Aortic Dissection: A life-threatening complication

September 19 is Aortic Dissection Awareness Day

Arteries are tubes that carry blood from the heart to the rest of the body. Arterial aneurysm, dissection, and rupture are key features of vascular Ehlers-Danlos syndrome (vEDS). There is also a greater risk for these complications in classical EDS (cEDS), kyphoscoliotic EDS (kEDS), and dermatosparaxis EDS (dEDS). These complications can affect minor and major arteries, including the aorta, which is the largest artery in the body. 

“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. 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.” – Assoc. Prof. Dr. Alan Hakim, Consultant Rheumatologist, Acute Physician, and Director of Education for The Ehlers-Danlos Society.

The International Consortium on EDS & HSD Vascular Working Group recommends the following strategies for people with vEDS in order to improve outcomes of arterial complications: 

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

For more information on the Ehlers-Danlos syndromes: 

Types of Ehlers-Danlos syndromes 

Diagnosis, natural history, and management in vascular Ehlers-Danlos syndrome (Byers et al., 2017) 

Vascular phenotypes in nonvascular subtypes of the Ehlers-Danlos syndrome: a systematic review (D’hondt et al., 2017) 

The ascending aortic aneurysm: When to intervene? (Saliba et al., 2015) 

Comparing American, European and Asian practice guidelines for aortic diseases (Rozado et al., 2017) 

A multi-institutional experience in the aortic and arterial pathology in individuals with genetically confirmed vascular Ehlers-Danlos syndrome (Shalhub et al., 2019) 

Assessment of arterial damage in vascular Ehlers-Danlos syndrome: A retrospective multicentric cohort (Adham et al., 2022) 

Cardiovascular Management of Aortopathy in Children: A Scientific Statement From the American Heart Association (Morris et al., 2024)