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Double Aortic Arch 0b68477f-f05a-4e4c-a1a8-02fe29c292db
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ee6ece9d-ad74-458c-a8df-11628ae7f879 Arzu Canan, MD
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10bb95ac-a27a-4ebe-833b-e59fea07734b Santiago Martínez-Jiménez, MD, FACR
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Cardiac cardiac fa90100b-619c-430e-8074-b5b9789bab39
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Cardiac 3dc4caa1-09b8-4045-b792-da5ec170e56b 19 03/09/25 Double Aortic Arch Cardiac, Diagnosis, Aorta, Double Aortic Arch Double Aortic Arch | STATdx Double Aortic Arch DX true
Cardiac
Diagnosis
Aorta
Double Aortic Arch

title: "Double Aortic Arch" docid: "0b68477f-f05a-4e4c-a1a8-02fe29c292db" authors:

  • key: "ee6ece9d-ad74-458c-a8df-11628ae7f879" value: "Arzu Canan, MD"
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  • "Cardiac"
  • "Diagnosis"
  • "Aorta"
  • "Double Aortic Arch"

KEY FACTS

  • Terminology

    • Double aortic arch (DAA) - Persistent right and left aortic arches, each one giving rise to separate ipsilateral subclavian and carotid arteries

  • Imaging

    • Chest radiography - Frontal projection: Bilateral paratracheal opacities, bilateral tracheal indentations - Lateral projection: Posterior tracheal indentation
    • CTA - Right aortic arch - Larger and higher in most patients (right dominant) - Left aortic arch - Often smaller than right aortic arch - 4-artery sign: Symmetric take-off of 4 aortic branches on axial image at thoracic inlet (2 ventral carotids and 2 dorsal subclavians) - 1 descending aorta, usually contralateral to dominant arch (i.e., left)
    • Airway CT - Tracheomalacia: Tracheal collapse adjacent to vascular ring during expiration - Bronchomalacia: Left main bronchus collapse adjacent to midline descending aorta during expiration

  • Clinical Issues

    • Most common symptomatic vascular ring
    • Typically manifests in neonates
    • Children - Dyspnea, often during feeding - Stridor and wheezing (exacerbated by crying) - Tachypnea, apnea
    • Adults - May be asymptomatic
    • Treatment: Surgical division of smaller or atretic aortic arch and ligamentum arteriosus

TERMINOLOGY

  • Abbreviations

    • Double aortic arch (DAA)

  • Definitions

    • Persistent right and left aortic arches, each one giving rise to separate ipsilateral subclavian and carotid arteries
    • Variants - Both arches patent and functioning - Right arch patent, left arch atretic - Difficult to differentiate from right aortic arch mirror image branching

IMAGING

  • General Features

    • Best diagnostic clue

      - Chest radiography: Bilateral paratracheal opacities with concentric midtracheal narrowing

  • Radiographic Findings

    • Radiography

      - Frontal projection
        - Bilateral paratracheal opacities
        - Bilateral tracheal indentations
- Lateral
        - Posterior tracheal indentation

  • Fluoroscopic Findings

    • Esophagram

      - Frontal projection: S-shaped, bilateral indentations on contrast-filled esophagus, right higher and larger than left
- Lateral view: Large posterior indentation, often oblique

  • CT Findings

    • CTA

      - Right aortic arch
        - Larger in most patients (right dominant)
        - More cephalad than left
        - Courses behind esophagus
- Left aortic arch
        - Often smaller than right aortic arch
        - Rarely same size (codominant) or larger (left dominant)
        - Left aortic arch atresia can be confused with right aortic arch
                  - Inferior tethering of left subclavian artery
                  - ± aortic diverticulum
                  - Posterior course of proximal head and neck vessels
                  - Focal narrowing of airway
- **4-artery sign**: Symmetric take-off of 4 aortic branches on axial image at thoracic inlet (2 ventral carotids and 2 dorsal subclavians)
- 1 descending aorta, usually contralateral to dominant arch (i.e., left)

    • Airway CT - Inspiration and expiration CT may help differentiate tracheomalacia from tracheal stenosis - Tracheomalacia: Tracheal collapse adjacent to vascular ring during expiration - Bronchomalacia: Left main bronchus collapse adjacent to midline descending aorta during expiration

  • MR Findings

    • As accurate as CT in assessing vascular anatomy and tracheal stenosis
    • Of value in young individuals due to lack of ionizing radiation
    • Same findings as CT

  • Echocardiographic Findings

    • Echocardiogram

      - Suprasternal notch view is most helpful, showing 2 separate aortic arches, each giving rise to separate carotid and subclavian arteries
- Poor assessment of airway compression

