Aortic coarctation pathophysiology: Difference between revisions

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{{Aortic coarctation}}
{{Aortic coarctation}}
{{CMG}}; '''Associate Editor(s)-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, M.B.B.S.]][mailto:psingh@perfuse.org], {{CZ}}; '''Assistant Editor(s)-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]][mailto:kfeeney@perfuse.org]
{{CMG}}; '''Associate Editor(s)-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, M.B.B.S.]][mailto:psingh13579@gmail.com], {{CZ}}; '''Assistant Editor(s)-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]][mailto:kfeeney@elon.edu]


==Overview==
==Overview==
An aortic coarctation results from both, congenital and acquired means. Factors directly influencing the pathophysiology include defect location and sites of secondary dilation.
An aortic coarctation results from both, [[congenital]] and [[acquired]] means. Factors directly influencing the pathophysiology include defect location and sites of secondary [[dilation]].


==Pathophysiology==
==Pathophysiology==
[[Image:COA_PAth.jpg‎|center|450px]]
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<gallery heights="225" widths="225">
Image:COA_PAth.jpg‎|Coarctation of the descending aorta.
Image:Coarctation and PDA.png|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
</gallery>
</div>
Coarctation of the aorta can be:
Coarctation of the aorta can be:
*'''Congenital coarctation''' resulting from an infolding of the aortic media that incorportaes ductal tissue, forming a ridge that eccentrically narrows the lumen of the vessel. Subsequent intimal proliferation on the ridge leads to progressive narrowing of the vessel lumen. There is a dilatation before and after the narrowing, giving the aorta an hourglass appearance. The exact etiology of the aortic abnormality remains unclear but likely involves a defect in the vascular wall of the aorta due to reduced antegrade intrauterine blood flow or to constriction of ductal tissue extending into the thoracic aorta.  
*[[Congenital]] coarctation resulting from an infolding of the aortic media that incorportaes ductal tissue, forming a ridge that eccentrically narrows the lumen of the vessel. Subsequent intimal proliferation on the ridge leads to progressive narrowing of the vessel lumen. There is a [[dilatation]] before and after the narrowing, giving the [[aorta]] an hourglass appearance. The exact etiology of the aortic abnormality remains unclear but likely involves a defect in the vascular wall of the [[aorta]] due to reduced antegrade intrauterine [[blood flow]] or to constriction of ductal tissue extending into the [[thoracic aorta]].  
*'''Acquired coarctation''' occurring in systemic arteritides such as [[Takayasu arteritis]]. Additionally it may occur in rare cases of severe [[atherosclerosis]].
*[[Acquired]] coarctation occurring in systemic arteritides such as [[Takayasu arteritis]]. Additionally it may occur in rare cases of severe [[atherosclerosis]].
<br clear="left"/>


===Defect location===
===Defect Location===
#95% of the lesions are located distal to the left [[subclavian artery]] and proximal to the [[ductus arteriosus]] (preductal coarctation) or just at or distal to the ductus (postductal coarctation).
#5% of coarctations are located proximal to the left [[subclavian artery]], or rarely in the abdominal [[aorta]].
#In some cases, coarctation presents as a long segment or a tubular hypoplasia.
The stenosis is caused by an infolding of the left posterolateral aspect of the aortic wall resulting in an eccentric narrowing.
[[Image:COA.jpg|center|500px]]
[[Image:COA.jpg|center|500px]]
<br clear="left"/>
*95% of the lesions are located distal to the left [[subclavian artery]] and proximal to the [[ductus arteriosus]] (preductal coarctation) or just at or distal to the ductus (postductal coarctation).
*5% of coarctations are located proximal to the left [[subclavian artery]], or rarely in the [[abdominal aorta]].
*In some cases, coarctation presents as a long segment or a tubular [[hypoplasia]].
*The [[stenosis]] is caused by an infolding of the left posterolateral aspect of the aortic wall resulting in an eccentric narrowing.


===Sites of secondary dilation===
===Sites of Secondary Dilation===
#Aorta proximal to the coarct
*[[Aorta]] proximal to the coarct
#Aorta distal to the coarctation
*[[Aorta]] distal to the coarctation
#Left subclavian artery
*[[Left subclavian artery]]
#The narrowing progresses throughout life, and extensive collaterals develop from the subclavian (predominantly) and [[axillary arteries]] through:
*The narrowing progresses throughout life, and extensive [[collaterals]] develop from the subclavian (predominantly) and [[axillary arteries]] through:
##[[Internal mammary artery]]
:#[[Internal mammary artery]]
##[[Scapular artery]]
:#[[Scapular artery]]
##[[Intercostal arteries]]
:#[[Intercostal arteries]]
##Epigastric arteries
:#Epigastric arteries
##[[Anterior spinal arteries]]
:#[[Anterior spinal arteries]]


==Pathology==
===Genetics===
* Aortic coarctation, like many [[congenital heart disease]]s, is more common in patients with other [[genetic condition]]s.
* As many as 10-25% of patients with [[Turner syndrome]] have an accompanying coarctation of the aorta.
===Gross Pathology===
===Gross Pathology===
<small> [http://www.peir.net Images courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small>
<small> [http://www.peir.net Images courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small>
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===Associated Conditions===
* It is commonly associated with [[bicuspid aortic valve]].
* There is 5 fold increase in the intracranial [[aneurysm]] in patient with coarctation.
==Videos==
{{#ev:youtube|SiNJfvK_qeI}}


==References==
==References==
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{{WH}}
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[[CME Category::Cardiology]]


[[Category:Cardiology]]
[[Category:Cardiology]]
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[[Category:Disease]]
[[Category:Disease]]
[[Category:Congenital heart disease]]
[[Category:Congenital heart disease]]
[[Category:Overview complete]]
[[Category:Mature chapter]]

Latest revision as of 16:03, 11 July 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S.[2], Cafer Zorkun, M.D., Ph.D. [3]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S.[4]

Overview

An aortic coarctation results from both, congenital and acquired means. Factors directly influencing the pathophysiology include defect location and sites of secondary dilation.

Pathophysiology

Coarctation of the aorta can be:

  • Congenital coarctation resulting from an infolding of the aortic media that incorportaes ductal tissue, forming a ridge that eccentrically narrows the lumen of the vessel. Subsequent intimal proliferation on the ridge leads to progressive narrowing of the vessel lumen. There is a dilatation before and after the narrowing, giving the aorta an hourglass appearance. The exact etiology of the aortic abnormality remains unclear but likely involves a defect in the vascular wall of the aorta due to reduced antegrade intrauterine blood flow or to constriction of ductal tissue extending into the thoracic aorta.
  • Acquired coarctation occurring in systemic arteritides such as Takayasu arteritis. Additionally it may occur in rare cases of severe atherosclerosis.


Defect Location


  • 95% of the lesions are located distal to the left subclavian artery and proximal to the ductus arteriosus (preductal coarctation) or just at or distal to the ductus (postductal coarctation).
  • 5% of coarctations are located proximal to the left subclavian artery, or rarely in the abdominal aorta.
  • In some cases, coarctation presents as a long segment or a tubular hypoplasia.
  • The stenosis is caused by an infolding of the left posterolateral aspect of the aortic wall resulting in an eccentric narrowing.

Sites of Secondary Dilation

  1. Internal mammary artery
  2. Scapular artery
  3. Intercostal arteries
  4. Epigastric arteries
  5. Anterior spinal arteries

Genetics

Gross Pathology

Images courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

Associated Conditions

Videos

{{#ev:youtube|SiNJfvK_qeI}}

References

Template:WH Template:WS CME Category::Cardiology