Aortic coarctation natural history, complications and prognosis: Difference between revisions

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==Overview==
==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.
In 80% of patients there are usually sufficient symptoms for coarctation to be 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==
==Natural History==

Revision as of 02:09, 18 October 2012

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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

In 80% of patients there are usually sufficient symptoms for coarctation to be 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. [1] [2]
  • 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

Premature death occurs due to

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

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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