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


CONCLUSIONS
ARD is a common finding in EDS. Longitudinal studies are indicated to determine progression of ARD and its clinical significance.
Wenstrup
Richard J
RJ
Division of Human Genetics, Children's Hospital Research Foundation, Cincinnati, OH 45229-3039, USA.
Meyer
Richard A
RA
Lyle
Jennifer S
JS
Hoechstetter
Leah
L
Rose
Peter S
PS
Levy
Howard P
HP
Francomano
Claire A
CA
D000293:Adolescent; D000328:Adult; D001011:Aorta / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004108:Dilatation, Pathologic; D004452:Echocardiography; D004535:Ehlers-Danlos Syndrome / Q000473:pathology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged