Ehlers-Danlos with disease of the aorta and mitral valve

A woman aged 46 years with a history of vascular Ehlers Danlos syndrome presented with atypical chest pain. She had previously required a mitral valve replacement for mitral valve prolapse. She was found to have a Stanford type A aortic dissection and underwent aortic root, ascending aorta, and hemiarch replacement. The features that should alert clinicians to aortic dissection are reviewed, along with a brief discussion of the manifestations of vascular Ehlers Danlos syndrome.