Ehlers-Danlos syndrome is a group of syndrome related to connective tissue disorder.1 Genetic causes determine how a person with Ehlers-Danlos syndrome is going to be affected. Various signs and symptoms of the syndrome include tissue fragility, joint hypermobility, and skin hyperextensibility. There are thirteen kinds of Ehlers-Danlos syndrome and one of them is hypermobile type. The gene mutation is the cause of Ehlers-Danlos syndrome.1 While the gene mutations of most of the type are known, in case of the hypermobile type it is yet to be determined. Due to the unavailability of the genetic or molecular cause responsible for the Ehlers-Danlos syndrome hypermobile type, the tests that can confirm and diagnose the syndrome of hypermobile type are unavailable too. The nature of inheritance is that of autosomal dominant manner.

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Cardiovascular Autonomic Dysfunction in Ehlers-Danlos Syndrome Hypermobile Type

An individual’s heart rate and blood pressure are looked after and also controlled by one’s autonomic nervous system.2 Autonomic dysfunction disrupts normal functioning within the body and is capable of making long term and life-threatening damages. The heart rate increases in many cases leading to heart complications. Other kinds of symptoms that become prevalent are low blood pressure, digestive system disorders, disturbed bladder function and sweat regulation. Autonomic dysfunction affects the heart. The cardiovascular blood vessels are also affected and are seen to generate certain symptoms like lightheadedness and fainting.

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Studies and research have revealed a connection between the autonomic nervous system and Ehlers-Danlos syndrome hypermobile type. The symptoms described above which are typically exhibited by persons suffering from autonomic nervous system dysfunction can also be found in the individuals suffering from Ehlers-Danlos syndrome hypermobile type. The similarity in symptoms includes heart rate, cardiovascular blood vessels, digestive system problems and pupil, bladder and sweating dysfunction.

Causes of Cardiovascular Autonomic Dysfunction in Ehlers-Danlos Syndrome Hypermobile Type

The actual causes that might be responsible for cardiovascular autonomic dysfunction in Ehlers-Danlos hypermobile type have still not been determined. The fact that the causes are unknown might also cause certain problems during diagnosis. Connective tissues, when becoming abnormal, can be said to cause the symptoms. This abnormality often leads to lightheadedness. Another factor that can be held responsible is nerve damage, loose connective tissue and sometimes medications with side effects. 29 percent of patient’s possessed overactive noradrenaline, a study revealed[1]. These patients also had a fast heart rate which happened after standing. This has also been stated as a cause.

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Signs and Symptoms Associated with Cardiovascular Dysfunction in Ehlers-Danlos Syndrome Hypermobile Type

The signs and symptoms are various and should not be neglected under any cost. Common symptoms are low blood pressure and at the same, the blood pooling level is also seen to increase especially in the legs after one tries to stand and also around the gut often after eating. People with hypermobility syndrome develop a kind of abnormality in the major blood cells and are seen to be able to stretch beyond normal in a way that suggests too much mobility or in some cases hypermobility. Other symptoms include low circulation in blood volumes, problems or complications arising out of medications with side effects, the autoimmune system related issues and problem related to autonomic system that regulate the heart rate, and blood pressure.

Diagnosis of Cardiovascular Autonomic Dysfunction in Ehlers-Danlos Syndrome Hypermobile Type

Since, the symptoms are difficult to understand, the diagnosis can be faulty. One of the key things that the doctor needs to keep in mind while diagnosing a person is the symptoms alongside a detailed medical history of the patient. Other important elements that might help in diagnosis are modifying factors, triggers, the effect on daily life and family history. Diagnosis can be a bit difficult in this particular case.

Treatment of Cardiovascular Autonomic Dysfunction in Ehlers-Danlos Syndrome Hypermobile Type

Treatments for cardiovascular autonomic dysfunction in Ehlers-Danlos syndrome hypermobile type hasn’t been developed yet. The kind of treatments that are used is of non-drug variety. Different kinds of exercises are recommended. For a successful result, it is advised that meal be avoided one hour before exercise.

Conclusion

Cardiovascular autonomic dysfunction in Ehlers-Danlos syndrome hypermobile type needs a lot of research which will help the doctors identify other causes of the disease better. Extensive research about the present factors and symptoms of the disease can help create awareness which can save many lives and help the patients in living a better life.

References:  

Reference:  

  1. Tinkle, B., Castori, M., Berglund, B., Cohen, H., Grahame, R., Kazkaz, H., & Levy, H. Hypermobile Ehlers-Danlos syndrome (a.k.a. Ehlers-Danlos syndrome Type III and Ehlers-Danlos syndrome hypermobility type): Clinical description and natural history. American Journal Of Medical Genetics Part C: Seminars In Medical Genetics, (2017). 48-69. doi: 10.1002/ajmg.c.31538

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Pramod Kerkar

Written, Edited or Reviewed By:

, MD,FFARCSI

Pain Assist Inc.

Last Modified On: August 19, 2019

This article does not provide medical advice. See disclaimer

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