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Evidence

Pilot Study to Evaluate Effect of Antihypertensive Therapy on the Rates of Clinical Events in Patients with Vascular Ehlers-Danlos Syndrome

Date: 07/12/2019

Poster presented at: Society for Vascular Medicine

Location and date: McLean, VA; May 29 – June 1, 2019

Authors: Srinivas Tetali, Tara Grabowsky, Mike Bassett, Ben Kandel , Tyler Folta, William Andrews

Objectives:

  • Despite the severity of vascular Ehlers-Danlos syndrome (vEDS) relative to other subtypes of Ehlers-Danlos syndromes (EDS), United States medical coding groups all EDS under a single code. This has limited studying vEDS outcomes and standard of care.

Conclusions:

  • This study assessed the effect of antihypertensive therapy on the rate of clinical events in patients with vEDS in the United States.
  • This study assessed the effect of antihypertensive therapy on the rate of clinical events in patients with vEDS in the United States.
  • Using big data claims analyses and vEDS-associated event criteria, we identified a presumed vEDS population based on phenotypic presentation.
  • The prevalence of vEDS in our population is slightly higher than expected based on reported prevalence for genotypically-confirmed cases of COL3A1 mutation carriers. Therefore, we may be capturing some additional phenotypically similar subtypes of EDS or those due to mutations other than COL3A1.
  • Demographics of our population were similar to prior reports.
  • The rate of clinical events, including arterial ruptures and intestinal perforations, reflects a high clinical burden for these patients.

Pilot Study to Evaluate Effect of Antihypertensive Therapy on the Rates of Clinical Events in Patients with Vascular Ehlers-Danlos Syndrome

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