Prevalence of aortic root dilation in the Ehlers-Danlos syndrome

Richard J. Wenstrup, Richard A. Meyer, Jennifer S. Lyle, Leah Hoechstetter, Peter S. Rose, Howard P. Levy, Claire A. Francomano

Research output: Contribution to journalArticlepeer-review

87 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)112-117
Number of pages6
JournalGenetics in Medicine
Issue number3
StatePublished - May 2002
Externally publishedYes


  • Aorta
  • Aortic dilation
  • Echocardiogram
  • Ehlers-Danlos

ASJC Scopus subject areas

  • Genetics(clinical)


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