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Last reviewed: 21 Apr 2025
Last updated: 10 Dec 2024

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's syndrome
  • DiGeorge's syndrome
  • hypoplastic left heart syndrome
  • Shone's complex
  • PHACE syndrome

Diagnostic investigations

1st investigations to order

  • electrocardiogram (ECG)
  • chest x-ray (CXR)
  • echocardiogram

Investigations to consider

  • computed tomography (CT) angiography
  • magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA)
  • cardiac catheterisation

Treatment algorithm

Contributors

Authors

Jeffrey Gossett, MD
Jeffrey Gossett

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

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

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

Reference articles

A full list of sources referenced in this topic is available here.

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