Aortic coarctation natural history, complications and prognosis: Difference between revisions
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==Natural History== | ==Natural History== | ||
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]]. | 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]], [[TGA|transposition of the great vessels]], and [[mitral valve disease]]. | :*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]]. | ||
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{{WH}} | {{WH}} | ||
{{WS}} | {{WS}} | ||
[[CME Category::Cardiology]] | |||
[[Category:Cardiology]] | [[Category:Cardiology]] | ||
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.