@article{Celletti_Borsellino_Castori_Censi_Calcagnini_Camerota_Strano_2020, title={A new insight on postural tachycardia syndrome in 102 adults with hypermobile Ehlers-Danlos Syndrome/hypermobility spectrum disorder }, volume={90}, url={https://www.monaldi-archives.org/macd/article/view/1286}, DOI={10.4081/monaldi.2020.1286}, abstractNote={<p>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.</p>}, number={2}, journal={Monaldi Archives for Chest Disease}, author={Celletti, Claudia and Borsellino, Beatrice and Castori, Marco and Censi, Federica and Calcagnini, Giovanni and Camerota, Filippo and Strano, Stefano}, year={2020}, month={May} }