Prevalence of aortic root dilation in the Ehlers-Danlos syndrome.

PURPOSE: To determine the prevalence of proximal aortic abnormalities in patients with Ehlers-Danlos syndrome (EDS).

METHODS

In a prospective cohort study, aortic measurements by two-dimensional echocardiography were performed on consecutive EDS patients.

RESULTS

Twenty-eight percent (20 of 71) had aortic root dilation (ARD) (> +2 SD above population based norms). Fourteen of 42 individuals with the classical form of EDS (types I/II) and 6 of 29 individuals with the hypermobile form (type III) had ARD, with no gender differences.

CONCLUSION

ARD is a common finding in EDS. Longitudinal studies are indicated to determine progression of ARD and its clinical significance.