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Key Features

  • Narrowing of the aortic arch distal to the origin of the left subclavian artery

  • In most patients, it is due to displaced ductal tissue in the aorta

  • Collateral circulation through intercostal arteries and branches of subclavian arteries

  • Cause of secondary hypertension

  • Bicuspid aortic valve in 50%

Clinical Findings

  • Usually no symptoms until hypertension produces left ventricular (LV) failure

  • Cerebral hemorrhage occurs rarely

  • Strong arterial pulsations in the neck and suprasternal notch

  • Hypertension in the arms, but blood pressure is normal or low in the legs

  • Delayed or weak femoral pulsations

  • Harsh systolic murmur heard in the back

  • Continuous murmurs are heard if collaterals are present around the coarctation and are a clue that the coarctation is likely severe

  • Coarctation can be a major risk in pregnant women

Diagnosis

  • ECG: left ventricular hypertrophy (LVH)

  • Chest radiograph: may show scalloping of the inferior portion of the ribs as a result of enlarged collateral intercostal arteries

  • Doppler echocardiography is diagnostic and can estimate severity of obstruction

  • MRI or CT provides excellent visualization of the local anatomy of the coarctation

  • MRA is diagnostic test of choice

  • Cardiac catheterization: measurement of gradient across stenosis

Treatment

  • Coarctation with peak gradient > 20 mm Hg should be repaired

  • Increased collateral flow may reduce gradient seen, even in severe coarctation

  • Younger than age 40

    • Surgery advisable if patient has refractory hypertension or significant LVH

    • Surgical mortality rate is 1–4%, and carries risk of spinal cord ischemia

  • Older than age 50

    • Surgical mortality rate is considerable

    • Percutaneous stenting is now the procedure of choice if anatomy suitable

  • Most untreated patients suffer complications, LV failure, or cerebral hemorrhage

  • About 25% of corrected patients remain hypertensive because of permanent changes in the renin-angiotensin system, endothelial dysfunction, aortic stiffness, altered arch morphology, and increased ventricular stiffness

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