340 lines
24 KiB
Markdown
340 lines
24 KiB
Markdown
---
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title: "Double Aortic Arch"
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docid: "0b68477f-f05a-4e4c-a1a8-02fe29c292db"
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authors:
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- key: "ee6ece9d-ad74-458c-a8df-11628ae7f879"
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value: "Arzu Canan, MD"
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- key: "10bb95ac-a27a-4ebe-833b-e59fea07734b"
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value: "Santiago Mart\u00ednez-Jim\u00e9nez, MD, FACR"
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breadcrumbs:
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-
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name: "Cardiac"
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slug: "cardiac"
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treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
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-
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name: "Diagnosis"
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slug: "diagnosis"
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treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
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-
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name: "Aorta"
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slug: "aorta"
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treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
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-
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name: "Double Aortic Arch"
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slug: "double-aortic-arch"
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treeNodeId: null
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category: "Cardiac"
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documentVersionId: "3dc4caa1-09b8-4045-b792-da5ec170e56b"
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imageCount: 19
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lastUpdated: "03/09/25"
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pageDescription: "Double Aortic Arch"
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pageKeywords: "Cardiac, Diagnosis, Aorta, Double Aortic Arch"
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pageTitle: "Double Aortic Arch | STATdx"
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enhancedTitle: "Double Aortic Arch"
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type: "DX"
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references: true
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breadcrumbs:
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- "Cardiac"
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- "Diagnosis"
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- "Aorta"
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- "Double Aortic Arch"
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---
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# KEY FACTS
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- ## Terminology
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- Double aortic arch (DAA)
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- Persistent right and left aortic arches, each one giving rise to separate ipsilateral subclavian and carotid arteries
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- ## Imaging
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- Chest radiography
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- Frontal projection: Bilateral paratracheal opacities, bilateral tracheal indentations
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- Lateral projection: Posterior tracheal indentation
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- CTA
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- Right aortic arch
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- Larger and higher in most patients (right dominant)
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- Left aortic arch
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- Often smaller than right aortic arch
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- 4-artery sign: Symmetric take-off of 4 aortic branches on axial image at thoracic inlet (2 ventral carotids and 2 dorsal subclavians)
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- 1 descending aorta, usually contralateral to dominant arch (i.e., left)
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- Airway CT
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- Tracheomalacia: Tracheal collapse adjacent to vascular ring during expiration
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- Bronchomalacia: Left main bronchus collapse adjacent to midline descending aorta during expiration
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- ## Clinical Issues
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- Most common symptomatic vascular ring
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- Typically manifests in neonates
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- Children
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- Dyspnea, often during feeding
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- Stridor and wheezing (exacerbated by crying)
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- Tachypnea, apnea
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- Adults
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- May be asymptomatic
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- Treatment: Surgical division of smaller or atretic aortic arch and ligamentum arteriosus
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# TERMINOLOGY
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- ## Abbreviations
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- Double aortic arch (DAA)
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- ## Definitions
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- Persistent right and left aortic arches, each one giving rise to separate ipsilateral subclavian and carotid arteries
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- Variants
