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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Elevated systolic blood pressure in the upper extremities (always in right arm)

  • Normal systolic blood pressure in lower extremities (often in left arm)

  • Radial–femoral pulse delay

  • Left ventricular prominence, "3" sign, rib notching on chest radiograph

  • Coarctation visible by imaging

  • Distal aortic pressure decrease by Doppler echocardiography or catheterization

GENERAL CONSIDERATIONS

  • Male predominance

  • Most commonly located distal to the left subclavian artery

  • Associated with a bicuspid aortic valve 70% of the time

  • Usually diagnosed in childhood by routine physical examination

  • Symptoms may arise during the second and third decades of life

    • – Coarctation should be suspected in the patient in his 20s or 30s presenting with hypertension

  • Seen in more than 10% of patients with Turner’s syndrome

  • Early detection and repair are important to forestall the accelerated development of coronary artery disease and congestive heart failure

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Usually asymptomatic

  • Nonspecific symptoms:

    • – Exertional dyspnea

    • – Headache

    • – Epistaxis

    • – Leg fatigue

  • Hemorrhagic cerebrovascular accidents

  • Endarteritis

  • Possible congestive heart failure in the adult with longstanding hypertension related to coarctation or difficult-to-control hypertension

  • Aortic rupture or dissection

  • Aortic aneurysm due to poststenotic dilatation

  • Infective endocarditis of an associated bicuspid aortic valve

PHYSICAL EXAM FINDINGS

  • Elevated systolic blood pressure in the right arm

  • Reduced systolic blood pressure in the legs

  • Radial–femoral pulse delay

  • Palpable intercostal arteries (collaterals)

  • Brisk carotid upstroke

  • Hyperdynamic left ventricular impulse

  • Late systolic murmur between the scapulae to the left of the spine

  • Ejection click and systolic ejection murmur (with or without diastolic murmur of aortic insufficiency) with associated bicuspid aortic valve

DIFFERENTIAL DIAGNOSIS

  • Other causes of systemic hypertension

  • Aortoiliac disease

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • No specific studies

ELECTROCARDIOGRAPHY

  • ECG findings:

    • – Left ventricular hypertrophy

    • – Left atrial enlargement

    • – Atrial fibrillation

IMAGING STUDIES

  • Chest x-ray:

    • – Rib notching

    • – "3"sign (dilated left subclavian artery and dilated distal aorta forming the upper and lower curvatures, respectively)

    • – Left ventricular and left atrial enlargement

  • Echocardiography:

    • – Precordial 2-dimensional echocardiogram from the suprasternal notch may reveal the coarctation

    • – Color-flow Doppler acceleration in the descending aorta with persistent diastolic forward flow

  • Magnetic resonance angiography/CT:

    • – Can localize and define the extent of narrowing with a high degree of accuracy

    • – Also used for postoperative evaluation

DIAGNOSTIC PROCEDURES

  • Cardiac catheterization (rarely necessary):

    • – When noninvasive imaging cannot fully define the anatomy

    • – When concomitant coronary artery disease is suspected

TREATMENT

CARDIOLOGY REFERRAL

  • When aortic coarctation is diagnosed or suspected, patient should be referred to an adult congenital heart ...

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