Aortic coarctation natural history, prognosis and complications


 * Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S.[mailto:psingh@perfuse.org], ; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S.[mailto:kfeeney@perfuse.org]

Overview
An aortic coarctation is primarily symptomatic enough during childhood to be examined, approximately 80% of cases are diagnosed at this time. The remaining 20% of cases are often less symptomatic, less severe but will ultimately require correction or irreversible organ damage can occur. Common complications, when left untreated, include: 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.
 * 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.