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Essentials of Diagnosis
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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
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General Considerations
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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 transcoarctation 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
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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
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
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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 poststenotic aortic dilation along with LV enlargement may be present
The coarctation region and the poststenotic dilation of the descending aorta may result in a "3" sign along the aortic shadow on the PA chest radiograph (the notch in the "3" representing the area ...