The DOI matches its Crossref record; 113 of the 114 references it cites were found in a registry or an index.
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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In the citing paper
“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]
Citations
Cites
Lenders, J. W. M., Eisenhofer, G., Mannelli, M., & Pacák, K. (2005) · The Lancet · doi:10.1016/s0140-6736(05)67139-5 · Cited by 1,541
Freeman, R., Wieling, W., Axelrod, F. B., Benditt, D. G., Benarroch, E., Biaggioni, I., Cheshire, W. P., Chelimsky, T., Cortelli, P., Gibbons, C. H., et al. (2011) · Autonomic Neuroscience · doi:10.1016/j.autneu.2011.02.004 · Cited by 1,243
Self-citationPaulson, O. B., Strandgaard, S., & Edvinsson, L. (1990) · PubMed · Cited by 1,093
Autoantibodies to ganglionic acetylcholine receptors in autoimmune autonomic neuropathies
Vernino, S., Low, P. A., Fealey, R. D., Stewart, J. D., Farrugia, G., & Lennon, V. A. (2000) · New England Journal of Medicine · doi:10.1056/nejm200009213431204 · Cited by 674
Autonomic Failure: A Textbook of Clinical Disorders of the Autonomic Nervous System.
(1984) · Annals of Internal Medicine · doi:10.7326/0003-4819-100-6-930_4 · Cited by 639
Orthostatic Intolerance and Tachycardia Associated with Norepinephrine-Transporter Deficiency
Shannon, J. R., Flattem, N. L., Jordan, J., Jacob, G., Black, B. K., Biaggioni, I., Blakely, R. D., & Robertson, D. (2000) · New England Journal of Medicine · doi:10.1056/nejm200002243420803 · Cited by 594
Idiopathic postural orthostatic tachycardia syndrome: An attenuated form of acute pandysautonomia?
Schondorf, R., & Low, P. A. (1993) · Neurology · doi:10.1212/wnl.43.1_part_1.132 · Cited by 548
Postural orthostatic tachycardia syndrome: the Mayo clinic experience
Thieben, M. J., Sandroni, P., Sletten, D. M., Benrud-Larson, L. M., Fealey, R. D., Vernino, S., Low, P. A., Lennon, V. A., & Shen, W.-K. (2007) · Mayo Clinic Proceedings · doi:10.1016/s0025-6196(11)61027-6 · Cited by 516
The neuropathic postural tachycardia syndrome
Jacob, G., Costa, F., Shannon, J. R., Robertson, R. M., Wathen, M., Stein, M., Biaggioni, I., Ertl, A., Black, B., & Robertson, D. (2000) · New England Journal of Medicine · doi:10.1056/nejm200010053431404 · Cited by 398
Dysautonomia in the joint hypermobility syndrome
Gazit, Y., Nahir, A. M., Grahame, R., & Jacob, G. (2003) · The American Journal of Medicine · doi:10.1016/s0002-9343(03)00235-3 · Cited by 390
Self-citation
Research works printed but not linked: 1
- Kit, L. C. et al. Overactive bladder in patients with postural tachycardia syndrome [abstract 1505]. J. Urol. 185 (Suppl.), e603–e604 (2011).
Cited by
Sorted by citations
A framework for the classification of joint hypermobility and related conditions
Castori, M., Tinkle, B., Levy, H., Grahame, R., Malfait, F., & Hakim, A. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31539 · Cited by 602
“To date, the strongest associations are with anxiety disorders [Sinibaldi et al., 2015], orthostatic tachycardia [Mathias et al., 2011], a variety of functional gastrointestinal disorders [Zarate et al., 2010], and pelvic and bladder dysfunction [de Kort et al., 2003; Veit-Rubin et al., 2016].”
BackgroundSelf-citationTinkle, B., Castori, M., Berglund, B., Cohen, H., Grahame, R., Kazkaz, H., & Levy, H. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31538 · Cited by 499
“Some additional medication options include midodrine, fludrocortisone, and pyridostigmine [Mathias et al., 2011].”
BackgroundSelf-citationPostural tachycardia syndrome: a heterogeneous and multifactorial disorder
Benarroch, E. E. (2012) · Mayo Clinic Proceedings · doi:10.1016/j.mayocp.2012.08.013 · Cited by 370
“Management The management of POTS involves nonpharmacological (Table 3) and pharmacological (Table 4) approaches.(12)”
BackgroundMethodPostural orthostatic tachycardia syndrome: clinical presentation, aetiology and management
Fedorowski, A. (2019) · Journal of Internal Medicine · doi:10.1111/joim.12852 · Cited by 352
Vernino, S., Bourne, K. M., Stiles, L. E., Grubb, B. P., Fedorowski, A., Stewart, J. M., Arnold, A. C., Pace, L. A., Axelsson, J., Boris, J. R., et al. (2021) · Autonomic Neuroscience · doi:10.1016/j.autneu.2021.102828 · Cited by 304
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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