Aortic coarctation natural history, complications and prognosis

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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:
  • 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 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.

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.

References

  1. Giuliani et al, Cardiology: Fundamentals and Practice, Second Edition, Mosby Year Book, Boston, 1991, pp. 1670-1676.
  2. Deal K, Wooley CF. Coarctation of the Aorta and Pregnancy. Annals of Internal Medicine 1973, 78:706-710.

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