Vascular Ehlers-Danlos Syndrome — EECC MCQ

Correct answer: C — Yes — in vascular EDS, the aorta is at risk of rupture or dissection at much smaller diameters than in Marfan syndrome due to extreme arterial fragility; close surveillance and avoidance of arterial interventions where possible is recommended

Vascular Ehlers-Danlos syndrome (vEDS, type IV, COL3A1 mutations) is characterised by extreme arterial fragility. Unlike Marfan syndrome (where the aorta is elastic but prone to progressive dilatation), vEDS arteries are structurally weak with a propensity for spontaneous rupture or dissection at relatively normal diameters. Standard surgical thresholds from Marfan syndrome do NOT apply — arterial rupture can occur without prior dilatation. The 2025 ESC CVD in Pregnancy Guidelines classify vEDS as mWHO IV due to the high risk of aortic/arterial rupture. Surgical intervention is avoided when possible because tissue friability makes surgical repair extremely challenging. Celiprolol has shown some benefit in reducing vascular events (BBEST trial).

Reference: ESC (2024): Aortic Disease Guidelines; ESC (2025): CVD in Pregnancy Guidelines