CTICasesLeft Cervical Aortic Arch with Pseudocoarctation and Hypoplastic Right Upper Lobe
Imaging findings
CT from over a decade earlier shows a very high-riding aortic arch lying in the neck, a left cervical aortic arch, with anomalous branching. The left common carotid is small, the left vertebral arises separately, and an aberrant right subclavian artery crosses the mediastinum to supply the right subclavian and axillary vessels without giving rise to other branches; there is no double arch and no right arch. The arch shows a hooked or figure-three configuration typical of pseudocoarctation, best appreciated on MIP. The right upper lobe is small with a diminutive right upper lobe vessel, and in place of a normal right upper lobe bronchus there is a single small branch, with a blind-ending tracheal bronchus above that has cartilage and is a true airway rather than a diverticulum. A middle lobe bronchus is present and a hypoplastic third lobe is likely; the right pulmonary artery does not appear hyperarterial. There is no situs abnormality such as polysplenia in the upper abdomen. The patient had a prosthetic aortic valve and a functionally bicuspid valve with aortic stenosis.
Key takeaways
Cervical aortic arch characteristically has anomalous branching, and roughly half of patients with pseudocoarctation have an associated aortic valve abnormality, here a bicuspid valve with stenosis. A left cervical arch is distinctly unusual; a right cervical arch is more often associated with DiGeorge syndrome, as is an interrupted arch in which the descending aorta is supplied through collateral flow. The blind-ending tracheal bronchus is the likely cause rather than the consequence of the hypoplastic right upper lobe, the bud having stalled before reaching the periphery. When something looks unusual on a radiograph it is worth reviewing old studies; this anomaly had gone unremarked for fifteen years.
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