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Genet Med. 2002 May-Jun;4(3):112-7.

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

Author information

1
Division of Human Genetics, Children's Hospital Research Foundation, Cincinnati, OH 45229-3039, USA.

Abstract

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.

PMID:
12180144
DOI:
10.109700125817-200205000-00003
[Indexed for MEDLINE]

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