesophageal 80%; intertracheoesophageal 15%; pretracheal 5% - ### Size - Usually normal vessel caliber - Dilated proximal segment in Kommerell diverticulum - Aneurysm may be present - ### Morphology - No vascular ring formed by left aortic arch and ARSA - Trachea and esophagus are in middle between aortic arch and ARSA - Loose vascular ring if ARSA originates from Kommerell diverticulum - Right ductus/ligamentum arteriosum completes ring - Ductus arising from junction between diverticulum and normal-sized subclavian artery - Seen in 15-30% of ARSA - Complete vascular ring if ARSA with circumflex right descending thoracic aorta and right ductus - Arch itself crosses midline, posterior to esophagus - Right ductus extends from descending aorta to right pulmonary artery, completing vascular ring - ## Radiographic Findings - Chest x-ray may show enlargement of superior mediastinum - Barium esophagogram shows persistent esophageal narrowing at level of extrinsic compression with oblique course - ## CT Findings - ### CTA - Excellent modality to demonstrate aortic arch configuration and branching pattern - MPR, MIP, and VRT reconstructions help to understand origin and course of supraaortic trunks - Evaluates esophageal and tracheal compression - NECT: May be seen incidentally - 4D CT: May show dynamic esophageal compression during cardiac cycle due to distension of ARSA during systole - ## MR Findings - ### MRA - Arch vessel branching pattern is (from right to left): Right common carotid artery; left common carotid artery; left subclavian artery; right subclavian artery (RSA) - Course: Obliquely from caudal left to cranial right - Aneurysm of ARSA: 1.5x d of distal subclavian artery - Left ductus may be seen; no vascular ring - Since there is vasculature on only 3 sides of trachea and esophagus - Posterior esophageal compression in 10% - Usually in 4th or 5th decade - Stretching, sclerosis/calcification, aneurysm of ARSA - Complete vascular ring may be seen if - Kommerell diverticulum: Dilation of origin of ARSA - Diameter of orifice of diverticulum and distance from furthest aortic wall to end of diverticulum measured - Circumflex right descending thoracic aorta with arch behind esophagus - ### SSFP cine - May show dynamic esophageal compression during cardiac cycle due to vessel distension during systole - ## Imaging Recommendations - ### Best imaging tool - CTA or MRA - ### Protocol advice - ECG gating is not necessary if only evaluation of vascular ring is desired - High-pitch helical mode of dual source CT scanner (Flash) mode, provide anatomic information # DIFFERENTIAL DIAGNOSIS - ## Major Aortopulmonary Collateral Arteries - Collateral vessels arising from descending aorta that end in lungs in patients with pulmonary artery atresia - [Right Arch With Aberrant Left Subclavian Artery](/document/right-aortic-arch/5f186c96-4cc3-453e-840d-12ebfad13115) - Mirror image of left arch with ARSA - Caused by persistent right 4th arch and regression of left 4th arch in between left common carotid and left subclavian arteries - Aberrant left subclavian artery originates as last branch from right aortic arch and courses behind esophagus to reach left - 90% have left ductus vascular ring - 2nd most common cause of complete vascular ring - ## Retroesophageal Diverticulum - Outpouching at origin of aberrant left subclavian artery in right aortic arch; seen in 60% of these cases # PATHOLOGY - ## General Features - ### Etiology - Embryologic insult during formation of aortic arches - Right 4th arch regresses between RSA and right common carotid artery, including ductus arteriosus - Distal right dorsal aorta forms proximal RSA - Distal portion from 7th right intersegmental artery - Kommerell diverticulum: Remnant of dorsal aortic arch - Persistence of right 6th arch component that forms right ductus arteriosus - Left circumflex aorta - Regression of right 4th arch between right common carotid and right subclavian arteries - Persistence of right 6th arch component that forms ductus - Right-sided descending aorta - Distal left dorsal aorta forms definitive distal aortic arch - ### Associated abnormalities - ARSA is present in 35% of patients with Down syndrome - Kommerell diverticulum: 14.9% - Aneurysm of distal RSCA: 12.8% - Others: Coarctation; patent ductus arteriosus; ventricular septal defect; truncus bicaroticus; type B interrupted arch - ## Staging, Grading, & Classification - Retroesophageal course: 80% - Interesophageotracheal course: 15% - Pretracheal course: 5% # CLINICAL ISSUES - ## Presentation - ### Most common signs/symptoms - 90-95% asymptomatic; usually incidental finding in imaging studies - ### Other signs/symptoms - Adults: Dysphagia (lusoria), dyspnea, back pain, arm claudication, abnormal chest x-ray - Infants: Cough, stridor, occasionally aspiration pneumonia - Rare: Ruptured diverticulum; dissection - ## Demographics - Age: Any; mean at detection 41.8 ± 26.6 years - Sex: F:M = 3:1 - Epidemiology - Most common congenital aortic arch abnormality - Prevalence 0.5-2.0% of population - ## Natural History & Prognosis - Caution required during esophageal surgeries - Caution required in thyroid and parathyroid surgeries - Right inferior laryngeal nerve passes directly from vagus nerve at level of larynx to neck - ## Treatment - Surgery for symptoms, aneurysm, or large diverticulum - Diverticulum orifice > 3-cm or > 5-cm depth between wall adjacent to trachea and opposite aortic wall 6f7d98b5-f858-4f63-8153-640ab0b652fd ## References # Selected References 1. [Robb CL et al: Subclavian artery: anatomic review and imaging evaluation of abnormalities. Radiographics. 42(7):2149-65, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36053845%5Bpmid%5D) 1. [Mazzaccaro D et al: Analysis of origin of the supra-aortic trunks from the aortic arch. J Vasc Surg. 67(2):399-408, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=28830708%5Bpmid%5D) 1. [Hanneman K et al: Congenital variants and anomalies of the aortic arch. Radiographics. 37(1):32-51, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27860551%5Bpmid%5D) 1. [Polednak AP: Prevalence of the aberrant right subclavian artery reported in a published systematic review of cadaveric studies: the impact of an outlier. Clin Anat. 30(8):1024-8, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28514512%5Bpmid%5D) 1. [Allen D et al: Arteria lusoria: an anomalous finding during right transradial coronary intervention. Case Rep Cardiol. 2016:8079856, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27478652%5Bpmid%5D) 1. [Tanaka A et al: Kommerell's diverticulum in the current era: a comprehensive review. Gen Thorac Cardiovasc Surg. 63(5):245-59, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25636900%5Bpmid%5D) 1. [Etesami M et al: Computed tomography in the evaluation of vascular rings and slings. Insights Imaging. 5(4):507-21, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25008430%5Bpmid%5D) 1. [Mahmodlou R et al: Aberrant right subclavian artery: a life-threatening anomaly that should be considered during esophagectomy. J Surg Tech Case Rep. 6(2):61-3, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25598945%5Bpmid%5D) 1. [Roofthooft MT et al: Down syndrome and aberrant right subclavian artery. Eur J Pediatr. 167(9):1033-6, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18172685%5Bpmid%5D) 1. [Donnelly LF et al: Aberrant subclavian arteries: cross-sectional imaging findings in infants and children referred for evaluation of extrinsic airway compression. AJR 178:1269-74, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=11959744%5Bpmid%5D) ## Images ### Selected Images ![CTA cinematic rendering reconstruction shows 5 branches from the aortic arch. From the proximal to distal, the branches are: Right common carotid artery , left internal carotid artery , left external carotid artery , left subclavian artery , and aberrant right subclavian artery (ARSA) . Mild aortic coarctation is also noted.](images/app.statdx.com_image_thumbnail_87b003df-dee1-4d51-890f-1f947af7b590_annotated_true_size_900_quality_90_181333cebaf67cc8ff05e7b3df5c1183cab2cecf.jpg) *CTA cinematic rendering reconstruction shows 5 branches from the aortic arch. From the proximal to distal, the branches are: Right common carotid artery , left internal carotid artery , left external carotid artery , left subclavian artery , and aberrant right subclavian artery (ARSA) . Mild aortic coarctation is also noted.* ![CTA cinematic rendering reconstruction shows 5 branches from the aortic arch. From the proximal to distal, the branches are: Right common carotid artery , left internal carotid artery , left external carotid artery , left subclavian artery , and aberrant right subclavian artery (ARSA) . Mild aortic coarctation is also noted.](images/app.statdx.com_image_thumbnail_87b003df-dee1-4d51-890f-1f947af7b590_size_174_quality_85_dd72dcdebe2270b75139f261d01d291a567f9199.jpg) *CTA cinematic rendering reconstruction shows 5 branches from the aortic arch. From the proximal to distal, the branches are: Right common carotid artery , left internal carotid artery , left external carotid artery , left subclavian artery , and aberrant right subclavian artery (ARSA) . Mild aortic coarctation is also noted.* ![CTA cinematic rendering reconstruction shows ARSA that originates as the last branch from the aortic arch and courses behind the esophagus to reach the right.](images/app.statdx.com_image_thumbnail_5b07f1b3-a752-4095-ad8c-7ca7731742a9_annotated_true_size_900_quality_90_f3adb19d920434bdcd7536e7019c5ee36ace95e5.jpg) *CTA cinematic rendering reconstruction shows ARSA that originates as the last branch from the aortic arch and courses behind the esophagus to reach the right.