  • Imaging Recommendations

    • Best imaging tool

      - MR and CT are equally accurate in assessing vascular and tracheal anatomy

    • Protocol advice

      - Multiplanar reformations are helpful in delineating arch anatomy and tracheal abnormalities

DIFFERENTIAL DIAGNOSIS

  • Right Aortic Arch With Aberrant Left Subclavian Artery and Kommerell Diverticulum

    • Kommerell diverticulum may mimic left aortic arch on frontal chest radiograph
    • Tracheal indentation on lateral chest radiograph
    • Differentiation usually requires cross-sectional imaging

  • Right Aortic Arch With Mirror Image Branching and Aortic Diverticulum

    • Lack of inferior tethering of left subclavian artery
    • Aortic diverticulum is more common in DAA with atretic left aortic arch
    • 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

  • Left Pulmonary Artery Sling

    • Anterior esophageal and posterior tracheal indentations
    • May be associated with tracheomalacia

  • Innominate Artery Compression Syndrome

    • Anterior tracheal indentation without esophageal compression

  • Mediastinal Mass

    • Mediastinal masses can cause tracheal compression

PATHOLOGY

  • General Features

    • Etiology

      - Persistence of right and left 4th aortic arches

    • Associated abnormalities

      - Often not associated with congenital heart disease
- 20% associated with congenital heart disease
        - Tetralogy of Fallot (most common)
        - Ventricular septal defect
        - Aortic coarctation
        - Patent ductus arteriosus
        - Transposition of great arteries
        - Truncus arteriosus
- Tracheobronchomalacia
        - May cause persistent airways symptoms after surgery

  • Gross Pathologic & Surgical Features

    • Tight vascular ring with tracheal and esophageal compression
    • Dominance: Right (~ 70%) > left (~ 20%) > codominant (~ 5%)
    • Smaller left arch may be partially atretic

CLINICAL ISSUES

  • Presentation

    • Most common signs/symptoms

      - Children
        - Dyspnea, often during feeding
        - Stridor and wheezing (exacerbated by crying)
        - Tachypnea, apnea
        - Recurrent respiratory infections
        - Dysphagia
- Adults
        - May be asymptomatic
        - Esophageal obstruction (i.e., dysphagia)

  • Demographics

    • Most common symptomatic vascular ring
    • Typically manifests in neonates
    • Affects 0.05-0.3% of general population

  • Treatment

    • Surgical division of smaller or atretic aortic arch and ligamentum arteriosus

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References

Selected References

  1. Gikandi A et al: Outcomes of patients undergoing surgery for complete vascular rings. J Am Coll Cardiol. 84(14):1279-92, 2024
  2. Li S et al: Congenital abnormalities of the aortic arch: revisiting the 1964 Stewart classification. Cardiovasc Pathol. 39:38-50, 2018
  3. Hanneman K et al: Congenital variants and anomalies of the aortic arch. Radiographics. 37(1):32-51, 2017
  4. 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
  5. 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
  6. 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
  7. Kanne JP et al: Right aortic arch and its variants. J Cardiovasc Comput Tomogr. 4(5):293-300, 2010
  8. Kellenberger CJ: Aortic arch malformations. Pediatr Radiol. 40(6):876-84, 2010
  9. 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
  10. 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

Images

Selected Images

Composite axial CTA at contiguous levels in a patient with a double aortic arch (DAA) shows symmetric take-off of 4 aortic branches 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) . Composite axial CTA at contiguous levels in a patient with a double aortic arch (DAA) shows symmetric take-off of 4 aortic branches 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) .

Composite axial CTA at contiguous levels in a patient with a double aortic arch (DAA) shows symmetric take-off of 4 aortic branches 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) . Composite axial CTA at contiguous levels in a patient with a double aortic arch (DAA) shows symmetric take-off of 4 aortic branches 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) .

Composite axial CTA in the same patient shows the larger RAA and smaller left aortic arch . Note the left descending thoracic aorta , which is typically contralateral to the dominant arch. Composite axial CTA in the same patient shows the larger RAA and smaller left aortic arch . Note the left descending thoracic aorta , which is typically contralateral to the dominant arch.

Coronal CTA in the same patient shows a cephalad, larger RAA and a more caudal, smaller left aortic arch . Note the mild tracheal impression of the RAA. A larger RAA is the most common variant seen in DAA. Coronal CTA in the same patient shows a cephalad, larger RAA and a more caudal, smaller left aortic arch . Note the mild tracheal impression of the RAA. A larger RAA is the most common variant seen in DAA.

