Aortic coarctation

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Aortic coarctation
Classification and external resources
ICD-10 Q25.1
ICD-9 747.10
OMIM 120000
DiseasesDB 2876
eMedicine med/154 
MeSH D001017
Sketch showing heart with coarctation of the aorta. A: Coarctation (narrowing) of the aorta. 1:inferior caval vein, 2:right pulmonary veins, 3: right pulmonary artery, 4:superior caval vein, 5:left pulmonary artery, 6:left pulmonary veins, 7:right ventricle, 8:left ventricle, 9:pulmonary artery, 10:Aorta
Schematic drawing of alternative locations of a coarctation of the aorta, relative to the ductus arteriosus. A: ductal coarctation, B: preductal coarctation, C: postductal coarctation. 1: Aorta ascendens, 2: Arteria pulmonalis, 3: Ductus arteriosus, 4: Aorta descendens, 5: Trunchus brachiocephalicus, 6: Arteria carotis communis sinister, 7: Arteria subclavia sinister

Coarctation of the aorta, or Aortic coarctation, is the name given to a congenital condition whereby the aorta narrows in the area where the ductus arteriosus (ligamentum arteriosum after regression) inserts.

Contents

Types

There are three types:[1]

  1. Preductal coarctation: The narrowing is proximal to the ductus arteriosus. If severe, blood flow to the aorta distal to the narrowing (supplying lower body) is dependent on a patent ductus arteriosus, and hence its closure can be life-threatening. Preductal coarctation results when an intracardiac anomaly during fetal life decreases blood flow through the left side of the heart, leading to hypoplastic development of the aorta. This is the type often seen in infants with Turner Syndrome.[2]
  2. Ductal coarctation: The narrowing occurs at the insertion of the ductus arteriosus. This kind usually appears when the ductus arteriosus closes.
  3. Postductal coarctation: The narrowing is distal to the insertion of the ductus arteriosus. Even with an open ductus arteriosus blood flow to the lower body can be impaired. Newborns with this type of coarctation may be critically sick from birth. This type is most common in adults. It is associated with notching of the ribs, hypertension in the upper extremities, and weak pulses in the lower extremities. Postductal coarctation is most likely the result of muscular ductal (ductus arteriosis) extends into the aorta during fetal life.

Signs and symptoms

Arterial hypertension in the right arm with normal to low blood pressure in the lower extremities is classic. Poor peripheral pulses in the femoral arteries may be found in severe cases.

If the coarctation is situated before the left subclavian artery, asynchronous radial pulses will be detected in the right and left arms. A radial-femoral delay between the right arm and the femoral artery would be apparent, whilst no such delay would occur under left arm radial-femoral palpation.

A coarctation occurring after the left subclavian artery will produce synchronous radial pulses, but radial-femoral delay will be present under palpation in either arm.

Imaging and diagnosis

With imaging, resorption of the lower part of the ribs may be seen, due to increased blood flow over the neurovascular bundle that runs there. Post-stenotic dilation of the aorta results in a classic 'reverse 3 sign' on x-ray. The characteristic bulging of the sign is caused by dilatation of the aorta due to an indrawing of the aortic wall at the site of cervical rib obstruction, with consequent post-stenotic dilation. This physiology results in the reversed '3' image for which the sign is named.[3][4][5]When the esophagus is filled with barium, a reverse 3 or E sign is often seen and represents a mirror image of the areas of prestenotic and poststenotic dilatation. [6]


Coarctation of the aorta can be accurately diagnosed with magnetic resonance angiography. In teenagers and adults echocardiograms may not be conclusive. In adults with untreated coarctation blood often reaches the lower body through collaterals, eg. internal thoracic arteries via. the subclavian arteries. Those can be seen on MR or angiography. An untreated coarctation may also result in hypertrophy of the left ventricle.

A case of coarctation of the aorta was published in the New England Journal of Medicine in 2007 showing chest X-Rays and MRT Images.[7]

Therapy

Therapy is conservative if asymptomatic, but may require surgical resection of the narrow segment if there is arterial hypertension. In some cases angioplasty can be performed to dilate the narrowed artery. If the coarctation is left untreated, arterial hypertension may become permanent due to irreversible changes in some organs (such as the kidney).

References

  1. ^ Valdes-Cruz LM, Cayre RO: Echocardiographic diagnosis of congenital heart disease. Philadelphia, 1998.
  2. ^ Cotran, R., V. Kumar, and N. Fausto (2005). Robbins Pathologic Basis of Disease, 7th Ed.. W.B. Saunders. ISBN 0-8089-2302-1. 
  3. ^ Sabatine, Marc (February 15, 2000). Pocket Medicine. Lippincott Williams & Wilkins, 256 pages. ISBN 0781716497. 
  4. ^ Blecha, Matthew J. (August 30, 2005). "General Surgery ABSITE and Board Review (Pearls of Wisdom)". McGraw-Hill.
  5. ^ Brady Pregerson (October 1, 2006). "Quick Essentials: Emergency Medicine, 2nd Edition". ED Insight Books.
  6. ^ Singh, Vibhuti. Aorta, Coarctation. E-medicine. 
  7. ^ Quiros-Lopez R, Garcia-Alegria J (2007). "A medical mystery -- high blood pressure". N Engl J Med 356 (25): 2630. doi:10.1056/NEJMicm061308. PMID 17582073. 

External links

Congenital vascular defects (Q25-Q28, 747)
Great arteries
Great veins
Other

Retrieved from "http://en.wikipedia.org/wiki/Coarctation_of_the_aorta"

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