* ![Coronal oblique MIP CTA shows a prominent Kommerell diverticulum , which is a dilatation of a proximal ARSA that originates in the proximal descending aorta .](images/app.statdx.com_image_thumbnail_e8369730-c3a0-4d3c-951e-64e97e28a790_annotated_true_size_900_quality_90_4744d1e5862c4927ed8e0f5e0b6da2df0ddf2438.jpg) *Coronal oblique MIP CTA shows a prominent Kommerell diverticulum , which is a dilatation of a proximal ARSA that originates in the proximal descending aorta .* ![Axial oblique MIP reconstruction from a CTA shows an ARSA with retroesophageal course causing esophageal compression between aortic arch, trachea , and ARSA.](images/app.statdx.com_image_thumbnail_0af47315-e009-418b-b6c2-32ebd3f16093_annotated_true_size_900_quality_90_8ff89f9fbc57fa49b018023c0278052b96bbcbc0.jpg) *Axial oblique MIP reconstruction from a CTA shows an ARSA with retroesophageal course causing esophageal compression between aortic arch, trachea , and ARSA.* ### Additional Images ![Axial oblique MIP reconstruction from a CTA shows an ARSA that causes esophageal compression .](images/app.statdx.com_image_thumbnail_2f68eef5-7229-4b15-80e8-48288b800bf6_annotated_true_size_900_quality_90_e7ec9c4ab51558f814d276c5587f2f7981a3ccf8.jpg) *Axial oblique MIP reconstruction from a CTA shows an ARSA that causes esophageal compression .* ![3D reconstruction of the airways and lungs from a CT in the same patient shows severe esophageal luminal narrowing from an ARSA.](images/app.statdx.com_image_thumbnail_cc7f9c26-8e09-4797-9d92-d945300d839d_annotated_true_size_900_quality_90_3f36fa7eba8d7d3f6aea48f7cad0a149f35b7111.jpg) *3D reconstruction of the airways and lungs from a CT in the same patient shows severe esophageal luminal narrowing from an ARSA.* ![MRA 3D posterior oblique view shows 6 branches from the aortic arch. From proximal to distal the branches are: A common trunk dividing into right common carotid artery and right vertebral artery , left internal carotid artery , left external carotid artery , left vertebral artery , left subclavian artery , and ARSA .](images/app.statdx.com_image_thumbnail_ee1ce812-91ce-4836-a7d8-26c425081e90_annotated_true_size_900_quality_90_5423ed25cea6e5dcb724ee235086d4bb1aa9edb7.jpg) *MRA 3D posterior oblique view shows 6 branches from the aortic arch. From proximal to distal the branches are: A common trunk dividing into right common carotid artery and right vertebral artery , left internal carotid artery , left external carotid artery , left vertebral artery , left subclavian artery , and ARSA .* ![Sagittal T2-weighted MR of the cervical spine shows a round structure between the spine and esophagus , consistent with an aberrant right subclavian artery causing esophageal compression.](images/app.statdx.com_image_thumbnail_356d60a2-4bd6-49f5-b42a-92cbf56ed310_annotated_true_size_900_quality_90_345fa28de59d4099fbe439e8bae8e6e6d0059ede.jpg) *Sagittal T2-weighted MR of the cervical spine shows a round structure between the spine and esophagus , consistent with an aberrant right subclavian artery causing esophageal compression.* ![Coronal MIP reconstruction from a MRA shows an ARSA and a normal left subclavian artery , both originating at the same level in the distal aortic arch .](c8b57a9a-fb9d-4ba0-8664-f0858766afd4) *Coronal MIP reconstruction from a MRA shows an ARSA and a normal left subclavian artery , both originating at the same level in the distal aortic arch .* ![CTA cinematic rendering reconstruction shows a right-sided aortic arch with mirror-image branching and aberrant left subclavian artery , which originates as the last branch from the arch.](a50bffb9-1de4-4290-a08b-d616bd9ab56e) *CTA cinematic rendering reconstruction shows a right-sided aortic arch with mirror-image branching and aberrant left subclavian artery , which originates as the last branch from the arch.* ![Coronal MIP CTA reconstruction in a 56-year-old man shows an ARSA originating from the proximal descending thoracic aorta and extending towards the right.](026a4678-e026-4f81-8710-5a5d578c85a4) *Coronal MIP CTA reconstruction in a 56-year-old man shows an ARSA originating from the proximal descending thoracic aorta and extending towards the right.* ![Axial MIP CTA reconstruction in a 56-year-old man shows an ARSA originating from the proximal descending thoracic aorta and extending towards the right, with compression of the esophagus .](3bbf98f6-e0dc-4c40-84d4-f932dab7ea36) *Axial MIP CTA reconstruction in a 56-year-old man shows an ARSA originating from the proximal descending thoracic aorta and extending towards the right, with compression of the esophagus .*