A new insight on postural tachycardia syndrome in 102 adults with hypermobile Ehlers-Danlos Syndrome/hypermobility spectrum disorder

  • Claudia Celletti | clacelletti@gmail.com Physical Medicine and Rehabilitation, Umberto I University Hospital, Rome, Italy. https://orcid.org/0000-0001-7154-5882
  • Beatrice Borsellino Department of Heart and Great Vessels “A. Reale”, Sapienza University of Rome, Italy.
  • Marco Castori Division of Medical Genetics, Fondazione IRCCS-Casa Sollievo della Sofferenza, San Giovanni Rotondo (FG), Italy.
  • Federica Censi Department of Technology and Health, Italian Institute of Health, Rome, Italy.
  • Giovanni Calcagnini Department of Technology and Health, Italian Institute of Health, Rome, Italy.
  • Filippo Camerota Physical Medicine and Rehabilitation, Umberto I University Hospital, Rome, Italy.
  • Stefano Strano Department of Heart and Great Vessels “A. Reale”, Sapienza University of Rome, Italy. https://orcid.org/0000-0003-2888-367X

Abstract

There is an association between joint hypermobility, hypermobile Ehlers-Danlos syndrome (hEDS) and different forms of orthostatic intolerance. Objective: to explore autonomic profile in a large cohort of adults with hEDS and hypermobility spectrum disorder (hEDS/HSD) with a multimodal approach. In this observational retrospective study, heart rate, blood pressure and baroreflex sensitivity were estimated in 102 hEDS/HSD subjects during deep breathing, Valsalva maneuver, standing up: 30-15 ratio, Head-Up Tilt and sustained handgrip. Abnormal results and head-up tilt test were common and included postural orthostatic tachycardia syndrome (POTS; 48%), orthostatic intolerance (25.5%) and hypotension (3.9%). Baroreflex sensitivity was significantly different in individuals with POTS compared to the others. This study confirms the high rate and heterogeneity of abnormal autonomic regulation in hEDS/HSD, and suggests the baroreflex sensitivity might distinguish comorbid POTS from other profiles in this subgroup of patients. Abnormal autonomic regulation is common in adults with hEDS/HSD and should be regularly assessed for tailoring the management approach.

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Published
2020-05-20
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Issue
Section
Cardiology - Original Articles
Keywords:
autonomic regulation, hypermobile Ehlers-Danlos syndrome, hypermobility spectrum disorder, orthostatic intolerance
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How to Cite
Celletti, C., Borsellino, B., Castori, M., Censi, F., Calcagnini, G., Camerota, F., & Strano, S. (2020). A new insight on postural tachycardia syndrome in 102 adults with hypermobile Ehlers-Danlos Syndrome/hypermobility spectrum disorder . Monaldi Archives for Chest Disease, 90(2). https://doi.org/10.4081/monaldi.2020.1286