Coarctation of the aorta most frequently involves a short segment narrowing of the aorta in the proximal descending thoracic segment immediately subadjacent to the ductus arteriosis. As a result of this focal narrowing, there is systolic hypertension in the upper extremities.

Coarctation occurs twice as often in males as in females. In addition, there is a familial component to inheritance with a 2% risk of associated congenital heart disease in an infant with either an affected sibling or parent. One-half of these affected infants will have a coarctation as their congenital abnormality. In addition, of all patients with aortic coarctations, 60-70% have associated cardiac abnormalities, most commonly anomalies of the aortic or mitral valve.

The time of clinical presentation is dependent on the degree of stenosis at the level of the coarctation. The more severe coarctations present in the first year of life secondary to heart failure and failure to thrive or as a result of associated cardiac anomalies. Older children may present in adolescence with claudication symptoms in the lower extremities. Later presentations in adulthood may occur with hypertension or mediastinal abnormalities of chest radiographs.

While symptoms may be mild initially, untreated aortic coarctation carries a high morbidity and mortality rate. Persistent hypertension may lead to aortic aneurysm and rupture as well as intracranial bleed. There is also a 10% mortality rate for females during pregnancy with uncorrected coarctation with a 90% overall complication rate during pregnancy, with rates similar to normal rates in patients who have undergone correction.

A characteristic appearance of the aorta on chest radiographs is a "reverse 3" configuration of the aortic knob and proximal descending aorta. In addition, there may be dilatation proximal or immediately distal to the coarctation. The cardiac size in young adulthood is frequently at the upper limits of normal. Transesophageal echo and angiography have been used in the past for evaluation with much of this supplanted by MRI at present.

Collateral circulation using the intercostal arteries may produce bilateral rib notching along the inferior edge, usually of ribs 3 - 9.
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