Summary
Definition
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
- claudication
- headache
- systolic ejection click
- other cardiac anomalies
Risk factors
- male sex
- young age
- family history
- Turner syndrome
- DiGeorge syndrome
- hypoplastic left heart syndrome
- Shone complex
- PHACE syndrome
Diagnostic tests
1st tests to order
- electrocardiogram (ECG)
- chest x-ray (CXR)
- echocardiogram
Tests to consider
- computed tomography (CT) angiography
- magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA)
- cardiac catheterization
Treatment algorithm
critical coarctation
noncritical coarctation
recurrent coarctation
Contributors
Authors
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
NY
Disclosures
JG declares that he has no competing interests.
Acknowledgements
Dr Jeffrey Gossett wishes to gratefully acknowledge Dr Anna Kamp, a previous contributor to this topic. AK declares that she has no competing interests.
Disclosures
AK declares that she has no competing interests.
Peer reviewers
Sheba John, MD, FAAP
Associate Professor
Pediatric Cardiology/Adult Congenital Cardiology
University of Utah
Salt Lake City
UT
Disclosures
SJ declares that she has no competing interests.
Daniel A. Cox, DO, FACC, FAAP, FAHA
Associate Professor
Adult Congenital and Pediatric Cardiology
University of Utah/Primary Children's Hospital
Salt Lake City
UT
Disclosures
DAC 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
London
UK
Disclosures
SK declares that he has no competing interests.
References
Key articles
Erbel R, Aboyans V, Boileau C, et al; Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). 2014 ESC guidelines on the diagnosis and treatment of aortic diseases: document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. Eur Heart J. 2014 Nov 1;35(41):2873-926.Full text
Writing Committee Members, Isselbacher EM, Preventza O, et al. 2022 ACC/AHA Guideline for the diagnosis and management of aortic disease: A report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2022 Dec 13;80(24):e223-e393.Full text Abstract
Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J. 2020 Aug 29;ehaa554.Full text
Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on clinical practice guidelines. J Am Coll Cardiol. 2019 Apr 2;73(12):e81-192.Full text
Writing Committee; Riambau V, Böckler D, Brunkwall J, et al. Editor's choice - management of descending thoracic aorta diseases: clinical practice guidelines of the European Society for Vascular Surgery (ESVS). Eur J Vasc Endovasc Surg. 2017 Jan;53(1):4-52.Full text
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Aortic stenosis (AS)
- Interrupted aortic arch
- Left ventricular outflow tract obstruction
More DifferentialsGuidelines
- ACR appropriateness criteria: congenital or acquired heart disease
- Guidelines for cardiovascular intervention in adults with congenital heart disease
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