Pseudocoarctation of the aorta is a very rare anomaly characterised by kinking or buckling of the descending aorta at the level of the ligamentum arteriosum without a pressure gradient across the lesion.
It is thought to be of congenital origin, and characterised by elongation and kinking of the aorta at the level of the ligamentum arteriosum.
Its exact aetiology 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.
Rarely reported associations include 3:
- congenital cardiac anomalies
- aneurysmal dilatation beyond the lesion
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
- visualisation 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.
Treatment and prognosis
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.
History and etymology
It is thought to have been first described by Dotter, Steinberg, Souders and co-workers in 1951 3,5.
Consider the true coarctation of the aorta
- evidence of collateral circulation (e.g rib notching)
- 1. Smyth PT, Edwards JE. Pseudocoarctation, kinking or buckling of the aorta. Circulation. 1972;46 (5): 1027-32. doi:10.1161/01.CIR.46.5.1027 - Pubmed citation
- 2. Adaletli I, Kurugoglu S, Davutoglu V et-al. Pseudocoarctation. Can J Cardiol. 2007;23 (8): 675-6. Free text at pubmed - Pubmed citation
- 3. Son JS, Hong KB, Chung DC. Pseudocoarctation of the aorta associated with the anomalous origin of the left vertebral artery: a case report. Korean J Radiol. 2008;9 (3): 283-5. doi:10.3348/kjr.2008.9.3.283 - Free text at pubmed - Pubmed citation
- 4. Joseph M, Leclerc Y, Hutchison SJ. Aortic pseudocoarctation causing refractory hypertension. N. Engl. J. Med. 2002;346 (10): 784-5. doi:10.1056/NEJM200203073461019 - Pubmed citation
- 5. SOUDERS CR, PEARSON CM, ADAMS HD. An aortic deformity simulating mediastinal tumor: a subclinical form of coarctation. Dis Chest. 2004;20 (1): 35-45. Pubmed citation
- 6. Gaupp RJ, Fagan CJ, Davis M et-al. Pseudocoarctation of the aorta. J Comput Assist Tomogr. 1981;5 (4): 571-3. Pubmed citation
- 7. Kessler RM, Miller KB, Pett S et-al. Pseudocoarctation of the aorta presenting as a mediastinal mass with dysphagia. Ann. Thorac. Surg. 1993;55 (4): 1003-5. Pubmed citation
- 8. Kimura K, Ohtake H, Kato H et-al. Pseudocoarctation of the aorta complicated by thoracic aortic aneurysm. Asian Cardiovasc Thorac Ann. 2011;19 (3-4): 265-7. doi:10.1177/0218492311407782 - Pubmed citation
- acute aortic syndrome
- thoracic aortic aneurysm
- abdominal aortic aneurysm
- endovascular aneurysm repair (EVAR)
- reporting tips for aortic aneurysms
- aortic coarctation
- aortic pseudocoarctation
- cervical aortic arch
- interrupted aortic arch
- transposition of the great arteries
- variant anatomy of the aortic arch
- traumatic aortic injury