(VII- I-2A.2) Postpartum aortic dissection: Ehlers Danlos Syndrome Agnieszka Rosławiecka MD, Anna Kabłak-Ziembicka MD, PhD, Mariusz Trystuła, MD, PhD, Tadeusz Przewłocki MD, PhD, Prof. Piotr Podolec MD, PhD
Background The Ehlers-Danlos Syndrome (EDS) is clinically and genetically heterogenic group of connective tissue diseases caused by collagen biosynthesis impropriety (1:25 000). The Vascular type of EDS is caused by mutation in gene for type 3 procollagen (COL3A1). The vascular type of EDS is rare (<4%) but the most dangerous type of EDS. Vascular walls defect caused by collagen biosynthesis impropriety stand for propensity to spontaneous ruptures and dissections of thin-walled arteries. Vessel rupture is typical for not primarily widened …