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

2020 ◽  
Vol 90 (2) ◽  
Author(s):  
Claudia Celletti ◽  
Beatrice Borsellino ◽  
Marco Castori ◽  
Federica Censi ◽  
Giovanni Calcagnini ◽  
...  
Keyword(s):  
Baroreflex Sensitivity ◽  
Orthostatic Intolerance ◽  
Autonomic Regulation ◽  
Spectrum Disorder ◽  
Postural Tachycardia Syndrome ◽  
Management Approach ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Ehlers Danlos Syndrome ◽  
Head Up Tilt ◽  
Danlos Syndrome

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.

scholarly journals Orthostatic Intolerance and Postural Orthostatic Tachycardia Syndrome in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome, Hypermobility Type: Neurovegetative Dysregulation or Autonomic Failure?

2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Claudia Celletti ◽  
Filippo Camerota ◽  
Marco Castori ◽  
Federica Censi ◽  
Laura Gioffrè ◽  
...  
Keyword(s):  
Spectral Analysis ◽  
Baroreflex Sensitivity ◽  
Orthostatic Intolerance ◽  
Joint Hypermobility ◽  
Tilt Test ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Hypermobility Syndrome ◽  
Joint Hypermobility Syndrome ◽  
Ehlers Danlos Syndrome ◽  
Danlos Syndrome

Background. Joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type (JHS/EDS-HT), is a hereditary connective tissue disorder mainly characterized by generalized joint hypermobility, skin texture abnormalities, and visceral and vascular dysfunctions, also comprising symptoms of autonomic dysfunction. This study aims to further evaluate cardiovascular autonomic involvement in JHS/EDS-HT by a battery of functional tests.Methods. The response to cardiovascular reflex tests comprising deep breathing, Valsalva maneuver, 30/15 ratio, handgrip test, and head-up tilt test was studied in 35 JHS/EDS-HT adults. Heart rate and blood pressure variability was also investigated by spectral analysis in comparison to age and sex healthy matched group.Results. Valsalva ratio was normal in all patients, but 37.2% of them were not able to finish the test. At tilt, 48.6% patients showed postural orthostatic tachycardia, 31.4% orthostatic intolerance, 20% normal results. Only one patient had orthostatic hypotension. Spectral analysis showed significant higher baroreflex sensitivity values at rest compared to controls.Conclusions.This study confirms the abnormal cardiovascular autonomic profile in adults with JHS/EDS-HT and found the higher baroreflex sensitivity as a potential disease marker and clue for future research.

When POTS is the tip of the iceberg: Rare cases of dysautonomia as a possible manifestation of another disorder

Lupus ◽  
2021 ◽  
pp. 096120332098858
Author(s):  
Svetlana Blitshteyn
Keyword(s):  
Clinical Features ◽  
Orthostatic Intolerance ◽  
Medical Condition ◽  
Small Fiber Neuropathy ◽  
Postural Tachycardia Syndrome ◽  
Ehlers Danlos Syndrome ◽  
Postural Tachycardia ◽  
Small Fiber ◽  
Age Range ◽  
Danlos Syndrome

Background Postural tachycardia syndrome (POTS) is a heterogenous disorder of the autonomic nervous system that is commonly associated with small fiber neuropathy, Ehlers-Danlos Syndrome and autoimmune disorders, but association with rare conditions may also occur. Methods Reported here are clinical features, diagnostic tests and treatment outcomes of 6 unique patients who presented with POTS and were subsequently diagnosed with Fabry disease, McArdle disease, Complex V mitochondrial disease, carcinoid tumor, Hodgkin’s lymphoma and chemotherapy-induced neuropathy. Results All patients (age range 15-57 years, 3 females, 3 males) presented with orthostatic intolerance of at least 6 months duration, and all patients had co-morbid small fiber neuropathy. Five patients presented with symptoms of POTS months to years before the underlying or associated medical condition was discovered, and three out of six patients experienced either complete resolution or significant improvement of POTS after treatment of the underlying or associated medical condition. Conclusion In rare cases, POTS can present as a possible manifestation of genetic, neoplastic or neurotoxic disorders. Unusual clinical features that fall outside of the typical spectrum of dysautonomia can point toward the presence of another disorder and help guide further diagnostic investigation.

scholarly journals Postural tachycardia syndrome and other forms of orthostatic intolerance in Ehlers-Danlos syndrome

2018 ◽  
Vol 215 ◽  
pp. 89-96 ◽  
Author(s):  
Maria Roma ◽  
Colleen L. Marden ◽  
Inge De Wandele ◽  
Clair A. Francomano ◽  
Peter C. Rowe
Keyword(s):  
Orthostatic Intolerance ◽  
Postural Tachycardia Syndrome ◽  
Ehlers Danlos Syndrome ◽  
Postural Tachycardia ◽  
Danlos Syndrome

Case 67: Ehlers-Danlos Syndrome with Postural Tachycardia Syndrome and Psychogenic Pseudosyncope

2019 ◽  
pp. 349-352
Author(s):  
Peter Novak
Keyword(s):  
Blood Pressure ◽  
Blood Flow ◽  
Cerebral Blood Flow ◽  
Small Fiber Neuropathy ◽  
Postural Tachycardia Syndrome ◽  
Ehlers Danlos Syndrome ◽  
Postural Tachycardia ◽  
Small Fiber ◽  
Danlos Syndrome

Patient became unresponsive during the first minute of the tilt; she was tachycardic but the blood pressure and cerebral blood flow were stable. Psychogenic pseudosyncope can be superimposed on postural tachycardia syndrome (POTS). Pseudosyncope, small fiber neuropathy, and POTS can be associated with hypermobile Ehlers-Danlos syndrome.

Case 65: Ehlers-Danlos Syndrome with Postural Tachycardia Syndrome, Presyncope, and Anxiety

2019 ◽  
pp. 340-344
Author(s):  
Peter Novak
Keyword(s):  
Tilt Test ◽  
Postural Tachycardia Syndrome ◽  
Ehlers Danlos Syndrome ◽  
Postural Tachycardia ◽  
Danlos Syndrome

The tilt test showed a combination of anxiety, postural tachycardia syndrome (POTS), and presyncope. The POTS in this patient was mild, but anxiety may exacerbate symptoms.

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