Coarctation of the aorta refers to a narrowing of the aortic lumen. It can be primarily divided into two types
- infantile (pre-ductal) form
- adult (juxta-ductal, post-ductal or middle aortic) form
An infantile coarctation is characterised by diffuse hypoplasia or narrowing of the aorta from just distal to brachiocephalic artery to the level of ductus arteriosus, typically with a more discrete area of constriction just proximal to the ductus but distal to the origin of the left subclavian artery. Therefore the blood supply to the descending aorta is via the patent ductus arteriosus.
An adult coarctation in contrast is characterised by a short segment abrupt stenosis of the post-ductal aorta. It is due to thickening of the aortic media and typically occurs just distal to the ligamentum arteriosum (remnant of the ductus arteriosus).
Coarctations account for between 5-8 % of all congenital heart defects. They are more frequent in males : M:F ratio of ~ 2-3:1
As is the case with many congenital abnormalities, coarctation of the aorta is associated with other anomalies.
cardiac : coarctations are frequently associated with other congenital heart defects and conditions which include
- bicuspid aortic valve : most common associated defect and seen in 75-80 %
- ventricular septal defect (VSD)
- cyanotic congenital lesions including
- mitral valve defects including
- there can also be non cardiac associations such as
- recognised syndromic associations include
Plain film - chest radiograph
- figure of 3 sign - contour abnormality of the aorta
- inferior rib notching - Roesler sign
- secondary to dilated intercostal collateral vessels
- seen only in long standing cases, and therefore not seen in infancy
- seen in 70% of cases presenting in older children or adults
- if unilaterally seen on the left, then this suggests an associated aberrant right subclavian artery arising after the coarctation
- if unilaterally seen on the right, then the origin of the left subclavian artery is distal to the coarctation
- most often involves 4th-8th ribs
- occasionally involves 3rd and 9th ribs
- does not involve 1st and 2nd ribs (the associated arteries are branches of the costocervical trunk, and thus proximal to coarctation)
- may also show evidence of left ventricular hypertrophy
Useful in assessing for infantile coarctations. The suprasternal notch - long axis views are particularly considered helpful. The fetal right ventricle can be appear enlarged in severe coarctations although this alone is not a specific feature. Occasionally an aortic arch view may directly show a narrowing.
Angiography - CTA / MRA / DSA
All modalities are capable of delineating the coarctation as well as collateral vessels, most common collateral pathway being subclavian artery to internal mammary artery to intercostal arteries (resulting in inferior rib notching) to post-coarctation part of descending thoracic aorta.
Treatment and prognosis
Urgency of treatment depends on the presence of congestive cardiac failure. This is usually the case in severe coarctations found in infancy. In less severe cases, elective treatment when the child is older (typically ~ 2 years of age) is preferred 3.
Treatment can be either primary surgical repair with excision of the coarctation and end-to-end anastomosis, or balloon angioplasty.
- neonatal heart failure
- subarachnoid haemorrhage from ruptured berry aneurysm
- aortic dissection
- infective endocarditis - in the context of an added infective insult
- mycotic aneurysm - in the context of an added infective insult
- pseudo-coarctation of the aorta - elongation, narrowing or kinking with no pressure gradient or collateral formation : no rib notching
- chronic large vessel arteritis - e.g chronic phase of Takayasu arteritis
Congenital heart disease
There is more than one way to present the variety of congenital heart diseases. Whichever way they are categorised, it is helpful to have a working understanding of normal and fetal circulation, as well as an understanding of the segmental approach to imaging in congenital heart disease.
congenital heart disease
- normal relationship between chambers and valves
- atrioventricular valves
- outflow tract
- great vessels
- venous inflow
- anomalous valves
- abnormal relationship of chambers and valves
- atrioventricular abnormality
- great vessel connection abnormality
- congenital heart disease - chest x-ray approach
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- 3. Hay WW, Hayward AR, Levin MJ et-al. Current pediatric diagnosis & treatment. McGraw-Hill/Appleton & Lange. (2002) ISBN:0071383840. Read it at Google Books - Find it at Amazon
- 4. Sebastià C, Quiroga S, Boyé R et-al. Aortic stenosis: spectrum of diseases depicted at multisection CT. Radiographics. 2003;23 Spec No (suppl 1): S79-91. Radiographics (full text) - doi:10.1148/rg.23si035506 - Pubmed citation
- 5. Cooper R, Ritter S, Rothe W et-al. Circulation. 1987;75 (3): . doi:10.1161/01.CIR.75.3.600
- 6. Litmanovich D, Bankier AA, Cantin L et-al. CT and MRI in diseases of the aorta. AJR Am J Roentgenol. 2009;193 (4): 928-40. doi:10.2214/AJR.08.2166 - Pubmed citation
Synonyms & Alternative Spellings
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