Clinical Features/Diagnostics/Treatment

Asymptomatic as long as PDA is present or if aortic narrowing is mild

Differential cyanosis: cyanosis of the lower extremities (when the left subclavian artery outflow is involved, cyanosis might also be seen in the left arm)

Brachial-femoral delay: weak femoral pulses

↑ Blood pressure (BP) in upper extremities and ↓ BP in lower extremities

• If distal narrowing of the left subclavian artery: ↑ BP in both arms and ↓ BP in both legs

• If origin of left subclavian artery is involved: BP in the right arm > left arm.

Cold feet and lower-extremity claudication upon physical exertion

Systolic ejection murmur over left posterior hemithorax and/or continuous murmur below left clavicula and between the shoulder blades

Strong apical impulse displaced to the left

Headache, epistaxis, tinnitus

In severe stenosis: See “Nonspecific findings” and “Heart failure” in “Overview” above.

In severe stenosis: shock and multiorgan failure when ductus arteriosus closes

Blood pressure measurements

• Best initial test

• BP measurements for upper and lower extremities to check for brachial-femoral delay

Pulse oximetry: ↓ SpO2

Doppler echocardiography (confirmatory test): location and extent of stenosis; detection of concurrent anomalies (VSD, PDA, bicuspid aortic valve)

X-ray

• Cardiomegaly and ↑ pulmonary vascular markings

• Figure of 3 sign: the result of an hourglass-like narrowing of the aorta caused by pre- and postdilatation of the aorta with an indentation at the site of coarctation

• Rib notching: a radiographic sign caused by collateral circulation between the internal thoracic and intercostal arteries.

  • Enlarged vessels compress the neighboring ribs, causing pressure atrophy.

  • Classically affects the inferior border of the 3rd– 8thribs

MRI or CT

• In complicated cases and in adults

• To determine the length of coarctation and for intervention planning

Genetic testing: for Turner syndrome

Initial management: infusion of prostaglandin (PGE1)

Surgical correction or balloon angioplasty: for most patients < 5 years of age. Older patients may have a transcatheter intervention with stent placement.

Follow-up and monitor for restenosis, aortic aneurysm, and aortic dissection.

Secondary hypertension

Aortic dissection and rupture

Berry aneurysm → cerebral hemorrhage [22]

Heart failure

Infective endocarditis