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

Division of Human Genetics, Children's Hospital Research Foundation, Cincinnati, OH 45229-3039, USA.
Genetics in Medicine (Impact Factor: 6.44). 06/2002; 4(3):112-7. DOI: 10.1097/00125817-200205000-00003
Source: PubMed

ABSTRACT To determine the prevalence of proximal aortic abnormalities in patients with Ehlers-Danlos syndrome (EDS).
In a prospective cohort study, aortic measurements by two-dimensional echocardiography were performed on consecutive EDS patients.
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.
ARD is a common finding in EDS. Longitudinal studies are indicated to determine progression of ARD and its clinical significance.

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