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Virtual Pediatric Hospital: Paediapaedia: Coarctation of the Aorta Paediapaedia: Cardiovascular Diseases

Coarctation of the Aorta, Adult Type

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Adult type is usually not diagnosed until late childhood or early adulthood when hypertension and a difference in arm and leg pulses is detected on routine physical.

Etiology/Pathophysiology:
Usually caused by a congenital indentation in the posterior wall of the aorta that usually is distal to the origin of the left subclavian artery at the level of the ductus arteriosus. Infantile type coarctation causes a diffuse narrowing while adult type coarctation causes a focal narrowing.

The collatateral circulation is via the internal mammary arteries, the costocervical trunk, and the intercostal arteries.

Seventy-five percent of patients have a bicuspid aortic valve, and ASD / VSD / PDA / mitral valve deformities can also be seen.

Pathology:
Not applicable

Imaging Findings:
The adult type often has intercostal rib notching due to collateral flow through dilated pulsatile intercostal arteries and it is seen in 75% of older children with coarctation. It is unusual to see rib notching before 6 years of age. It is seen in the 3rd - 9th ribs because the 1st and 2nd ribs intercostal arteries come from the thyocervical trunk which is above the coarctation. Irregular wavy densities seen on the lateral film reflect dilation of the internal mammary arteries.

Another sign in the adult type is the "3" sign which is created by a prominent left subclavian artery, the coarctation and post stenotic dilation of the descending aorta. The barium swallow shows a reversed "3" which represents the aortic impression on the esophagus above and below the coarctation.

DDX:

References:
See References Chapter.

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