What Every Vascular Surgeon Should Know About Vascular Ehlers-Danlos Syndrome.

Vascular Ehlers-Danlos syndrome (VEDS) vascular type is a rare autosomal dominant disorder caused by pathogenic variants in the COL3A1, resulting in abnormal type III collagen and a high risk of arterial dissection, rupture, and other life-threatening complications at a young age. Diagnosis requires a high index of clinical suspicion and confirmatory genetic testing, which also enables cascade screening and informs prognosis through genotype-phenotype correlations. Management is centered on multidisciplinary care, including vascular surgery, cardiology, and genetics, with baseline head-to-pelvis vascular imaging followed by annual to biannual surveillance. Medical therapy emphasizes strict blood pressure control, typically with beta-blockers and angiotensin receptor blockers, alongside lifestyle modification and avoidance of high-risk medications. Although historically associated with high morbidity, both open and endovascular interventions are increasingly feasible with careful patient selection and meticulous technique, though risks of iatrogenic injury and device-related complications remain substantial. Longitudinal care requires ongoing surveillance and psychosocial support, and pregnancy carries significant maternal risk necessitating specialized management. Advances in genetic characterization and operative strategies have improved outcomes; however, substantial morbidity persists, and future efforts are focused on integrating biologic and ultrastructural markers of tissue integrity to refine risk stratification and enable personalized decision making.