Coarctation of the Aorta

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 ...