[HTML][HTML] Vascular Ehlers-Danlos syndrome management: the Paris way, a step forward on a long road
J De Backer, T De Backer - Journal of the American College of Cardiology, 2019 - jacc.org
J De Backer, T De Backer
Journal of the American College of Cardiology, 2019•jacc.orgOf all hereditary connective tissue diseases, vascular Ehlers-Danlos Syndrome (vEDS) is
pre-eminently the most feared—mainly due to the unpredictability in the occurrence of
potentially serious/fatal vascular events such as arterial dissection or rupture. This rare
condition is caused by pathogenic variants in the COL3A1 gene and affects the digestive
system, the uterus, the skin, and the pulmonary system in addition to the vascular system (1).
The median life expectancy—based on several previous large-scale studies—is 48 to 51 …
pre-eminently the most feared—mainly due to the unpredictability in the occurrence of
potentially serious/fatal vascular events such as arterial dissection or rupture. This rare
condition is caused by pathogenic variants in the COL3A1 gene and affects the digestive
system, the uterus, the skin, and the pulmonary system in addition to the vascular system (1).
The median life expectancy—based on several previous large-scale studies—is 48 to 51 …
Of all hereditary connective tissue diseases, vascular Ehlers-Danlos Syndrome (vEDS) is pre-eminently the most feared—mainly due to the unpredictability in the occurrence of potentially serious/fatal vascular events such as arterial dissection or rupture. This rare condition is caused by pathogenic variants in the COL3A1 gene and affects the digestive system, the uterus, the skin, and the pulmonary system in addition to the vascular system (1).
The median life expectancy—based on several previous large-scale studies—is 48 to 51 years (2, 3). The age of onset of vascular events shows a large variability, but a majority of the initial events occur in the second to third decade of life (4); however, especially in men, increased mortality in adolescence has been noted (3).
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