Aortic coarctation epidemiology and demographics
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Cafer Zorkun, M.D., Ph.D. [2], Priyamvada Singh, M.B.B.S.[3]
Overview
Coarctation of the aorta is a common congenital malformation. It occurs in about 7% of patients with congenital heart defects. It is more common in males than females with a ratio of 2:1. Up to 25% of patients with Turner syndrome have coarctation of the aorta. It is 7 times more common among caucasians than asians.
Epidemiology and demographics
United States of America
- Aortic coarctation is a common heart defect.
- It forms approximately 6-10% of all congenital heart disease cases.
- In live births, it accounts for approximately 5-7% of congenital heart disease in severely ill infants.
International
- The prevalence is lower in Asian countries compared to American and European countries.
Mortality
- 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.
Race
- Demographically, aortic coarctation has a certain link to specific races.
- It is more common in Caucasians, with approximately 7 times more cases in Caucasians versus Asian.
- The incidence has also been noted, in some populations, to be lower in Native American races than Caucasians.
Gender
- It is more common in males than females with a ratio of 2:1 and a prevalence of 2-5 times occurring more frequently in males than females.
Age
- The diagnosis is often missed in first year of life.
- Generally, patients with coarctation of the aorta present early in life with congestive heart failure or later in life with hypertension.
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.
Other associated conditions
Cardiac
- Bicuspid aortic valve (associated to 30-40% of all cases)
- Ventricular septal defect
- Patent ductus arteriosus
- Mitral stenosis
- Aortic valve stenosis
- Aortic hypoplasia
Non-cardiac
- Intracerebral aneurysm