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Cited by 38Cardiovascular Syncope and Autonomic DisordersHeart Rate Variability and Autonomic ControlFibromyalgia and Chronic Fatigue Syndrome Research

Postural tachycardia in hypermobile Ehlers-Danlos syndrome: A distinct subtype?

Miglis, Mitchell G., Schultz, Brittany, Muppidi, Srikanth·2017·Autonomic Neuroscience
DOI 10.1016/j.autneu.2017.10.001
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Abstract

Introduction It is not clear if patients with postural tachycardia syndrome (POTS) and Ehlers-Danlos syndrome (hEDS) differ from patients with POTS due to other etiologies. We compared the results of autonomic testing and healthcare utilization in POTS patients with and without hEDS.Methods Patients with POTS+hEDS (n=20) and POTS controls without hypermobility (n=20) were included in the study. All patients underwent autonomic testing, and the electronic medical records were reviewed to determine the number and types of medications patients were taking, as well as the number of outpatient, emergency department, and inpatient visits over the prior year.Results Patients with hEDS had twice as many outpatient visits (21 v. 10, p=0.012), were taking more prescription medications (8 vs. 5.5, p=0.030), and were more likely to see a pain physician (70% vs 25%, p=0.005). Autonomic testing demonstrated a slight reduction in heart rate variability and slightly lower blood pressures on tilt table testing in hEDS patients, however for most patients these variables remained within the range of normal. Orthostatic tachycardia on tilt table testing was greater in POTS controls (46bpm vs 39bpm, p=0.018). Abnormal QSweat responses were common in both groups (38% of POTS+hEDS and 36% of POTS controls).Conclusions While autonomic testing results were not significantly different between groups, patients with POTS+hEDS took more medications and had greater markers of healthcare utilization, with chronic pain likely playing a prominent role.

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  • There is some evidence that the copresence of HSD/hEDS and PoTS may represent a more severe disease phenotype.

    “There is some evidence that the copresence of the two disorders may represent a more severe disease phenotype. 36 , 43 , 70 , 71”

    From Hypermobile Ehlers–Danlos syndrome and disorders of the gastrointestinal tract: What the gastroenterologist needs to know (2022) · cites this paper as [70]

  • Many conditions such as mast cell activation disorders, Ehlers-Danlos syndrome, deconditioning, and genetic mutations of norepinephrine transporters coexist with POTS, and their downstream effects funnel into the three principal pathophysiologic mechanisms.

    “While many conditions, such as mast cell activation disorders (9), Ehlers-Danlos syndrome (10, 11), deconditioning (12), and genetic mutations of norepinephrine transporters (13), coexist with POTS, their downstream effects also funnel into these three principal pathophysiologic mechanisms.”

    From Postural orthostatic tachycardia syndrome: mechanisms and new therapies (2020) · cites this paper as [10]

  • Antidepressant use is likely high in POTS patients, given the high incidence of polypharmacy and the perception of increased depression in this population.

    “Given the high incidence of polypharmacy in POTS patients (10), coupled with the perception of increased depression in POTS patients (74), antidepressant use is likely high in this patient population.”

    From Postural orthostatic tachycardia syndrome: mechanisms and new therapies (2020) · cites this paper as [10]

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Authors
Miglis, M. G., Schultz, B., & Muppidi, S.
Affiliations
Stanford Medicine (US)
Corresponding author
Miglis, Mitchell G.
ORCID
Venue
Autonomic Neuroscience
Publisher
Elsevier BV
Published
2017-12
Volume
208
Pages
146-149
ISSN
1566-0702 (ISSN-L)
DOI
10.1016/j.autneu.2017.10.001
OpenAlex
W2763832722
Cited by
38
References
24
Citations by year
2026: 2 · 2025: 1 · 2024: 3 · 2023: 1 · 2022: 2 · 2021: 23 · 2020: 2 · 2019: 2 · 2018: 2
FWCI
1.69
Type
journal article
Language
en
Open access
closed
Licence
Locations
  • Autonomic Neuroscience (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Surgery › Cardiovascular Syncope and Autonomic Disorders
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Heart Rate Variability and Autonomic Control
  • Health Sciences › Medicine › Psychiatry and Mental health › Fibromyalgia and Chronic Fatigue Syndrome Research
Keywords
postural orthostatic tachycardia syndrome, hypermobile Ehlers-Danlos syndrome, autonomic dysfunction, heart rate variability, tilt table test, orthostatic tachycardia, health care utilization, prescription drug use, chronic pain
MeSH terms
Adult, Blood Pressure, Ehlers-Danlos Syndrome, Female, Heart Rate, Humans, Male, Patient Acceptance of Health Care, Retrospective Studies, Comorbidity, Tilt-Table Test, Postural Orthostatic Tachycardia Syndrome, Young Adult, Chronic Pain

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