Sagittal CTA in the same patient shows the distal RAA causing a posterior indentation of the trachea. Also note the ascending aorta (with slab artifact) and the proximal left aortic arch . Sagittal CTA in the same patient shows the distal RAA causing a posterior indentation of the trachea. Also note the ascending aorta (with slab artifact) and the proximal left aortic arch .

Frontal radiograph in the same patient shows mild concentric narrowing of the midtrachea with more prominent right paratracheal nodular opacity. Frontal radiograph in the same patient shows mild concentric narrowing of the midtrachea with more prominent right paratracheal nodular opacity.

Lateral radiograph in the same patient shows the posterior tracheal indentation 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. Lateral radiograph in the same patient shows the posterior tracheal indentation 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.

Sagittal 3D reformation of chest CTA shows a patent RAA and smaller left aortic arch . 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. Sagittal 3D reformation of chest CTA shows a patent RAA and smaller left aortic arch . 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.

Graphic shows a DAA with a complete vascular ring encircling and compressing the trachea and esophagus. Graphic shows a DAA with a complete vascular ring encircling and compressing the trachea and esophagus.

Esophagram in a neonate with stridor shows right and smaller left indentations of the esophagus on frontal view due to a DAA. There is posterior indentation in the lateral view related to the RAA. Esophagram in a neonate with stridor shows right and smaller left indentations of the esophagus on frontal view due to a DAA. There is posterior indentation in the lateral view related to the RAA.

Frontal 3D reformation from a chest CT in an asymptomatic patient with a DAA shows higher and larger right vs. left tracheal indentations on the AP reformation. Note posterior indentation related to the RAA in the lateral reformation. Frontal 3D reformation from a chest CT in an asymptomatic patient with a DAA shows higher and larger right vs. left tracheal indentations on the AP reformation. Note posterior indentation related to the RAA in the lateral reformation.

Lateral chest radiograph in a patient with a DAA shows abnormal posterior tracheal indentation . This can also be seen in the setting of other vascular rings, such as those with diverticulum of Kommerell or pulmonary artery sling. Lateral chest radiograph in a patient with a DAA shows abnormal posterior tracheal indentation . This can also be seen in the setting of other vascular rings, such as those with diverticulum of Kommerell or pulmonary artery sling.

Composite axial CTA at contiguous levels shows the RAA and left aortic arch 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 ). There is a left descending thoracic aorta . Composite axial CTA at contiguous levels shows the RAA and left aortic arch 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 ). There is a left descending thoracic aorta .

Frontal chest radiograph in a patient with a DAA with an atretic left arch shows right paratracheal opacity related to the RAA with marked tracheal indentation . The same imaging finding can also be seen in an isolated RAA. Frontal chest radiograph in a patient with a DAA with an atretic left arch shows right paratracheal opacity related to the RAA with marked tracheal indentation . The same imaging finding can also be seen in an isolated RAA.

Composite axial CTA at contiguous levels in the same patient shows symmetric take-off of 4 aortic branches at the thoracic inlet (i.e., 4-artery sign). Note the presence of a large RAA , which is mildly deviating the trachea to the left. Composite axial CTA at contiguous levels in the same patient shows symmetric take-off of 4 aortic branches at the thoracic inlet (i.e., 4-artery sign). Note the presence of a large RAA , which is mildly deviating the trachea to the left.

Composite axial CTA at contiguous levels in the same patient shows an atretic left aortic arch with a posteriorly tethered left subclavian artery . 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. Composite axial CTA at contiguous levels in the same patient shows an atretic left aortic arch with a posteriorly tethered left subclavian artery . 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.

Sagittal oblique SSD CTA in the same patient shows the posterior tethering of the left subclavian artery and the atretic left aortic arch , resulting in a vascular ring. Sagittal oblique SSD CTA in the same patient shows the posterior tethering of the left subclavian artery and the atretic left aortic arch , resulting in a vascular ring.

Additional Images

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 and a left aortic arch . 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 and a left aortic arch .

Axial chest CTA in the same patient shows that the RAA and left aortic arch have joined into 1 descending thoracic aorta . The trachea remains slightly narrowed. Axial chest CTA in the same patient shows that the RAA and left aortic arch have joined into 1 descending thoracic aorta . The trachea remains slightly narrowed.

Axial chest CTA more inferiorly shows a common left descending thoracic aorta . The trachea now resumes a normal diameter. Axial chest CTA more inferiorly shows a common left descending thoracic aorta . The trachea now resumes a normal diameter.

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