Acute aortic syndrome (aortic dissection)

Statistics
  • Incidence 3–16/100 000 per year.

  • Men > women 2:1.

  • Peak incidence age 50–60y.

  • True incidence may be underestimated because mortality can be attributed to another cardiac condition.

  • In a city or region of the UK with a population of 2 million people, roughly 140 people per year will die from this acute aortic dissection (Br Journal Cardiol 2023;30:16).

  • Acute aortic dissection accounted for 1542 UK hospital admissions in 2022–23 (out of a total of >6 million) (BMJ 2024;386:e080870).
  • Acute aortic syndrome (AAS) is an umbrella term for a number of conditions affecting the aorta. It encompasses:

    • Acute aortic dissection (accounts for around three-quarters of AAS (BMJ 2024;386:e080870)).
    • Intra-mural haematoma.
    • Penetrating aortic ulcer.

    This article focuses on the emergency presentation of acute aortic syndrome from a primary care perspective, drawing on a Lancet review and a BMJ Easily Missed article (Lancet 2023;401:773, BMJ 2024;386:e080870).

    This article was updated in January 2025.

    When to suspect acute aortic syndrome

    Think of acute aortic syndrome in patients with:

    • Chest, back or abdominal pain.
    • Syncope.
    • Features of malperfusion, e.g. limb, mesenteric or renal ischaemia.

    It is more likely in:

    • Males >50y (but can occur in younger patients).
    • Those with high-risk features (see below).

    Around one-third of patients with AAS will initially be misdiagnosed, which is associated with a doubling of mortality (BMJ 2024;386:e080870).

    High-risk presenting features

    The aortic dissection detection risk score provides a structured algorithm for assessing AAS risk, and may be used in secondary care as part of the assessment of patients with possible aortic dissection symptoms (BMJ 2024;386:e080870).

    Those without any of the high-risk features below have a low risk of acute aortic syndrome. However, if we suspect acute aortic syndrome in primary care (even without high-risk features), we should arrange urgent transfer to secondary care (999).

    Aortic dissection detection risk score (ADD-RS)
    High-risk conditions
  • Marfan syndrome.

  • Family history of aortic disease.

  • Known aortic valve disease.

  • Recent aortic manipulation.

  • Known thoracic aneurysm.
  • Score 1 point for EACH.
    High-risk pain features
    Chest, back or abdominal pain that is described as:
  • Abrupt in onset.

  • Severe in intensity.

  • Tearing or ripping in nature.
  • Score a total of 1 point if ANY are present.
    High-risk examination findings
  • Pulse deficits.

  • Systolic BP differential.

  • Focal neurological deficits with pain.

  • New murmur of aortic insufficiency with pain.

  • Hypotension.
  • Score a total of 1 point if ANY are present.
    Scores ≥2 indicate higher risk. A score of 0 is low risk and may not need further imaging.
    For those with a score of 1, the score may be combined with D-dimer testing to increase specificity.

    The Lancet review highlights some additional factors not considered in the ADD-RS (Lancet 2023;401:773):

    • Medications: quinolones, glucocorticoids, immunosuppressants, cytotoxic medication.
    • Some vascular subtypes of Ehlers-Danlos syndrome.
    • Obstructive sleep apnoea.
    • Certain hereditary cardiovascular malformations such as coarctation and Turner’s syndrome.
    • Acute limb, renal or mesenteric ischaemia.

    Management

    • Arrange urgent hospital transfer via 999 ambulance.
    • Oxygen and advanced life support (if equipped/skilled to provide this) while waiting.
    • Complications can include: aortic rupture/death; pericardial effusion/cardiac tamponade; aortic regurgitation; renal, mesenteric or limb ischaemia.

    Secondary care treatment

    • Type A aortic dissection (ascending aorta or arch): always needs urgent surgical repair.
    • Type B aortic dissection (descending aorta): if stable, medical management to reduce BP and/or heart rate. Surgery may be considered if the patient is unstable/experiencing pain or ischaemia.

    Long-term management

    • Surveillance imaging at 1, 3, 6 and 12m, then annually.
    • Control other cardiovascular risk factors with tight blood pressure control (systolic <120mmHg).
    • Screen relatives if appropriate (secondary care will decide this).
    Acute aortic syndrome
  • High-risk features for acute aortic dissection include:

  • Sudden, severe tearing or ripping pain in the chest, back or abdomen.

    Syncope, hypotension, asymmetry of pulse or BP, focal neurological deficit or new aortic murmur with pain.

    Signs of ischaemia (mesenteric, limb or renal ischaemia).
  • Predisposing conditions include:

  • Marfan syndrome.
    Family or personal history of aortic disease.
    Some connective tissue disorders.

    Obstructive sleep apnoea.

    Medication: quinolones, glucocorticoids, immunosuppressants, cytotoxic medication.
  • Our role in primary care is in recognising acute presentation and arranging urgent (999) transfer to secondary care, and then in long-term management of other cardiovascular risk factors to reduce risk of recurrence.