327 lines
21 KiB
Markdown
327 lines
21 KiB
Markdown
---
|
||
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 <img src='img/arrows/WS.png'/>, left internal carotid artery <img src='img/arrows/CO.png'/>, left external carotid artery <img src='img/arrows/CC.png'/>, left subclavian artery <img src='img/arrows/WO.png'/>, and aberrant right subclavian artery (ARSA) <img src='img/arrows/WC.png'/>. Mild aortic coarctation <img src='img/arrows/CS.png'/> 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 <img src='img/arrows/WS.png'/>, left internal carotid artery <img src='img/arrows/CO.png'/>, left external carotid artery <img src='img/arrows/CC.png'/>, left subclavian artery <img src='img/arrows/WO.png'/>, and aberrant right subclavian artery (ARSA) <img src='img/arrows/WC.png'/>. Mild aortic coarctation <img src='img/arrows/CS.png'/> is also noted.*
|
||
|
||
|
||
*CTA cinematic rendering reconstruction shows ARSA <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WS.png'/>, which is a dilatation of a proximal ARSA <img src='img/arrows/WO.png'/> that originates in the proximal descending aorta <img src='img/arrows/WC.png'/>.*
|
||
|
||
|
||
*Axial oblique MIP reconstruction from a CTA shows an ARSA <img src='img/arrows/WS.png'/> with retroesophageal course causing esophageal compression <img src='img/arrows/WO.png'/> between aortic arch, trachea <img src='img/arrows/CS.png'/>, and ARSA.*
|
||
|
||
|
||
### Additional Images
|
||
|
||
|
||
*Axial oblique MIP reconstruction from a CTA shows an ARSA <img src='img/arrows/WS.png'/> that causes esophageal compression <img src='img/arrows/WO.png'/>.*
|
||
|
||
|
||
*3D reconstruction of the airways and lungs from a CT in the same patient shows severe esophageal luminal narrowing <img src='img/arrows/WS.png'/> 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 <img src='img/arrows/WS.png'/> dividing into right common carotid artery <img src='img/arrows/BS.png'/> and right vertebral artery <img src='img/arrows/BO.png'/>, left internal carotid artery <img src='img/arrows/CC.png'/>, left external carotid artery <img src='img/arrows/BC.png'/>, left vertebral artery <img src='img/arrows/WO.png'/>, left subclavian artery <img src='img/arrows/WC.png'/>, and ARSA <img src='img/arrows/CS.png'/>.*
|
||
|
||
|
||
*Sagittal T2-weighted MR of the cervical spine shows a round structure between the spine <img src='img/arrows/WO.png'/> and esophagus <img src='img/arrows/CO.png'/>, consistent with an aberrant right subclavian artery <img src='img/arrows/WS.png'/> causing esophageal compression.*
|
||
|
||
|
||
*Coronal MIP reconstruction from a MRA shows an ARSA <img src='img/arrows/WS.png'/> and a normal left subclavian artery <img src='img/arrows/WO.png'/>, both originating at the same level in the distal aortic arch <img src='img/arrows/CO.png'/>.*
|
||
|
||
|
||
*CTA cinematic rendering reconstruction shows a right-sided aortic arch <img src='img/arrows/CO.png'/> with mirror-image branching <img src='img/arrows/WS.png'/> and aberrant left subclavian artery <img src='img/arrows/WO.png'/>, which originates as the last branch from the arch.*
|
||
|
||
|
||
*Coronal MIP CTA reconstruction in a 56-year-old man shows an ARSA <img src='img/arrows/CS.png'/> originating from the proximal descending thoracic aorta <img src='img/arrows/WO.png'/> and extending towards the right.*
|
||
|
||
|
||
*Axial MIP CTA reconstruction in a 56-year-old man shows an ARSA <img src='img/arrows/CS.png'/> originating from the proximal descending thoracic aorta <img src='img/arrows/WO.png'/> and extending towards the right, with compression of the esophagus <img src='img/arrows/WS.png'/>.*
|
||
|