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For further information, see CMDT Part 10-05: Coarctation of the Aorta

Key Features

Essentials of Diagnosis

  • Usual presentation is systemic hypertension

  • Echocardiography/Doppler is diagnostic; a peak gradient of > 20 mm Hg may be significant due to collaterals around the coarctation reducing gradient despite severe obstruction

  • Associated bicuspid aortic valve in 50–80% of patients

  • Delayed pulse in femoral artery compared to brachial artery

  • Systolic pressure is higher in upper extremities than in lower extremities; diastolic pressures are similar

General Considerations

  • Coarctation of the aorta consists of localized narrowing of the aortic arch just distal to the origin of the left subclavian artery

  • If the stenosis is severe,

    • Collateral circulation develops around the coarctation site through the intercostal arteries and the branches of the subclavian arteries

    • A lower trans-coarctation gradient can result by enabling blood flow to bypass the obstruction

  • Coarctation is a cause of secondary hypertension and should be considered in young patients with elevated blood pressure

  • The renin-angiotensin system is often abnormal and contributes to the residual hypertension occasionally seen even after coarctation repair

  • A bicuspid valve is seen in approximately 50–80% of the cases, and there is an increased incidence of cerebral berry aneurysms

  • Significant native or recurrent aortic coarctation has been defined as follows

    • Upper extremity/lower extremity resting peak-to-peak gradient > 20 mm Hg or mean Doppler systolic gradient > 20 mm Hg

    • Upper extremity/lower extremity gradient > 10 mm Hg or mean Doppler gradient > 10 mm Hg when there is either decreased LV systolic function or aortic regurgitation

    • Upper extremity/lower extremity gradient > 10 mm Hg or mean Doppler gradient > 10 mm Hg when there is evidence for collateral flow around the coarctation

  • The 2020 European Society of Cardiology guidelines have expanded the severity criteria and suggest stenting is appropriate if the patient is normotensive but has a peak gradient of > 20 mm Hg or if the stenosis by angiography is > 50%

Clinical Findings

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

  • Cerebral hemorrhage occurs rarely

  • Increased frequency of intracranial aneurysm

  • 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

  • A continuous murmur heard superiorly and midline in the back or over the left anterior chest may be present when large collaterals occur and is a clue that the coarctation is severe

  • Aortic regurgitation or stenosis murmur due to an associated bicuspid aortic valve may be present

  • Coarctation is associated with Turner syndrome (a sex chromosomal abnormality [XO]); a webbed neck may be present in these patients

Diagnosis

Imaging

  • Radiography

    • May show scalloping of the inferior portion of the ribs (rib notching) due to enlarged collateral intercostal arteries

    • Dilation of the left subclavian artery and ...

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