---
title: Vascular Ehlers–Danlos syndrome
description: What vascular Ehlers–Danlos syndrome is, why it makes arteries fragile, how it is detected, which treatment and monitoring approaches clinical guidelines describe, and when to call an ambulance.
type: disease
aliases:
  - vEDS
  - vascular EDS
  - Ehlers–Danlos syndrome, vascular type
  - Ehlers–Danlos syndrome type IV
sources:
  - tier: guideline
    org: aha-acc
    title: '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'
    year: 2022
    doi: 10.1161/CIR.0000000000001106
    url: https://doi.org/10.1161/CIR.0000000000001106
  - tier: guideline
    org: esc
    title: 2024 ESC Guidelines for the management of peripheral arterial and aortic diseases
    year: 2024
    doi: 10.1093/eurheartj/ehae179
    url: https://doi.org/10.1093/eurheartj/ehae179
  - tier: guideline
    org: minzdrav-rf
    title: Аневризмы грудной и торакоабдоминальной аорты
    year: 2025
    url: https://cr.minzdrav.gov.ru/view-cr/919_1
  - tier: reference
    org: msd-manuals
    title: Ehlers-Danlos Syndromes (MSD Manual Professional Version)
    year: 2025
    url: https://www.msdmanuals.com/professional/pediatrics/connective-tissue-disorders-in-children/ehlers-danlos-syndromes
  - tier: reference
    org: msd-manuals
    title: Ehlers-Danlos Syndromes (MSD Manual Consumer Version)
    year: 2025
    url: https://www.msdmanuals.com/home/children-s-health-issues/connective-tissue-disorders-in-children/ehlers-danlos-syndromes
sources_checked: 2026-10-14
review: null
---

The Ehlers–Danlos syndromes are inherited disorders of collagen, a protein of connective tissue; 14 clinical types are recognised.<Cite n={[4, 5]} /> The vascular type is one of the most common and the most severe of them.<Cite n={4} /> It is caused by pathogenic variants in the COL3A1 gene, the gene for type III collagen.<Cite n={[1, 3]} /> It leads to spontaneous dissections, aneurysms and ruptures of the <Structure id="aorta">aorta</Structure> and other arteries at a young age.<Cite n={1} />

The American guideline on aortic disease (ACC/AHA) estimates that the vascular type affects about 1 in 50,000 to 100,000 people.<Cite n={1} />

<Diagram name="vascular-ehlers-danlos-syndrome">
  <p slot="normal">
    The diagram shows a segment of an artery cut lengthwise. Connective tissue is the tough, often
    fibrous tissue that binds the body's structures together and gives them support and
    elasticity.<Cite n={5} />
  </p>
  <p slot="disease">
    In vascular Ehlers–Danlos syndrome the arteries are fragile: they can bulge (an aneurysm),
    dissect and rupture.<Cite n={[4, 5]} /> The aorta and its branches can rupture or dissect even
    without significant widening, so the artery in the diagram keeps its width, but its wall is
    torn.<Cite n={1} />
  </p>
</Diagram>

## Why it happens

<Patient>

Connective tissue binds the body's structures together and gives them support and elasticity.<Cite n={5} /> In all types of Ehlers–Danlos syndrome it becomes abnormally fragile.<Cite n={5} /> In the vascular type the gene for type III collagen (COL3A1) is changed, and the arteries become fragile.<Cite n={[1, 3, 4]} /> How early and how severely the arteries are affected is linked to the specific change in the gene.<Cite n={1} />

The condition is inherited: each child of a person with vascular Ehlers–Danlos syndrome has a 50% chance of inheriting it.<Cite n={1} />

</Patient>

<Student>

Inheritance is autosomal dominant.<Cite n={1} /> Vascular Ehlers–Danlos syndrome is one of the syndromic heritable thoracic aortic diseases (HTAD), together with [Marfan syndrome](../marfan-syndrome/) and [Loeys–Dietz syndrome](../loeys-dietz-syndrome/).<Cite n={1} /> The onset and severity of arterial disease correlate with the specific COL3A1 pathogenic variant.<Cite n={1} /> The Russian Ministry of Health guideline lists monogenic connective tissue disorders, including Ehlers–Danlos syndrome, among the risk factors for dilatation of the ascending aorta.<Cite n={3} />

</Student>

<HeartModel highlight={['aorta']} />

## Symptoms

<Patient>

Fragile blood vessels can bulge (an aneurysm), dissect or rupture.<Cite n={[4, 5]} /> Possible problems include a [thoracic aortic aneurysm](../thoracic-aortic-aneurysm/), an [abdominal aortic aneurysm](../abdominal-aortic-aneurysm/), [aortic dissection](../aortic-dissection/) and rupture of arteries.<Cite n={1} /> Importantly, in the vascular type the aorta and its branches can rupture or dissect even when they are not noticeably widened.<Cite n={1} />

Other signs are easy bruising, translucent skin, atrophic scars and increased mobility of the small joints.<Cite n={1} /> Ruptures of the bowel and uterus, of muscles and tendons, and spontaneous pneumothorax (air collecting around a lung) can occur.<Cite n={[1, 4]} /> A common heart complication is [mitral valve prolapse](../mitral-valve-prolapse/).<Cite n={4} />

</Patient>

<Student>

For the vascular type, ACC/AHA list: thoracic and abdominal aortic aneurysms, arterial rupture, aortic dissection, mitral valve prolapse, bowel and uterine rupture, pneumothorax, translucent skin, atrophic scars, small joint hypermobility, easy bruising and carotid-cavernous fistula.<Cite n={1} /> Lethal complications, such as arterial rupture, are much more common in the vascular type than in the other types.<Cite n={4} />

