Aortic coarctation natural history, complications and prognosis

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.

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).

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
  • Coronary artery disease
  • Heart failure
  • Stroke
  • Aortic dissection or rupture

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.

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