Pseudocoarctation of the aorta

Pseudocoarctation of the aorta is a very rare anomaly characterized by kinking or buckling of the descending aorta at the level of the ligamentum arteriosum without a pressure gradient across the lesion.

Pathology

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.

Associations

Rarely reported associations include :

• congenital cardiac anomalies
• aneurysmal dilatation beyond the lesion 

Radiographic features

CT angiography

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

• 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 :

• 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.

Treatment and prognosis

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

History and etymology

It is thought to have been first described by Dotter, Steinberg, Souders and co-workers in 1951 .

Differential diagnosis

Consider the true coarctation of the aorta

• evidence of collateral circulation (e.g rib notching)

Siehe auch:

• Aortenisthmusstenose
• coarctation

und weiter:

• thoracic aortic stenosis : differential diagnosis