Vascular EDS

Tissue vulnerability revealed by ultrastructural skin analysis as a surrogate for arterial integrity in two cases of vascular Ehlers-Danlos syndrome.

Clinical presentation and operative outcomes in vascular Ehlers-Danlos syndrome (VEDS) are heterogeneous. We present two patients with genetically confirmed VEDS who exhibited markedly different iliac pathology phenotypes. One ruptured a common iliac artery at a near-normal diameter and the other to…

Genetic and molecular mechanisms of hereditary thoracic aortic aneurysm and dissection (Review).

Thoracic aortic aneurysm and dissection (TAAD) is a life‑threatening disease with an insidious onset and a largely elusive pathogenesis. Hereditary TAAD (HTAD) can be classified into syndromic forms, including Marfan syndrome, Loeys‑Dietz syndrome and vascular Ehlers‑Danlos syndrome and non‑syndromi…

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…

Sex differences in aortopathy, arteriopathy, and mortality in vascular Ehlers-Danlos syndrome.

Vascular Ehlers-Danlos syndrome (VEDS) is a rare autosomal dominant disorder due to pathogenic alterations in type III collagen structure and production, which results in increased risk of aortopathy and arteriopathy. Given the documented sex-related differences in aortic and other arterial aneurysm…

Vascular Ehlers-Danlos syndrome: should we treat asymptomatic patients?

Spontaneous celiac artery dissection revealing vascular Ehlers-Danlos syndrome.

Recurrent Acute Coronary Syndrome in Spontaneous Coronary Artery Dissection: Beyond the Guidelines.

Spontaneous coronary artery dissection (SCAD) is an important cause of acute coronary syndrome (ACS) in vascular Ehlers-Danlos syndrome (vEDS). A 35-year-old with vEDS, found to have SCAD on coronary computed tomography angiography, was admitted with recurrent episodes of ACS. After each event, a he…

Abdominal Aortic Pseudoaneurysm: A Rare Presentation of an Uncommon Disease.

Vascular Ehlers-Danlos syndrome (vEDS) is a rare connective tissue disorder characterized by arterial fragility and life-threatening vascular complications. Presentation in pediatric age group patients remains rare and is therefore not well characterized. A 14-year-old man presented with severe abdo…