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

• Published in: Monaldi archives for chest disease Vol. 90; no. 2
• Main Authors: Celletti, Claudia, Borsellino, Beatrice, Castori, Marco, Censi, Federica, Calcagnini, Giovanni, Camerota, Filippo, Strano, Stefano
• Format: Journal Article
• Language: English
• Published: Italy PAGEPress Publications 20-05-2020
• Subjects: Adult; autonomic regulation; Baroreflex - physiology; Blood Pressure; Case-Control Studies; Ehlers-Danlos Syndrome - complications; Ehlers-Danlos Syndrome - diagnosis; Ehlers-Danlos Syndrome - physiopathology; Female; Hand Strength - physiology; Heart Rate - physiology; Humans; hypermobile Ehlers-Danlos syndrome; hypermobility spectrum disorder; Joint Instability - physiopathology; Male; Middle Aged; orthostatic intolerance; Phenotype; Postural Orthostatic Tachycardia Syndrome - diagnosis; Postural Orthostatic Tachycardia Syndrome - etiology; Respiration; Retrospective Studies; Sympathetic Nervous System - physiopathology; Tilt-Table Test - methods; Valsalva Maneuver - physiology
• ISSN: 1122-0643; 2532-5264
• DOI: 10.4081/monaldi.2020.1286

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.