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Postural tachycardia syndrome—current experience and concepts
Abstract
Postural tachycardia syndrome (PoTS) is a poorly understood but important cause of orthostatic intolerance resulting from cardiovascular autonomic dysfunction. PoTS is distinct from the syndromes of autonomic failure usually associated with orthostatic hypotension, such as pure autonomic failure and multiple system atrophy. Individuals affected by PoTS are mainly young (aged between 15 years and 40 years) and predominantly female. The symptoms--palpitations, dizziness and occasionally syncope--mainly occur when the patient is standing upright, and are often relieved by sitting or lying flat. Common stimuli in daily life, such as modest exertion, food ingestion and heat, are now recognized to be capable of exacerbating the symptoms. Onset of the syndrome can be linked to infection, trauma, surgery or stress. PoTS can be associated with various other disorders; in particular, joint hypermobility syndrome (also known as Ehlers-Danlos syndrome hypermobility type, formerly termed Ehlers-Danlos syndrome type III). This Review describes the characteristics and neuroepidemiology of PoTS, and outlines possible pathophysiological mechanisms of this syndrome, as well as current and investigational treatments.
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“Differences in connective tissue may be a mediating factor for both these associations, causing pooling of blood in lax peripheral vessels [as in some forms of PoTS (59)] and enhancing sensitivity to tissue stretch and damage.”
From Joint Hypermobility Links Neurodivergence to Dysautonomia and Pain (2022) · cites this paper as [59]
In the citing paper
“often expressed as postural tachycardia syndrome (PoTS), a condition characterised by accelerated heart rate on standing, and other symptoms that overlap phenomenologically with anxiety.26”
From Variant connective tissue (joint hypermobility) and its relevance to depression and anxiety in adolescents: a cohort-based case–control study (2022) · cites this paper as [26]
In the citing paper
“Hypermobility is associated with dysautonomia,47 typically PoTS, symptoms of which have phenomenological overlap with anxiety.26”
From Variant connective tissue (joint hypermobility) and its relevance to depression and anxiety in adolescents: a cohort-based case–control study (2022) · cites this paper as [26]
In the citing paper
“it is known that both the interoceptive sensitivity in hypermobile individuals (and the dysautonomia in PoTS) extends beyond the cardiovascular system (Mathias et al., 2011).”
From Neuroimaging and psychophysiological investigation of the link between anxiety, enhanced affective reactivity and interoception in people with joint hypermobility (2014) · cites this paper as Mathias et al., 2011
In the citing paper
“The onset of POTS may be precipitated by a typical immunological stressor such as viral syndrome (often upper respiratory or gastrointestinal), physical trauma (such as concussion), menarche, pregnancy, or surgery (Boris and Bernadzikowski, 2018; Low et al., 1995; Mathias et al., 2011; Sandroni et al., 1999; Thieben et al., 2007; Watari et al., 2018).”
From Postural orthostatic tachycardia syndrome (POTS): State of the science and clinical care from a 2019 National Institutes of Health Expert Consensus Meeting - Part 1 (2021) · cites this paper as Mathias et al., 2011
In the citing paper
“In patients with joint hypermobility syndrome, a variant of Ehlers-Danlos Syndrome, the onset of POTS may be insidious and the symptoms may develop over years beginning with teenage [27].”
From Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management (2019) · cites this paper as [27]
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Self-citation
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“Symptoms of POTS can be aggravated by factors such as exercise and menstruation [7].”
BackgroundDiseases of the autonomic nervous system
Mathias, C. J., & Low, D. A. (2020) · doi:10.1093/med/9780198746690.003.0603 · Cited by 1
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Coexistence Of Postural Tachycardia Syndrome And Vasovagal Syncope In The Same Patient.
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Self-citationAn Approach to a Patient with Suspected Autonomic and Sleep Dysfunction
Chokroverty, S. (2021) · Springer eBooks · doi:10.1007/978-3-030-62263-3_12
Updates in postural tachycardia syndrome
Moon, J. (2021) · Annals of Clinical Neurophysiology · doi:10.14253/acn.2021.23.1.1
“Postural tachycardia syndrome (POTS) is a common cause of orthostatic intolerance that is characterized by an excessive increase in heart rate (HR) upon standing.(1,2) POTS not only causes the well-recognized symptoms of dizziness and palpitation, but also various other symptoms including headache, fatigue, profuse pe…”
BackgroundThe rheumatological heritable disorders of connective tissue
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Self-citationEhlers Danlos, POTS, and Occult Cerebrospinal Fluid Leak: A Case Report
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“While various stressors (including physical trauma, hormonal changes, and surgery) [17, 18] or genetic predisposition [19] can contribute to POTS, viral illnesses, particularly those leading to persistent symptoms, appear to be a common pathway to the development of this condition [16, 20–22].”
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“Complementary bedside tests, including deep breathing, the Valsalva maneuver, and tilt-table assessments, further illuminate autonomic responses under physiologic challenge [4-6].”
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Self-citation
Metadata
- Authors
- Mathias, C. J., Low, D. A., Iodice, V., Owens, A. P., Kirbis, M., & Grahame, R.
- Affiliations
- St Mary's Hospital (GB)
- University College London (GB)
- Corresponding author
- Mathias, Christopher J.
- Venue
- Nature Reviews Neurology
- Published
- 2011-12-06
- Volume
- 8
- Issue
- 1
- Pages
- 22-34
- ISSN
- 1759-4758 (ISSN-L)
- 1759-4766
- OpenAlex
- W2023404533
- Cited by
- 251
- References
- 114
- Citations by year
- 2026: 6 · 2025: 15 · 2024: 10 · 2023: 7 · 2022: 11 · 2021: 24 · 2020: 16 · 2019: 15 · 2018: 22 · 2017: 20 · 2016: 26 · 2015: 18 · 2014: 27 · 2013: 20 · 2012: 13
- FWCI
- 3.46
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Nature Reviews Neurology (publishedVersion)
- PubMed (publishedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Syncope and Autonomic Disorders
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Heart Rate Variability and Autonomic Control
- Health Sciences › Medicine › Clinical Neurology › Long-Term Effects of COVID-19
- Keywords
- postural orthostatic tachycardia syndrome, orthostatic intolerance, cardiac autonomic dysfunction, pathophysiology, Ehlers-Danlos syndrome hypermobility type, joint hypermobility syndrome, syncope, palpitations, dizziness, autonomic failure, multiple system atrophy, orthostatic hypotension, neuroepidemiology, treatment
- MeSH terms
- Humans, Postural Orthostatic Tachycardia Syndrome
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