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January 10, 2022Vascular MedicineOpen Access

Cardiovascular manifestations of hypermobile Ehlers–Danlos syndrome and hypermobility spectrum disorders

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Why the study?

The full phenotypic spectrum of cardiovascular complications in hypermobile Ehlers-Danlos syndrome had not been studied following updated nosology and diagnostic criteria.

What is the incidence and spectrum of cardiovascular involvement in patients with hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders?

Population

258 patients diagnosed with hEDS or HSD referred for joint hypermobility

Design

Retrospective review

Authors

EREman RashedTMTania Ruiz MayaJBJennifer Ann Black

Discussion

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Member takes

Overview

Supports echocardiography in symptomatic hEDS/HSD adults; leaves open progression risk and optimal surveillance intervals.

Structured PICO

What is the incidence and spectrum of cardiovascular involvement in patients with hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders?

P
Population
258 patients (> 94% adults) referred to a multidisciplinary clinic for evaluation of joint hypermobility between January 2017 and December 2020 and diagnosed with hypermobile Ehlers-Danlos syndrome (hEDS) or a hypermobility spectrum disorder (HSD)
O
Outcome
Incidence and spectrum of cardiovascular involvementsurrogate

Cardiovascular manifestations in adults with hEDS/HSD are typically mild and include mitral valve prolapse and aortic dilatation, though rare extra-aortic arterial involvement such as cervical artery dissection and spontaneous coronary artery dissection can occur.

Cite This Study

Rashed et al. (2022) studied this question.

synapsesocial.com/papers/6a702244ac440176ef28ca34https://doi.org/10.1177/1358863x211067566
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Also Consider

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

  1. 1Natural history of aortic root dilation through young adulthood in a hypermobile Ehlers–Danlos syndrome cohort2017 · 16 citations
  2. 2Two-dimensional transthoracic echocardiographic normal reference ranges for proximal aorta dimensions: results from the EACVI NORRE study2016 · 113 citations
  3. 3Vascular Complications of Ehlers-Danlos Syndrome: CT Findings2012 · 40 citations
  4. 4Incidence and Outcome of Cerebrovascular Events Related to Cervical Artery Dissection: The Dijon Stroke Registry2013 · 123 citations
  5. 5Internal carotid artery dissection in a community. Rochester, Minnesota, 1987-1992.1993 · 319 citations