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:

• 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.

• 75% of patients with coarctatin 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.

Calcification 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