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.^[1]

About 1 in 3 people with hypermobile EDS have autonomic dysfunction. 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 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.^[1]

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.^[1]

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

The long-term outlook (prognosis) for people with hypermobile Ehlers-Danlos syndrome 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.^[2][3]