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April 24, 2017American Journal of Medical Genetics Part A

Natural history of aortic root dilation through young adulthood in a hypermobile Ehlers–Danlos syndrome cohort

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Key result

Hypermobile Ehlers-Danlos syndrome carries ~14% aortic root dilation prevalence with no significant progression over time.

  • n=325

Population

325 patients with hypermobile Ehlers-Danlos syndrome at Cincinnati Children's Hospital Medical Center…

Design

Cohort

Authors

ARAlyssa RitterCACarrie AtzingerBHBrandon Hays

Discussion

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Overview

May support de-escalating routine echocardiography in hypermobile Ehlers-Danlos syndrome; leaves open need for prospective confirmation.

Study Design

Type

Cohort (n=325)

Multicenter

No

Structured PICO

P
Population
325 patients with hypermobile Ehlers-Danlos syndrome evaluated for aortic root dilation using up to four echocardiograms.
O
Outcome
Prevalence of aortic root dilation (AoD) and longitudinal changes in aortic root z-scoressurrogate

Aortic root dilation in young patients with hypermobile Ehlers-Danlos syndrome is relatively uncommon and does not progress over time, suggesting routine echocardiographic screening may be unnecessary.

Cite This Study

Ritter et al. (2017) conducted a cohort in Hypermobile Ehlers-Danlos syndrome (hEDS) (n=325). Hypermobile Ehlers-Danlos syndrome (hEDS) was evaluated on Prevalence of aortic root dilation (z-score ≥ 2.0). Among 325 patients with hypermobile Ehlers-Danlos syndrome, the prevalence of aortic root dilation (z-score ≥2.0) was 14.2%, with no significant progression in z-scores over time.

synapsesocial.com/papers/6aa6002786a9fcb249922d40https://doi.org/10.1002/ajmg.a.38243
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Dilation of the thoracic aorta: medical and surgical management2006 · 58 citations
  2. 2Clinical and Molecular Study of 320 Children With Marfan Syndrome and Related Type I Fibrillinopathies in a Series of 1009 Probands With Pathogenic <i>FBN1</i> Mutations2008 · 181 citations
  3. 32010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the Diagnosis and Management of Patients with Thoracic Aortic Disease2010 · 358 citations
  4. 4Ehlers-Danlos syndromes: Revised nosology, Villefranche, 19971998 · 1,750 citations
  5. 5Aortic root dilatation in Ehlers‐Danlos syndrome types I, II and III A report of five cases1998 · 31 citations