Aortic coarctation is characterised by a blood pressure differential between upper and lower extremities (upper >lower).
In neonates, may present with shock if there is inadequate systemic perfusion once the ductus arteriosus closes.
Physical examination may show diminished, absent, or delayed lower extremity pulses compared with upper extremity pulses.
Diagnosis is made by demonstration of aortic arch narrowing, typically by echocardiography.
Treatment may be surgical repair or percutaneous balloon dilation or stent placement.
Long-term complications include persistent hypertension or aneurysm formation at site of surgery or balloon.
Coarctation of the aorta is defined as a narrowing in the aorta, most commonly at the site of insertion of the ductus arteriosus, just distal to the left subclavian artery. Less commonly, there may be diffuse arch hypoplasia with a long segment of narrowing, proximal to the left subclavian artery, or the obstruction may be in the abdominal aorta. It is often associated with a diffuse arteriopathy and bicuspid aortic valve. It typically presents with upper extremity systolic hypertension or murmur. If the narrowing is severe, it may present in the newborn period once the ductus arteriosus closes as low cardiac output and shock (critical coarctation).
History and exam
Key diagnostic factors
- hypertension presenting at a young age or resistant to treatment
- diminished lower extremity pulses
- differential upper and lower extremity blood pressure (BP)
Other diagnostic factors
- systolic ejection murmur
- male sex
- genetic syndrome
- systolic ejection click
- other cardiac anomalies
- male sex
- young age
- family history
- Turner's syndrome
- DiGeorge's syndrome
- hypoplastic left heart syndrome
- Shone's complex
- PHACE syndrome
1st investigations to order
- electrocardiogram (ECG)
- chest x-ray (CXR)
Investigations to consider
- computed tomography (CT) angiography
- magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA)
- cardiac catheterisation
- Aortic stenosis (AS)
- Interrupted aortic arch
- Left ventricular outflow tract obstruction
- 2020 ESC Guidelines for the management of adult congenital heart disease: The Task Force for the management of adult congenital heart disease of the European Society of Cardiology (ESC)
- Management of descending thoracic aorta diseases
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