Pseudocoarctation of the aorta

Last revised by Rohit Sharma on 23 May 2024

Pseudocoarctation of the aorta is a rare anomaly characterized by kinking or buckling of the descending aorta at the level of the ligamentum arteriosum with no significant pressure gradient across the lesion (<10 mmHg).

Rarely reported associations include 3:

It is thought to be of congenital origin, and characterized by elongation and kinking of the aorta at the level of the ligamentum arteriosum.

Its exact etiology not well known. One proposed embryologic cause is a failure of compression of the third through the seventh segments of the dorsal aortic roots and the fourth arch segment.

The following features have been shown to be useful in diagnosis (according to case reports) 3:

  • demonstration that the abnormal mass in the mediastinum is part of the aorta

  • unusually aortic arch high in the mediastinum

  • visualization of the isthmic portion of the descending thoracic aorta that's not adjacent to the spine, but rather ventral to it; this is surrounded by aerated lung

  • a more caudal origin of the subclavian artery

Other reported features include 2:

  • the aortic arch of pseudocoarctation is elongated and may arise higher than the clavicle (associated with cervical aortic arch)

  • absence or only a mild degree of stenosis of the aortic lumen

  • the absence of collateral circulation

  • the absence of left ventricular hypertrophy and ascending aortic dilation

Cardiac catheterization and angiography provide a definitive diagnosis.

It is considered a benign entity with no specific intervention 8. Some advocate surgical treatment for all symptomatic patients and those with associated aneurysm formation 7.

It is thought to have been first described by Dotter, Steinberg, Souders et al. in 1951 3,5.

Consider true coarctation of the aorta:

ADVERTISEMENT: Supporters see fewer/no ads