A new insight on postural tachycardia syndrome in 102 adults with hypermobile Ehlers-Danlos Syndrome/hypermobility spectrum disorder
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 t...
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| Format: | Article |
| Language: | English |
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PAGEPress Publications
2020-05-01
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| Series: | Monaldi Archives for Chest Disease |
| Subjects: | |
| Online Access: | https://monaldi-archives.org/index.php/macd/article/view/1286 |
| _version_ | 1818667145081389056 |
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| author | Claudia Celletti Beatrice Borsellino Marco Castori Federica Censi Giovanni Calcagnini Filippo Camerota Stefano Strano |
| author_facet | Claudia Celletti Beatrice Borsellino Marco Castori Federica Censi Giovanni Calcagnini Filippo Camerota Stefano Strano |
| author_sort | Claudia Celletti |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-17T06:15:46Z |
| format | Article |
| id | doaj.art-bd623f6bc874433f9cf3a49755c7eb4f |
| institution | Directory Open Access Journal |
| issn | 1122-0643 2532-5264 |
| language | English |
| last_indexed | 2024-12-17T06:15:46Z |
| publishDate | 2020-05-01 |
| publisher | PAGEPress Publications |
| record_format | Article |
| series | Monaldi Archives for Chest Disease |
| spelling | doaj.art-bd623f6bc874433f9cf3a49755c7eb4f2022-12-21T22:00:31ZengPAGEPress PublicationsMonaldi Archives for Chest Disease1122-06432532-52642020-05-0190210.4081/monaldi.2020.1286A new insight on postural tachycardia syndrome in 102 adults with hypermobile Ehlers-Danlos Syndrome/hypermobility spectrum disorderClaudia Celletti0Beatrice Borsellino1Marco Castori2Federica Censi3Giovanni Calcagnini4Filippo Camerota5Stefano Strano6Physical Medicine and Rehabilitation, Umberto I University Hospital, RomeDepartment of Heart and Great Vessels “A. Reale”, Sapienza University of RomeDivision of Medical Genetics, Fondazione IRCCS-Casa Sollievo della Sofferenza, San Giovanni Rotondo (FG)Department of Technology and Health, Italian Institute of Health, RomeDepartment of Technology and Health, Italian Institute of Health, RomePhysical Medicine and Rehabilitation, Umberto I University Hospital, RomeDepartment of Heart and Great Vessels “A. Reale”, Sapienza University of RomeThere 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.https://monaldi-archives.org/index.php/macd/article/view/1286autonomic regulationhypermobile Ehlers-Danlos syndromehypermobility spectrum disorderorthostatic intolerance |
| spellingShingle | Claudia Celletti Beatrice Borsellino Marco Castori Federica Censi Giovanni Calcagnini Filippo Camerota Stefano Strano A new insight on postural tachycardia syndrome in 102 adults with hypermobile Ehlers-Danlos Syndrome/hypermobility spectrum disorder Monaldi Archives for Chest Disease autonomic regulation hypermobile Ehlers-Danlos syndrome hypermobility spectrum disorder orthostatic intolerance |
| title | A new insight on postural tachycardia syndrome in 102 adults with hypermobile Ehlers-Danlos Syndrome/hypermobility spectrum disorder |
| title_full | A new insight on postural tachycardia syndrome in 102 adults with hypermobile Ehlers-Danlos Syndrome/hypermobility spectrum disorder |
| title_fullStr | A new insight on postural tachycardia syndrome in 102 adults with hypermobile Ehlers-Danlos Syndrome/hypermobility spectrum disorder |
| title_full_unstemmed | A new insight on postural tachycardia syndrome in 102 adults with hypermobile Ehlers-Danlos Syndrome/hypermobility spectrum disorder |
| title_short | A new insight on postural tachycardia syndrome in 102 adults with hypermobile Ehlers-Danlos Syndrome/hypermobility spectrum disorder |
| title_sort | new insight on postural tachycardia syndrome in 102 adults with hypermobile ehlers danlos syndrome hypermobility spectrum disorder |
| topic | autonomic regulation hypermobile Ehlers-Danlos syndrome hypermobility spectrum disorder orthostatic intolerance |
| url | https://monaldi-archives.org/index.php/macd/article/view/1286 |
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