Fact-sheet: Double aortic arch
Updated on 04/22/2020 at 10:10 AM
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Definition
Embryonic failure of aortic arch involution: persistence of both arches.
Clinical features
Clinical symptoms are usually early, noted from the neonatal period or shortly thereafter, dominated by respiratory and digestive signs related to extrinsic compression of the aerodigestive tract.
Radiography
Thoracic distension, absence of an aortic knob with a cardiothoracic index of 0.5
Esophagography shows an abnormal indentation on the esophagus
Ultrasound
The descending aorta is on the left
The right arch is larger than the left
CT
The descending aorta is on the left
The right arch is larger than the left
Aggravating component to look for:
- arterial ligament
- Kommerell diverticulum (at the origin of the descending aorta)
MRI
Same semiology as on CT
Management
Division and suture of the non-dominant aortic arch (most often the left), with division of the arterial ligament and release of the esophagotracheal complex.