Aortic aneurysm, dissection, and rupture are known to occur in some of the rarer types of the Ehlers-Danlos syndromes. This can also occur in the medium and smaller arteries. Typically these arterial complications are recognized as a complication of the vascular type of Ehlers-Danlos syndrome (vEDS), but are also a greater risk in classical (cEDS), kyphoscoliotic (kEDS), and dermatosparaxis Ehlers-Danlos syndrome (dEDS) in particular. (D’hondt S. et al. 2018).
Dilation of the aortic root, the section of the aorta closest to the heart, is also a concern for some people with Ehlers-Danlos syndromes and is managed by experts in a similar way to other Heritable Disorders of Connective Tissue such as Marfan syndrome, and Loeys-Dietz syndrome (Saliba E et al. 2015, Rozado J et al. 2017).
“Aortic and other arterial dissection and rupture is a major complication and a cause of death in vascular EDS and other rare types of EDS.” states Assoc. Prof. Dr. Alan Hakim, Consultant Rheumatologist, Acute Physician, and Director of Education for The Ehlers-Danlos Society. “By raising awareness of the risks and their management, and supporting research to identify the underlying disease mechanisms and treatment, we aim 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 patients in order to improve outcomes in the event of an aortic or other arterial dissection:
- Form a knowledgeable medical care team and develop an emergency protocol in case of a vascular event.
- Screen regularly for possible arterial complications that may not yet be symptomatic.
- Keep blood pressure in the low-to-normal range to reduce strain on arterial walls.
- Know the signs and symptoms of arterial dissection and seek medical care immediately.
For more information on the Ehlers-Danlos syndromes:
Subtypes of Ehlers-Danlos syndrome
Diagnosis, natural history, and management in vascular Ehlers–Danlos syndrome.
D’Hondt S et al. Vascular phenotypes in nonvascular subtypes of the Ehlers-Danlos syndrome: a systematic review
Saliba E, Sia Y; In collaboration with. The ascending aortic aneurysm: When to intervene?. Int J Cardiol Heart Vasc. 2015;6:91-100.
Rozado J, Martin M, Pascual I, Hernandez-Vaquero D, Moris C. Comparing American, European and Asian practice guidelines for aortic diseases. J Thorac Dis. 2017;9(Suppl 6):S551-S560