Aortic coarctation natural history, complications and prognosis: 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: | {{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== | ||
80% of patients are diagnosed during childhood. In the remaining 20% of cases, the symptoms are often less severe, but the coarctation will ultimately require correction in order to prevent irreversible organ damage. Common complications among patients who go untreated include: [[aortic rupture]], [[infective endocarditis]], [[congestive heart failure]], and [[calcification of the aorta]]. | |||
==Natural History== | ==Natural History== | ||
In infants with a preductal coarct, the LV output goes to the upper extremities, and the RV output goes to the lower extremities through the patent ductus. | In infants with a preductal coarct, the [[Cardiac output|LV output]] goes to the upper extremities, and the RV output goes to the lower extremities through the [[patent ductus]]. | ||
* | *Childhood: | ||
:*80% of cases are diagnosed in childhood. | :*80% of cases are diagnosed in childhood. | ||
:*The preductal form is usually discovered in early infancy because it is usually severe. | :*The preductal form is usually discovered in early infancy because it is usually severe. | ||
:*Childhood coarctation is associated with a [[ventricular septal defect]] (VSD), tubular hypoplasia of the [[aortic arch]], [[transposition | :*Childhood coarctation is associated with a [[ventricular septal defect]] (VSD), tubular hypoplasia of the [[aortic arch]], [[TGA|transposition of the great vessels]], and [[mitral valve disease]]. | ||
* | *Adolescence: | ||
:*When first recognized in adolescents, coarctation of the aorta is generally asymptomatic. | :*When first recognized in adolescents, coarctation of the aorta is generally asymptomatic. | ||
* | *Adulthood: | ||
:*The postductal form is often less severe and discovered in adulthood. | :*The postductal form is often less severe and discovered in adulthood. | ||
:*Infrequently coarctation of the aorta is associated with other congenital abnormalities. | :*Infrequently coarctation of the aorta is associated with other [[congenital abnormalities]]. | ||
:*In patients over the age of 30, major complications leading to death are not uncommon. <ref>Giuliani et al, Cardiology: Fundamentals and Practice, Second Edition, Mosby Year Book, Boston, 1991, pp. 1670-1676.</ref> <ref>Deal K, Wooley CF. Coarctation of the Aorta and Pregnancy. Annals of Internal Medicine 1973, 78:706-710.</ref> | :*In patients over the age of 30, major complications leading to [[death]] are not uncommon. <ref>Giuliani et al, Cardiology: Fundamentals and Practice, Second Edition, Mosby Year Book, Boston, 1991, pp. 1670-1676.</ref><ref>Deal K, Wooley CF. Coarctation of the Aorta and Pregnancy. Annals of Internal Medicine 1973, 78:706-710.</ref> | ||
* 75% of patients with | * 75% of patients with coarctation will have hypertension at 30 years of their age. | ||
:*If the coarctation is left untreated, [[arterial hypertension]] may become permanent due to irreversible changes in some organs (such as the [[kidney]]). | :*If the coarctation is left untreated, [[arterial hypertension]] may become permanent due to irreversible changes in some organs (such as the [[kidney]]). | ||
==Complications== | ==Complications== | ||
About 50% of patients with coarctation of the aorta die within the first three decades of life, and more than 75% are dead by age 50 due to: | About 50% of patients with coarctation of the aorta die within the first three decades of life, and more than 75% are dead by age 50 due to: | ||
*[[Rupture of the aorta]] or [[aortic dissection]] | |||
*Most frequently in the third or fourth decade. | ** Most frequently in the third or fourth decade. | ||
*Dissections originate either proximally (secondary to hypertension and local stress)or distally (where the jet erodes the intima). | ** Dissections originate either proximally (secondary to [[hypertension]] and local stress)or distally (where the jet erodes the intima). | ||
*Ruptures may bleed into the esophagus, and [[hematemesis]] or [[melena]] may portend disaster. | ** Ruptures may [[bleed]] into the [[esophagus]], and [[hematemesis]] or [[melena]] may portend disaster. | ||
* [[Infective endocarditis]] or endarteritis | |||
*Most frequently in the second to fourth decade of life. | ** Most frequently in the second to fourth decade of life. | ||
* Rupture of the [[circle of Willis]] | |||
*Most frequently in the second or third decade of life. | ** Most frequently in the second or third decade of life. | ||
*Secondary to the increased incidence of aneurysms in this population and the presence of proximal hypertension. | ** Secondary to the increased incidence of [[aneurysms]] in this population and the presence of proximal [[hypertension]]. | ||
* [[Congestive heart failure]] | |||
*Common in infants, often occurs with associated abnormalities such as [[VSD]] or mitral valve disease. | ** Common in infants, often occurs with associated abnormalities such as [[VSD]] or [[mitral valve disease]]. | ||
*In the adult is secondary to [[hypertension]] associated with [[coronary artery disease]] or aortic valve disease. | ** In the adult is secondary to [[hypertension]] associated with [[coronary artery disease]] or [[aortic valve disease]]. | ||
