Aortic coarctation pathophysiology: Difference between revisions

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Latest revision as of 16:03, 11 July 2017

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 descending 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 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

Aorta proximal to the coarct
Aorta distal to the coarctation
Left subclavian artery
The narrowing progresses throughout life, and extensive collaterals develop from the subclavian (predominantly) and axillary arteries through:

Genetics

Aortic coarctation, like many congenital heart diseases, is more common in patients with other genetic conditions.
As many as 10-25% of patients with Turner syndrome have an accompanying coarctation of the aorta.

Gross Pathology

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

AORTA: Coarctation, Adult: Gross, fixed tissue, an excellent illustration of postductal coarctation
AORTA: Coarctation: Gross, hypoplastic aortic arch and infantile coarctation well demonstrated.
Localized Coarctation of the aorta.

Associated Conditions

It is commonly associated with bicuspid aortic valve.
There is 5 fold increase in the intracranial aneurysm in patient with coarctation.

Videos

References

Template:WH Template:WS CME Category::Cardiology

@@ Line 1: / Line 1: @@
+<div style="-webkit-user-select: none;">
+{|class="infobox" style="position: fixed; top: 65%; right: 10px; margin: 0 0 0 0; border: 0; float: right;
+|-
+| {{#ev:youtube|https://https://www.youtube.com/watch?v=0OqTJwZkRL4|350}}
+|-
+|}__NOTOC__
+{{Aortic coarctation}}
+{{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]
-{{Template:Aortic Coarctation}}
+==Overview==
-{{CMG}}
+An aortic coarctation results from both, [[congenital]] and [[acquired]] means. Factors directly influencing the pathophysiology include defect location and sites of secondary [[dilation]].
-'''Associate Editor-in-Chief:''' {{CZ}}
+==Pathophysiology==
+<div align="left">
+<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:
+*[[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]].
+<br clear="left"/>
+===Defect Location===
+[[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===
+*[[Aorta]] proximal to the coarct
+*[[Aorta]] distal to the coarctation
+*[[Left subclavian artery]]
+*The narrowing progresses throughout life, and extensive [[collaterals]] develop from the subclavian (predominantly) and [[axillary arteries]] through:
+:#[[Internal mammary artery]]
+:#[[Scapular artery]]
+:#[[Intercostal arteries]]
+:#Epigastric arteries
+:#[[Anterior spinal arteries]]
+===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===
+<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>
-==Pathophysiology==
+<div align="left">
-Coarctation of the aorta can be either congenital or acquired.
+<gallery heights="225" widths="225">
+Image:Aortic coarctation adult type.jpg|AORTA: Coarctation, Adult: Gross, fixed tissue, an excellent illustration of postductal coarctation
+Image:Hypoplastic aortic arch with infantile type coarctation.jpg|AORTA: Coarctation: Gross, hypoplastic aortic arch and infantile coarctation well demonstrated.
+Image:COA 3.jpg|Localized Coarctation of the aorta.
+</gallery>
+</div>
-'''Congenital coarctation''' results 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.
+===Associated Conditions===
+* It is commonly associated with [[bicuspid aortic valve]].
+* There is 5 fold increase in the intracranial [[aneurysm]] in patient with coarctation.
-'''Acquired coartation''' can occur in systemic arteritides such as [[Takayasu arteritis]]. Additionally it may occur in rare cases of severe [[atherosclerosis]].
+==Videos==
+{{#ev:youtube|SiNJfvK_qeI}}
 ==References==
 {{reflist|2}}
+{{WH}}
+{{WS}}
+[[CME Category::Cardiology]]
 [[Category:Cardiology]]
 [[Category:Pediatrics]]
-[[Category:DiseaseState]]
+[[Category:Disease]]
+[[Category:Congenital heart disease]]
-{{WH}}
-{{WS}}