Nomenclature of Vascular Ehlers-Danlos Syndrome
The abbreviation "v-EDS" (or vEDS) is used instead of "A-EDS" because the condition is called "vascular Ehlers-Danlos syndrome," not "arterial Ehlers-Danlos syndrome." The "v" stands for "vascular," which reflects the broader spectrum of vascular complications affecting arteries, veins, and other blood vessels throughout the body.
Why "Vascular" Rather Than "Arterial"
The terminology reflects the comprehensive nature of the disease:
Multiple vascular territories are affected: While arterial dissections, aneurysms, and ruptures are the most life-threatening complications, the syndrome affects the entire vascular tree, not just arteries 1, 2
Historically designated as "Type IV": The older classification system referred to this as "Ehlers-Danlos syndrome, vascular form or Type IV" 3, but modern nomenclature has moved away from numerical typing to descriptive names
Consistent international terminology: All major guidelines—including the 2024 ESC Guidelines 1, 2022 ACC/AHA Guidelines 2, and 2010 ACC/AHA/AATS Guidelines 3—uniformly use "vascular Ehlers-Danlos syndrome" abbreviated as "vEDS" or "v-EDS"
Standardized Nomenclature Across Medical Literature
The abbreviation "vEDS" appears consistently throughout:
- Clinical practice guidelines from major cardiovascular societies 1, 2
- Research literature describing diagnosis and management 4, 5, 6, 7, 8
- Genetic and molecular studies identifying COL3A1 pathogenic variants 1, 5, 6
The term "vascular" is more accurate because it encompasses the full range of complications: arterial dissections and ruptures (most common in medium-sized arteries), arteriovenous fistulas, and complications affecting hollow organs like the bowel and gravid uterus that share the same underlying collagen defect 1, 2.
Using "vEDS" maintains consistency with international medical literature and accurately reflects the systemic vascular nature of this rare autosomal dominant disorder caused by COL3A1 gene mutations 1, 2, 5.