Abstract

Ehlers-Danlos syndrome (EDS) is a heterogeneous group of hereditary disorders of connective tissue affecting the skin, ligaments, joints, blood vessels, and internal organs. Hypermobile EDS (hEDS) is the most common and the least severe subtype of EDS, characterized by generalized joint hypermobility, doughy skin and multi-systems involvement. Cardiovascular system involvements have been frequently reported in hEDS, and mitral valve prolapse and aortic root dilatation have been included in the 2017 international diagnostic criteria for hEDS.

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