Last reviewed: June 2020
Last updated: December  2018



History and exam

Key diagnostic factors

  • features of Marfan syndrome
  • features of Ehlers-Danlos syndrome
  • acute severe chest pain
  • interscapular and lower pain
  • left/right blood pressure differential
  • pulse deficit
  • diastolic murmur
  • syncope
  • hypotension

Other diagnostic factors

  • hypertension
  • dyspnoea
  • altered mental status
  • paraplegia
  • hemiparesis/paraesthesia
  • abdominal pain
  • limb pain/pallor
  • left-sided decreased breath sounds/dullness

Risk factors

  • hypertension
  • atherosclerotic aneurysmal disease
  • Marfan syndrome
  • Ehlers-Danlos syndrome
  • bicuspid aortic valve
  • annulo-aortic ectasia
  • coarctation
  • smoking
  • family history of aortic aneurysm or dissection
  • older age
  • giant cell arteritis
  • overlap connective-tissue disorders
  • surgical/catheter manipulation
  • cocaine/amfetamine use
  • heavy lifting
  • pregnancy

Diagnostic investigations

1st investigations to order

  • ECG
  • chest x-ray
  • cardiac enzymes
  • computed tomography angiography
  • renal function tests
  • liver function tests
  • lactate
  • full blood count
  • type and cross
More 1st investigations to order

Investigations to consider

  • D-dimer
  • trans-thoracic echocardiography
  • trans-oesophageal echocardiography
  • magnetic resonance angiography
  • intravascular ultrasound
  • smooth muscle myosin heavy chain protein
More investigations to consider

Treatment algorithm


Assistant Professor of Surgery

Division of Vascular Surgery and Endovascular Therapy

The Johns Hopkins University School of Medicine




CWH declares that she has no competing interests.


Vascular Surgery and Endovascular Therapy

Department of Surgery

The Johns Hopkins University School of Medicine




JHB declares that he has no competing interests.

Dr Caitlin W. Hicks and Dr James H. Black would like to gratefully acknowledge Professor Hazim J. Safi, Dr Jeffrey Wu, and Dr Eric E. Roselli, previous contributors to this topic. HJS, JW, and EER declare that they have no competing interests.

Peer reviewersVIEW ALL

Associate Professor of Surgery

Acting Chief

Division of Vascular and Endovascular Surgery

Montefiore Medical Center and the Albert Einstein College of Medicine




EL declares that he has no competing interests.

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