Aortic Dissection: A life-threatening complication for vascular Ehlers-Danlos syndrome

Posted September 18, 2018

The 19th of September is Aortic Dissection Awareness Day. Patients, families, clinicians, and organizations around the world will gather to share their stories and educate others about this life-threatening condition.

Arterial disease, including aneurysm and dissection, is known to occur in some of the more rare types of the Ehlers-Danlos syndromes. This can occur in the medium and smaller arteries. Aortic disease is seen primarily in the vascular type of Ehlers-Danlos syndrome (vEDS).

“Aortic and other arterial dissection is a major complication and a cause of death in vascular EDS.” states Dr. Alan Hakim, Chief Medical Officer for The Ehlers-Danlos Society. “By raising awareness we hope to improve patient outcomes and save lives.”

An aortic dissection is a tear in the inner layer of the aorta, the major artery carrying blood from the heart to the rest of the body. The tear causes the aorta to swell and may burst if medical intervention is not performed immediately.

The Ehlers-Danlos International Consortium Vascular Committee recommends the following strategies to vEDS patients in order to improve outcomes in the event of an aortic or other arterial dissection:

  1. Form a knowledgeable medical care team and develop an emergency protocol in case of a vascular event.
  2. Screen regularly for possible arterial complications that may not yet be symptomatic.
  3. Keep blood pressure in the low-to-normal range to reduce strain on arterial walls.
  4. Know the signs and symptoms of an arterial dissection and seek medical care immediately.

The Ehlers-Danlos Society demonstrates its commitment to awareness, education, and research for aortic and arterial dissections and other manifestations of vascular EDS through our work with the EDS International Consortium Vascular Committee and the vEDS Collaborative.

“We are honored to work with world-class researchers, clinicians, and patient organizations in the vEDS Collaborative as we seek to increase early detection of aortic and arterial dissection and improve patient outcomes. Together, we can push forward to save and improve lives.” states Lara Bloom, International Executive Director.

In addition, The Ehlers-Danlos Society will be participating in the upcoming GenTAC Alliance Aortic Summit. Lara Bloom has been invited to join the Patients, Families, and Clinicians Working Group for the GenTAC Alliance, which works to educate and communicate with patients, families, and clinicians about genetically-triggered thoracic aortic conditions.

A variety of Aortic Dissection Awareness events are being held around the world, from Fort Worth, Texas, to Stockholm, Sweden, to Dhaka, Bangladesh. These events range from medical conferences to patient gatherings. A full listing of events can be found here.

For more information on vascular Ehlers-Danlos syndrome:
Vascular Ehlers-Danlos syndrome
Diagnosis, natural history, and management in vascular Ehlers–Danlos syndrome.
Vascular phenotypes in nonvascular subtypes of the Ehlers-Danlos syndrome: a systematic review

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