* Calcified [[aortic stenosis]] | |||
*Result of associated [[bicuspid aortic valve]] disease that over time becomes calcified. | ** Result of associated [[bicuspid aortic valve]] disease that over time becomes calcified. | ||
* Coronary artery disease | * Premature death occurs due to | ||
* Heart failure | ** [[Coronary artery disease]] | ||
* Stroke | ** [[Heart failure]] | ||
* Aortic dissection or rupture | ** [[Stroke]] | ||
** [[Aortic dissection]] or rupture | |||
==Prognosis== | |||
Prognosis is variable. The prognosis of aortic coarctation depends on whether [[balloon angioplasty]] and [[stenting]] or the [[surgery]] has been done or not. | |||
* The mortality rate of aortic coarctation depends largely on the age of surgical repair. Left untreated, less than 20% of untreated patients live to the age of 50. However, with surgical repair, [[mortality rate]]s are far lower and survival is prolonged. | |||
* If repaired by the age of 14, the [[mortality rate]] during the 20 years following is only 9% (a 91% survival rate). | |||
* If repaired later than age 14, the [[mortality rate]] is 11% (a 79% survival rate). | |||
* Among expectant mothers with a coarctation of the aorta, the [[maternal mortality]] rate ranges between 3-8%. Even with repair, there is a risk of developing complications during [[pregnancy]]. | |||
==References== | ==References== | ||
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{{WS}} | {{WS}} | ||
[[CME Category::Cardiology]] | |||
[[Category:Cardiology]] | [[Category:Cardiology]] | ||
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[[Category:Disease]] | [[Category:Disease]] | ||
[[Category:Congenital heart disease]] | [[Category:Congenital heart disease]] | ||
Latest revision as of 22:19, 14 March 2016
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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
80% of patients are diagnosed during childhood. In the remaining 20% of cases, the symptoms are often less severe, but the coarctation will ultimately require correction in order to prevent irreversible organ damage. Common complications among patients who go untreated include: aortic rupture, infective endocarditis, congestive heart failure, and calcification of the aorta.
Natural History
In infants with a preductal coarct, the LV output goes to the upper extremities, and the RV output goes to the lower extremities through the patent ductus.
- Childhood:
- 80% of cases are diagnosed in childhood.
- The preductal form is usually discovered in early infancy because it is usually severe.
- Childhood coarctation is associated with a ventricular septal defect (VSD), tubular hypoplasia of the aortic arch, transposition of the great vessels, and mitral valve disease.
- Adolescence:
- When first recognized in adolescents, coarctation of the aorta is generally asymptomatic.
- Adulthood:
- The postductal form is often less severe and discovered in adulthood.
- Infrequently coarctation of the aorta is associated with other congenital abnormalities.
- In patients over the age of 30, major complications leading to death are not uncommon. [1][2]
- 75% of patients with coarctation will have hypertension at 30 years of their age.
- If the coarctation is left untreated, arterial hypertension may become permanent due to irreversible changes in some organs (such as the kidney).
Complications
About 50% of patients with coarctation of the aorta die within the first three decades of life, and more than 75% are dead by age 50 due to:
- Rupture of the aorta or aortic dissection
- Most frequently in the third or fourth decade.
- Dissections originate either proximally (secondary to hypertension and local stress)or distally (where the jet erodes the intima).
- Ruptures may bleed into the esophagus, and hematemesis or melena may portend disaster.
- Infective endocarditis or endarteritis
- Most frequently in the second to fourth decade of life.
- Rupture of the circle of Willis
- Most frequently in the second or third decade of life.
- Secondary to the increased incidence of aneurysms in this population and the presence of proximal hypertension.
- Congestive heart failure
- Common in infants, often occurs with associated abnormalities such as VSD or mitral valve disease.
- In the adult is secondary to hypertension associated with coronary artery disease or aortic valve disease.
- Calcified aortic stenosis
- Result of associated bicuspid aortic valve disease that over time becomes calcified.
- Premature death occurs due to
Prognosis
Prognosis is variable. The prognosis of aortic coarctation depends on whether balloon angioplasty and stenting or the surgery has been done or not.
- The mortality rate of aortic coarctation depends largely on the age of surgical repair. Left untreated, less than 20% of untreated patients live to the age of 50. However, with surgical repair, mortality rates are far lower and survival is prolonged.
- If repaired by the age of 14, the mortality rate during the 20 years following is only 9% (a 91% survival rate).
- If repaired later than age 14, the mortality rate is 11% (a 79% survival rate).
- Among expectant mothers with a coarctation of the aorta, the maternal mortality rate ranges between 3-8%. Even with repair, there is a risk of developing complications during pregnancy.