Should we be concerned about the potential for heart and/or cardiovascular problems?

Structural cardiac malformations are uncommon in the classic type. Mitral-valve prolapse and, less frequently, tricuspid valve prolapse may occur and can be diagnosed by echocardiography, CT or MRI. Stringent criteria should be used for the diagnosis of mitral valve prolapse. Spontaneous rupture of large arteries, along with intracranial aneurysms and arteriovenous fistulae, may occur in the rare patient with a severe form of classic EDS. Clinicians should consider evaluating for the presence, development and progression of aortic root dilation (ARD) in patients who have EDS diagnosed by current clinical criteria. [Wenstrup et al. Genet Med 2002; 4 (3): 112–117.]

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