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

doi:10.1097/00125817-200205000-00003

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

School of Medicine

Research output: Contribution to journal › Article › peer-review

87 Scopus citations

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.

Original language	English (US)
Pages (from-to)	112-117
Number of pages	6
Journal	Genetics in Medicine
Volume	4
Issue number	3
DOIs	https://doi.org/10.1097/00125817-200205000-00003
State	Published - May 2002

Keywords

Aorta
Aortic dilation
Echocardiogram
Ehlers-Danlos

ASJC Scopus subject areas

Genetics(clinical)

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10.1097/00125817-200205000-00003

Cite this

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title = "Prevalence of aortic root dilation in the Ehlers-Danlos syndrome",

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.",

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AU - Wenstrup, Richard J.

AU - Meyer, Richard A.

AU - Lyle, Jennifer S.

AU - Hoechstetter, Leah

AU - Rose, Peter S.

AU - Levy, Howard P.

AU - Francomano, Claire A.

PY - 2002/5

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N2 - 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.

AB - 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.

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KW - Aortic dilation

KW - Echocardiogram

KW - Ehlers-Danlos

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Prevalence of aortic root dilation in the Ehlers-Danlos syndrome

Abstract

Keywords

ASJC Scopus subject areas

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