</Student>

## How it is diagnosed

<Patient>

The diagnosis is based on clinical signs and confirmed by a genetic test.<Cite n={4} /> A detailed examination of a skin sample (biopsy) sometimes helps.<Cite n={4} /> If the changed gene is found, testing is recommended for blood relatives who may carry it, whatever their age.<Cite n={[1, 2]} /> Assessment is recommended at a centre with experience in caring for people with inherited aortic diseases.<Cite n={2} />

To find segments of the arteries at risk of rupture or dissection, regular checks of the aorta and peripheral arteries are recommended, using duplex ultrasound, CT or MRI of the blood vessels.<Cite n={[1, 2, 3]} /> How often to do this has not been established: there is no standard approach.<Cite n={[1, 4]} />

</Patient>

<Student>

Genetic confirmation is available for the vascular type and all other types except the hypermobile type; ultrastructural examination of a skin biopsy can help in diagnosing the classical, hypermobile and vascular types.<Cite n={4} /> COL3A1 is one of the 11 genes in genetic testing panels that are confirmed to confer a highly penetrant risk of thoracic aortic disease.<Cite n={[1, 3]} /> Thoracic aortic disease with syndromic features of Marfan syndrome, Loeys–Dietz syndrome or vascular Ehlers–Danlos syndrome is a risk factor for a familial form; in aortic root or ascending aortic aneurysm or thoracic aortic dissection with such risk factors, the ESC recommends genetic counselling at an expert centre and subsequent testing if indicated (I B).<Cite n={[1, 2, 3]} />

Regular surveillance of the aorta and peripheral arteries by duplex ultrasound, CT or MRI is recommended by the ESC (I C) and the Russian Ministry of Health guideline (I C).<Cite n={[2, 3]} /> ACC/AHA describe typical protocols that start with baseline MRI or CT from head to pelvis to evaluate the entire aorta and its branches.<Cite n={1} /> When Ehlers–Danlos syndrome is suspected, other connective tissue disorders with similar joint and skin features are also considered, such as [Marfan syndrome](../marfan-syndrome/).<Cite n={4} />

</Student>

## How it is treated

<Patient>

There is no way to cure Ehlers–Danlos syndrome or to correct the changes in connective tissue.<Cite n={5} /> Management includes patient education, lifestyle changes and avoiding invasive procedures when possible.<Cite n={1} /> Injuries should be avoided as far as possible.<Cite n={4} /> Management is tailored to the individual and decided together with the patient.<Cite n={2} />

The European guideline suggests considering treatment with one particular medicine. The American guideline notes that studies suggest this treatment may be of benefit, but the data were judged insufficient for its official approval in the US.<Cite n={[1, 2]} /> Whether any medicines are needed, and which, is for the doctor to decide.

Surgery on blood vessels is riskier than usual in this syndrome because the vessels are fragile and bleeding is more likely.<Cite n={[1, 3]} /> Rapid growth of an aneurysm or a dissection is a reason for treatment, and there are no data that would allow a threshold aneurysm size for preventive surgery to be set.<Cite n={[1, 3]} /> So the decision on surgery is made by the team of an expert aortic centre together with the patient.<Cite n={[1, 3]} />

There are few data on pregnancy in the vascular type, and the decision about it is difficult; it is made together with the doctors.<Cite n={1} /> Tissue fragility complicates labour and delivery.<Cite n={1} /> Genetic counselling before conception is recommended for couples in which one partner has the condition or is at risk of it.<Cite n={[1, 2]} /> Supervision by an obstetrician during pregnancy and delivery is essential.<Cite n={4} />

</Patient>

<Student>

ESC: treatment with a specific drug should be considered in vascular Ehlers–Danlos syndrome (IIa B); ACC/AHA note that studies suggest a benefit of this treatment, but the data were considered insufficient for its official approval in the US.<Cite n={[1, 2]} /> Open surgery requires meticulous technique to reduce trauma to vessels and tissues; interventional options include arterial embolisation and endovascular therapy, depending on individual circumstances.<Cite n={1} /> For an intact thoracoabdominal aneurysm requiring intervention, ACC/AHA and the Russian Ministry of Health guideline recommend open rather than endovascular repair.<Cite n={[1, 3]} /> For the decision on aortic replacement in dissection, the Russian Ministry of Health guideline recommends involving the team of an expert aortic centre and shared decision-making with the patient (IIb C).<Cite n={3} />

</Student>

## When to call an ambulance

The vascular type leads to spontaneous dissections and ruptures of the aorta and other arteries.<Cite n={1} /> The American guideline lists connective tissue diseases among the high-risk conditions for aortic dissection.<Cite n={1} /> Signs of possible aortic dissection that need an immediate call to your local emergency number (112 in the EU, 911 in the US and Canada, 999 in the UK): sudden, severe pain in the chest or back (sometimes in the abdomen), especially if it is worst at the very start.<Cite n={1} /> The pain of a dissection is not necessarily "tearing": more often it is described as sharp or stabbing.<Cite n={1} /> Aortic dissection can also show itself as fainting, signs of a stroke or sudden paralysis of the legs; in these cases, too, call an ambulance at once.<Cite n={1} /> More about the warning signs is in the article on [aortic dissection](../aortic-dissection/).

Rupture of an artery, rupture of an internal organ and pneumothorax are dangerous complications of the Ehlers–Danlos syndromes, and fatal complications, usually bleeding, occur most often in the vascular type.<Cite n={[4, 5]} /> As with any heart condition, call an ambulance for fainting, chest pain or severe shortness of breath: this is the site's general safety rule, repeated at the bottom of every page.

<Quiz />