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- Both arches patent and functioning
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- Right arch patent, left arch atretic
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- Difficult to differentiate from right aortic arch mirror image branching
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- Chest radiography: Bilateral paratracheal opacities with concentric midtracheal narrowing
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- ## Radiographic Findings
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- ### Radiography
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- Frontal projection
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- Bilateral paratracheal opacities
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- Bilateral tracheal indentations
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- Lateral
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- Posterior tracheal indentation
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- ## Fluoroscopic Findings
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- ### Esophagram
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- Frontal projection: S-shaped, bilateral indentations on contrast-filled esophagus, right higher and larger than left
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- Lateral view: Large posterior indentation, often oblique
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- ## CT Findings
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- ### CTA
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- Right aortic arch
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- Larger in most patients (right dominant)
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- More cephalad than left
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- Courses behind esophagus
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- Left aortic arch
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- Often smaller than right aortic arch
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- Rarely same size (codominant) or larger (left dominant)
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- Left aortic arch atresia can be confused with right aortic arch
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- Inferior tethering of left subclavian artery
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- ± aortic diverticulum
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- Posterior course of proximal head and neck vessels
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- Focal narrowing of airway
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- **4-artery sign**: Symmetric take-off of 4 aortic branches on axial image at thoracic inlet (2 ventral carotids and 2 dorsal subclavians)
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- 1 descending aorta, usually contralateral to dominant arch (i.e., left)
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- Airway CT
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- Inspiration and expiration CT may help differentiate tracheomalacia from tracheal stenosis
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- Tracheomalacia: Tracheal collapse adjacent to vascular ring during expiration
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- Bronchomalacia: Left main bronchus collapse adjacent to midline descending aorta during expiration
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- ## MR Findings
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- As accurate as CT in assessing vascular anatomy and tracheal stenosis
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- Of value in young individuals due to lack of ionizing radiation
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- Same findings as CT
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- ## Echocardiographic Findings
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- ### Echocardiogram
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- Suprasternal notch view is most helpful, showing 2 separate aortic arches, each giving rise to separate carotid and subclavian arteries
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- Poor assessment of airway compression
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- ## Imaging Recommendations
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- ### Best imaging tool
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- MR and CT are equally accurate in assessing vascular and tracheal anatomy
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- ### Protocol advice
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- Multiplanar reformations are helpful in delineating arch anatomy and tracheal abnormalities
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# DIFFERENTIAL DIAGNOSIS
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- ## Right Aortic Arch With Aberrant Left Subclavian Artery and Kommerell Diverticulum
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- Kommerell diverticulum may mimic left aortic arch on frontal chest radiograph
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- Tracheal indentation on lateral chest radiograph
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- Differentiation usually requires cross-sectional imaging
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- ## Right Aortic Arch With Mirror Image Branching and Aortic Diverticulum
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- Lack of inferior tethering of left subclavian artery
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- Aortic diverticulum is more common in DAA with atretic left aortic arch
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- DAA with atretic left aortic arch and right aortic arch with mirror image branching and aortic diverticulum are part of spectrum of vascular rings and have similar clinical implications
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- ## Left Pulmonary Artery Sling
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- Anterior esophageal and posterior tracheal indentations
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- May be associated with tracheomalacia
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- [Innominate Artery Compression Syndrome](/document/innominate-artery-compression-synd-/bc9f2319-6c01-4218-b75b-3481b2461738)
