Aortic coarctation epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editor-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Overview

Coarctation 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.

Epidemiology and demographics

• Aortic coarctation is a common congenital heart defect. While statistics vary across research, aortic coarctation occurs in approximately 6-10% of all congenital heart disease cases. In live births, aortic coarctation accounts for approximately 5-7% of congenital heart disease in severely ill infants.

• The mortality rate of aortic coarctation hinges primarily on the age of surgical intervention to repair the defect. Left untreated, less than 20% of untreated patients reach the age of 50. However, with surgical repair, mortality rates are far less severe. If repaired by the age of 14, the mortality rate for 20 years out post-surgery is 9% (a 91% survival rate). If repaired later than 14, the mortality rate is 11% (a 79% survival rate).

• In 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.
• Aortic coarctation 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.

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 accompanying conditions, that increase the likelihood of a coarctation of the aorta, include:
  • Ventricular septal defect
  • Patent ductus arteriosus
  • Mitral valve stenosis
  • Aortic valve stenosis
  • Bicuspid aortic valve - associated to 30-40% of all cases