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
Jeffrey Gossett, MD
Vice President and System Chief of Pediatric Cardiology
Professor of Pediatrics
Division of Pediatric Cardiology
Cohen Children’s Medical Center, Northwell Health
New Hyde Park
JG declares that he has no competing interests.
Dr Jeffrey Gossett wishes to gratefully acknowledge Dr Anna Kamp, a previous contributor to this topic. AK declares that she has no competing interests.
AK declares that she has no competing interests.
Ranjit Aiyagari, MD
Clinical Assistant Professor of Pediatrics
University of Michigan
RA declares that he has no competing interests.
Sachin Khambadkone, MD, DCH, DNB
Consultant Paediatric Cardiologist and Honorary Senior Lecturer
Great Ormond Street Hospital and Institute of Child Health
SK declares that he has no competing interests.
- 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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