Pseudocoarctation or congenital kinking of the aorta: radiologic considerations

Surgical Repair of a Pseudocoarctation with Cervical Aortic Arch Complicated by Multiple Aneurysms of the Aorta: A Case Report

“…For our patient the decision to proceed to the intervention was made on the combination of cervical aortic arch, pseudocoarctation and the presence of saccular aneurysms, even if the patient was asymptomatic. Unlikely many cases in the literature [8] we made median sternotomy in order to allow us to enlarge the aortic arch and to remove the aneurysmal portion. Our principal teaching point once the diagnosis of pseudocoarctation of the aorta is made is these patients must remain under a program of annual surveillance to identify the possible formation of aneurysm, in order to prevent lethal complication as rupture.…”

Pseudocoarctation and coarctation

“…The etiology is presumably due to failure of compression of the third through the tenth segments of the aortic roots and the fourth arch segment during the period of embryologic evolution [1] which result in an abnormally long aortic arch, often extending into the superior mediastinum and even above the clavicle. Many congenital cardiac diseases have been reported to be associated with pseudocoarctation such as bicuspid aortic valve, aortic stenosis, ventricular septal defect, patent ductus arteriiosus and trasposition of the great vessels [2,3].…”

Answer to What’s Your Diagnosis?

“…1 It is a congenital acute kink or anterior buckling of the thoracic aorta at the level of the ligamentum arteriosum. [1][2][3] The descending portion of the aortic arch is angulated sharply anteriorly and slightly downward. Proximal to the buckling, the aortic arch rises unusually high in the mediastinum.…”

“…The narrowing is insufficient, however, to provide a significant pressure gradient, which differs from true coarctation characterized by aortic pressure gradients, dilation of the ascending aorta and the presence of collateral circulation. [3][4][5] Embryogenesis of the pseudocoarctation of the aorta remains unclear, with many hypotheses relating to variability of the origins of aortic branches. The most accepted hypothesis is that there is a lack of compression and fusion of certain segments of the dorsal aortic root (third to tenth) and fourth arch, resulting in an abnormally long aortic arch, which twists at the point of insertion of the ligamentum arteriosum.…”

“…Associations between Turner syndrome, megacalycosis and the pseudocoarctation have been reported. 1,[3][4][5] The chest radiographic findings are nonspecific and can simulate those of mediastinal soft-tissue mass. The radiographic appearance is of smoothly marginated soft-tissue mass in the left paratracheal superior to the expected position of the aortic knob.…”