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- Anterior tracheal indentation without esophageal compression
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- ## Mediastinal Mass
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- Mediastinal masses can cause tracheal compression
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# PATHOLOGY
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- ## General Features
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- ### Etiology
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- Persistence of right and left 4th aortic arches
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- ### Associated abnormalities
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- Often not associated with congenital heart disease
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- 20% associated with congenital heart disease
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- Tetralogy of Fallot (most common)
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- Ventricular septal defect
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- Aortic coarctation
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- Patent ductus arteriosus
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- Transposition of great arteries
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- Truncus arteriosus
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- Tracheobronchomalacia
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- May cause persistent airways symptoms after surgery
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- ## Gross Pathologic & Surgical Features
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- Tight vascular ring with tracheal and esophageal compression
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- Dominance: Right (~ 70%) > left (~ 20%) > codominant (~ 5%)
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- Smaller left arch may be partially atretic
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- Children
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- Dyspnea, often during feeding
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- Stridor and wheezing (exacerbated by crying)
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- Tachypnea, apnea
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- Recurrent respiratory infections
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- Dysphagia
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- Adults
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- May be asymptomatic
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- Esophageal obstruction (i.e., dysphagia)
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- ## Demographics
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- Most common symptomatic vascular ring
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- Typically manifests in neonates
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- Affects 0.05-0.3% of general population
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- ## Treatment
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- Surgical division of smaller or atretic aortic arch and ligamentum arteriosus
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af9c9eb2-551e-4968-a9d6-7738cac6154b
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## References
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# Selected References
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1. [Gikandi A et al: Outcomes of patients undergoing surgery for complete vascular rings. J Am Coll Cardiol. 84(14):1279-92, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39322321%5Bpmid%5D)
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1. [Li S et al: Congenital abnormalities of the aortic arch: revisiting the 1964 Stewart classification. Cardiovasc Pathol. 39:38-50, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30623879%5Bpmid%5D)
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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)
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1. [Newman B et al: Persistent fifth arch anomalies - broadening the spectrum to include a variation of double aortic arch vascular ring. Pediatr Radiol. 46(13):1866-72, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27669708%5Bpmid%5D)
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1. [Ramos-Duran L et al: Developmental aortic arch anomalies in infants and children assessed with CT angiography. AJR Am J Roentgenol. 198(5):W466-74, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22528928%5Bpmid%5D)
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1. [Dillman JR et al: Common and uncommon vascular rings and slings: a multi-modality review. Pediatr Radiol. 41(11):1440-54; quiz 1489-90, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21594540%5Bpmid%5D)
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1. [Kanne JP et al: Right aortic arch and its variants. J Cardiovasc Comput Tomogr. 4(5):293-300, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20829147%5Bpmid%5D)
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1. [Kellenberger CJ: Aortic arch malformations. Pediatr Radiol. 40(6):876-84, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20354848%5Bpmid%5D)
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1. [Holmes KW et al: Magnetic resonance imaging of a distorted left subclavian artery course: an important clue to an unusual type of double aortic arch. Pediatr Cardiol. 27(3):316-20, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16565909%5Bpmid%5D)
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1. [Chan MS et al: Angiography and dynamic airway evaluation with MDCT in the diagnosis of double aortic arch associated with tracheomalacia. AJR Am J Roentgenol. 185(5):1248-51, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16247144%5Bpmid%5D)
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## Images
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### Selected Images
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*Composite axial CTA at contiguous levels in a patient with a double aortic arch (DAA) shows symmetric take-off of 4 aortic branches <img src='img/arrows/CS.png'/> at thoracic inlet (i.e., 2 carotids and 2 subclavian arteries), the so-called 4-artery sign. This sign has been described in the setting of DAA. Note the right aortic arch (RAA) <img src='img/arrows/CC.png'/>.*
