Abstract
Connective tissue disorders (CTDs) such as Marfan syndrome (MFS), Loeys-Dietz syndrome (LDS), vascular Ehlers-Danlos syndrome (vEDS), and related disorders have clinical and molecular diagnostic criteria, known risks for aortic dilation and dissection, and guidelines for cardiac surveillance and management. Some patients, however, do not meet diagnostic criteria for a well characterized genetic CTD but possess physical signs and symptoms consistent with connective tissue involvement. For this population of patients with CTD not otherwise specified (CTD-NOS), few data exist about cardiac risk or other morbidity.
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