Double aortic arch
Double aortic arch is the most common symptomatic type of the aortic arch variant. It may account for up to 50-60% of vascular rings.
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Clinical presentation
Double aortic arch is mostly diagnosed in childhood due to symptoms related to esophageal and/or tracheal obstruction. Respiratory symptoms can be more common in infancy or early childhood while adult patients complain of difficulties in swallowing rather than respiratory difficulties due to tracheal development.
Pathology
This anomaly is caused by persistence of the right and left embryonic fourth aortic arches, which results in formation of a vascular ring from the splitting of the ascending aorta into two limbs that pass to either side of the trachea and esophagus 5 (both of which get encircled), which then join as a single descending aorta.
When the minor arch is markedly hypoplastic or atretic (incomplete double aortic arch), the affected segment tends to be almost always distal to the left subclavian artery 9.
Subtypes
- right dominant arch: ~75-80%
- co-dominant arch: ~5%
- left dominant arch: ~25%
Associations
It is rarely associated with congenital heart disease 12,13.
Radiographic features
Plain radiograph
Right-sided aortic arch indenting the trachea and increase right paratracheal soft tissue thickness. Lack of air column in thoracic portion of the trachea sometimes seen.
Fluoroscopy: barium swallow
Contrast swallow studies are more helpful and demonstrate classic "reverse S" indentation of the contrast column on frontal view as well as posterior indentation on lateral view.
CT and MRI
Contrast-enhanced cross-sectional imaging is required to confirm the diagnosis and also to delineate details of anatomy in particular right or left arch dominance for surgical planning.
Treatment and prognosis
Surgical intervention with tracheostomy from the side of descending aorta and division of the minor (smaller) arch and ligamentum arteriosum is indicated for symptomatic patients. In appropriate cases, surgical planning is directed toward transection of either the smallest arch (if both arches are patent) or an atretic arch segment.
Related Radiopaedia articles
Anatomy: Thoracic
- thoracic skeleton
- thoracic cage
- thoracic spine
- articulations
- muscles of the thorax
- diaphragm
- intercostal space
- intercostal muscles
- variant anatomy
- spaces of the thorax
- thoracic viscera
- tracheobronchial tree
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lungs
-
bronchopulmonary segmental anatomy (Boyden Classification) (mnemonic)
- left lung
- right lung
- variant anatomy
- lung parenchyma
- hilum
- pleura
-
bronchopulmonary segmental anatomy (Boyden Classification) (mnemonic)
-
heart
- cardiac chambers
- heart valves
- pectinate muscles
- cardiac fibrous skeleton
- coronary arteries
- innervation of the heart
- fetal circulation
-
pericardium
- pericardial space
- oblique pericardial sinus
- transverse pericardial sinus
-
pericardial recesses
- aortic recesses
- pulmonic recesses
- postcaval recess
- pulmonary venous recesses
- pericardial ligaments
- epicardial fat pad
- esophagus
- thymus
- breast
- blood supply of the thorax
- arteries
-
thoracic aorta (development)
- ascending aorta
- aortic arch
- aortic isthmus
- descending aorta
- pulmonary trunk
-
thoracic aorta (development)
- veins
- superior vena cava (cavoatrial junction)
- inferior vena cava
-
coronary veins
- cardiac veins which drain into the coronary sinus
- great cardiac vein
- middle cardiac vein
- small cardiac vein
- posterior vein of the left ventricle
- vein of Marshall (oblique vein of the left atrium)
- anterior cardiac veins
- venae cordis minimae (smallest cardiac veins or thebesian veins)
- cardiac veins which drain into the coronary sinus
- pulmonary veins
- arteries
- lymphatics
- innervation of the thorax
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