Cross-sectional and longitudinal assessment of aortic root dilation and valvular anomalies in hypermobile and classic Ehlers-Danlos syndrome.

This study examines the prevalence and progression of aortic root dilation and valvular anomalies in individuals with hypermobile and classic Ehlers-Danlos Syndrome (EDS). The research involved a retrospective chart review to assess the prevalence of aortic dilation and mitral valve prolapse (MVP) and analyzed the longitudinal progression of aortic root size. Findings suggest that aortic dilation is less likely in patients whose first echocardiogram occurs in adolescence or adulthood compared to those in early childhood. Longitudinally, few cases of aortic dilation persisted beyond age 14, and no cases of new dilation occurred in adulthood when the aortic root was normal in childhood. MVP was present in 6% of patients, but mostly mild in nature. Despite these findings, aortic root size and MVP seem to have limited clinical impact, indicating that echocardiography could still be used for cardiovascular assessment, but screenings can be less frequent in asymptomatic adults.