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*Composite axial CTA at contiguous levels in a patient with a double aortic arch (DAA) shows symmetric take-off of 4 aortic branches <img src='img/arrows/CS.png'/> at thoracic inlet (i.e., 2 carotids and 2 subclavian arteries), the so-called 4-artery sign. This sign has been described in the setting of DAA. Note the right aortic arch (RAA) <img src='img/arrows/CC.png'/>.*
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*Composite axial CTA in the same patient shows the larger RAA <img src='img/arrows/CC.png'/> and smaller left aortic arch <img src='img/arrows/CO.png'/>. Note the left descending thoracic aorta <img src='img/arrows/CS.png'/>, which is typically contralateral to the dominant arch.*
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*Coronal CTA in the same patient shows a cephalad, larger RAA <img src='img/arrows/WS.png'/> and a more caudal, smaller left aortic arch <img src='img/arrows/WC.png'/>. Note the mild tracheal impression of the RAA. A larger RAA is the most common variant seen in DAA.*
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*Sagittal CTA in the same patient shows the distal RAA <img src='img/arrows/BS.png'/> causing a posterior indentation of the trachea. Also note the ascending aorta (with slab artifact) <img src='img/arrows/WO.png'/> and the proximal left aortic arch <img src='img/arrows/BC.png'/>.*
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*Frontal radiograph in the same patient shows mild concentric narrowing <img src='img/arrows/BS.png'/> of the midtrachea with more prominent right paratracheal nodular opacity.*
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*Lateral radiograph in the same patient shows the posterior tracheal indentation <img src='img/arrows/BS.png'/> from retrotracheal course of the RAA, a classic finding in DAA. This narrowing is often related to the distal portion of the RAA, as it courses posterior to the esophagus to join the RAA.*
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*Sagittal 3D reformation of chest CTA shows a patent RAA <img src='img/arrows/WC.png'/> and smaller left aortic arch <img src='img/arrows/WS.png'/>. There are 4 major symmetrical branches (2 ventral carotids and 2 dorsal subclavian arteries), each set arising form each aortic arch. This is known as the 4-artery sign. The trachea and esophagus (not shown) are completely surrounded by the vascular ring.*
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*Graphic shows a DAA with a complete vascular ring encircling and compressing the trachea and esophagus.*
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*Esophagram in a neonate with stridor shows right <img src='img/arrows/BC.png'/> and smaller left <img src='img/arrows/BS.png'/> indentations of the esophagus on frontal view due to a DAA. There is posterior indentation <img src='img/arrows/BO.png'/> in the lateral view related to the RAA.*
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*Frontal 3D reformation from a chest CT in an asymptomatic patient with a DAA shows higher and larger right <img src='img/arrows/BS.png'/> vs. left <img src='img/arrows/BC.png'/> tracheal indentations on the AP reformation. Note posterior indentation <img src='img/arrows/BO.png'/> related to the RAA in the lateral reformation.*
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*Lateral chest radiograph in a patient with a DAA shows abnormal posterior tracheal indentation <img src='img/arrows/CS.png'/>. This can also be seen in the setting of other vascular rings, such as those with diverticulum of Kommerell or pulmonary artery sling.*
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*Composite axial CTA at contiguous levels shows the RAA <img src='img/arrows/CC.png'/> and left aortic arch <img src='img/arrows/CO.png'/> similar in size. Note, however, the lack of the 4-artery sign (i.e., lack of symmetry of the 4 head/neck vessels at the thoracic inlet <img src='img/arrows/CS.png'/>). There is a left descending thoracic aorta <img src='img/arrows/WC.png'/>.*
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*Frontal chest radiograph in a patient with a DAA with an atretic left arch shows right paratracheal opacity <img src='img/arrows/CS.png'/> related to the RAA with marked tracheal indentation <img src='img/arrows/CC.png'/>. The same imaging finding can also be seen in an isolated RAA.*
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*Composite axial CTA at contiguous levels in the same patient shows symmetric take-off of 4 aortic branches <img src='img/arrows/CS.png'/> at the thoracic inlet (i.e., 4-artery sign). Note the presence of a large RAA <img src='img/arrows/CC.png'/>, which is mildly deviating the trachea to the left.*
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*Composite axial CTA at contiguous levels in the same patient shows an atretic left aortic arch <img src='img/arrows/CO.png'/> with a posteriorly tethered left subclavian artery <img src='img/arrows/CC.png'/>. The later is helpful to differentiate from an RAA with mirror image branching in which the take-off of the left subclavian artery tends to be more anterior.*
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*Sagittal oblique SSD CTA in the same patient shows the posterior tethering of the left subclavian artery <img src='img/arrows/CS.png'/> and the atretic left aortic arch <img src='img/arrows/CC.png'/>, resulting in a vascular ring.*
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### Additional Images
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*Axial chest CTA in an asymptomatic adult patient with a DAA with areas of partially atretic left aortic arch (cephalad to caudad progression) demonstrates a RAA <img src='img/arrows/WS.png'/> and a left aortic arch <img src='img/arrows/WC.png'/>.*
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*Axial chest CTA in the same patient shows that the RAA and left aortic arch have joined into 1 descending thoracic aorta <img src='img/arrows/WS.png'/>. The trachea remains slightly narrowed.*
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*Axial chest CTA more inferiorly shows a common left descending thoracic aorta <img src='img/arrows/WS.png'/>. The trachea now resumes a normal diameter.*
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