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

Monaldi Archives for Chest Disease

Beatrice Borsellino¹  Stefano Strano¹  Federica Censi²  Giovanni Calcagnini²  Marco Castori³  Claudia Celletti⁴  Filippo Camerota⁴ 

[1] Department of Heart and Great Vessels “A. Reale”, Sapienza University of Rome;Department of Technology and Health, Italian Institute of Health, Rome;Division of Medical Genetics, Fondazione IRCCS-Casa Sollievo della Sofferenza, San Giovanni Rotondo (FG);Physical Medicine and Rehabilitation, Umberto I University Hospital, Rome;

关键词: autonomic regulation;    hypermobile Ehlers-Danlos syndrome;    hypermobility spectrum disorder;    orthostatic intolerance;   

DOI  :  10.4081/monaldi.2020.1286

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown