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Diseases

Genetic and Rare Diseases Information Center (GARD)

Ehlers-Danlos syndrome, hypermobility type


Other Names for this Disease
  • Benign joint hypermobility syndrome
  • BJHS
  • EDS III
  • EDS3 (formerly)
  • Ehlers-Danlos syndrome type 3
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

My husband has Ehlers Danlos syndrome hypermobility type. He has severe syncope and very low blood pressure. What heart conditions are associated with Ehlers Danlos syndrome hypermobility type? Do people with Ehlers Danlos syndrome hypermobility type have a shortened life expectancy?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What heart symptoms are associated with Ehlers-Danlos syndrome, hypermobility type?

Three heart problems associated with Ehlers-Danlos syndrome (EDS), hypermobility type include autonomic dysfunction, aortic root dilatation (enlargement of the blood vessel that distributes blood from the heart to the rest of the body), and mitral valve prolapse.[1]

Autonomic dysfunction is experienced by one third (1 in 3) to one half (1 in 2) of people with EDS, hypermobility type. Symptoms include:[1]
  • Atypical chest pain
  • Palpitations at rest or on exertion
  • Orthostatic intolerance (an inability to maintain normal blood pressure when you stand up)
  • Neurally mediated hypotension (a sudden drop in blood pressure after standing for long periods, leading to symptoms such as dizziness, nausea, and fainting)
  • Postural orthostatic tachycardia syndrome (fast heart beat that occurs when a person stands up)

Aortic root dilatation is usually mild and occurs in one fourth (1 in 4) to one third (1 in 3) of people with EDS, hypermobility type. Risk for sudden death and aortic dissection is not increased unless there is significant dilatation. Currently, the long-term stability and ultimate prognosis of aortic root dilatation in people with EDS, hypermobility type is not known.[1]

The association of mitral valve prolapse and EDS, hypermobility type is still not certain. However it is possible that mild mitral valve prolapse may explain some of the chest pain and palpitations experienced by people with this form of EDS.[1]
Last updated: 5/26/2015

How might heart symptoms experienced by people with Ehlers-Danlos syndrome, hypermobility type be treated?

In general, aortic root enlargement in people with Ehlers-Danlos syndrome (EDS), hypermobility type may be treated with medications called beta blockers. Severe enlargement (greater than 4.5 - 5.0 cm), which is very rare in people with EDS, hypermobility type, may require surgery.[2]

Often the mitral valve prolapse is too mild to meet diagnostic criteria, and may not require special monitoring or treatment.[2]

Autonomic dysfunction, specifically hypotension and postural orthostatic tachycardia is treated as usual, with sodium and water to expand the blood volume, beta-blockade, fludrocortisone, and/or stimulants.[2]

To learn more about your treatment options we recommend that you speak with your healthcare provider.
Last updated: 5/26/2015

What is the long-term outlook for people with Ehlers-Danlos syndrome, hypermobility type?

The long-term outlook (prognosis) for people with Ehlers-Danlos syndrome (EDS), hypermobility type depends on the severity of the condition and the signs and symptoms present. Although this form of EDS does not typically impact life expectancy, musculoskeletal (muscle and bone) pain and joint instability can have a significant impact on daily function and quality of life.[3][2]
Last updated: 5/26/2015

References
Other Names for this Disease
  • Benign joint hypermobility syndrome
  • BJHS
  • EDS III
  • EDS3 (formerly)
  • Ehlers-Danlos syndrome type 3
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.