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What heart symptoms are associated with hypermobile Ehlers-Danlos syndrome?

Three heart problems associated with hypermobile Ehlers-Danlos syndrome 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

About 1 in 3 people with hypermobile EDS have autonomic dysfunction. Symptoms include:

  • 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 hypermobile EDS. 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.

The association of mitral valve prolapse and hypermobile EDS 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.