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 associations, causing pooling of blood in lax peripheral vessels and enhancing sensitivity to tissue stretch and damage.
“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]
Postural tachycardia syndrome (PoTS) is characterised by accelerated heart rate on standing, and other symptoms that overlap phenomenologically with anxiety.
“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]
Hypermobility is associated with dysautonomia, typically PoTS, symptoms of which have phenomenological overlap with anxiety.
“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]
The interoceptive sensitivity in hypermobile individuals (and the dysautonomia in PoTS) extends beyond the cardiovascular system.
“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
The onset of POTS may be precipitated by immunological stressors such as viral syndrome, physical trauma, menarche, pregnancy, or surgery.
“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 joint hypermobility syndrome, a variant of Ehlers-Danlos syndrome, POTS onset may be insidious and symptoms may develop over years starting in the teenage years.
“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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Postural tachycardia syndrome: time frequency mapping
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Treatment of autonomic neuropathy, postural tachycardia and orthostatic syncope with octreotide LAR
Hoeldtke, R. D., Bryner, K. D., Hoeldtke, M. E., & Hobbs, G. R. (2007) · Clinical Autonomic Research · doi:10.1007/s10286-007-0436-x · Cited by 30 · see citing works
Yusuf, S., & Camm, A. J. (2005) · Nature Clinical Practice Cardiovascular Medicine · doi:10.1038/ncpcardio0068 · Cited by 29 · see citing works
Arterial baroreflex control of heart rate during exercise in postural tachycardia syndrome
Masuki, S., Eisenach, J. H., Schrage, W. G., Dietz, N. M., Johnson, C. P., Wilkins, B. W., Dierkhising, R. A., Sandroni, P., Low, P. A., & Joyner, M. J. (2007) · Journal of Applied Physiology · doi:10.1152/japplphysiol.00176.2007 · Cited by 28 · see citing works
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Anxiety and β-adrenergic receptor function in a normal population
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“Connective tissue” between panic disorder and dysautonomia
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Kanjwal, K., Karabin, B. L., Sheikh, M. A., Kanjwal, Y., & Grubb, B. P. (2010) · Journal of Interventional Cardiac Electrophysiology · doi:10.1007/s10840-010-9506-4 · Cited by 15 · see citing works
THE TOLERANCE OF PHYSICAL EXERTION, AS SHOWN BY SOLDIERS SUFFERING FROM SO-CALLED "IRRITABLE HEART."
Lewis, T. A. (1918) · BMJ · doi:10.1136/bmj.1.2987.363 · Cited by 14 · see citing works
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Self-citationFamilial orthostatic tachycardia
Keller, N. R., & Robertson, D. (2006) · Current Opinion in Cardiology · doi:10.1097/01.hco.0000221577.41125.c8 · Cited by 11 · see citing works
Takotsubo cardiomyopathy in a patient with postural tachycardia syndrome
Khurana, R. K. (2007) · Clinical Autonomic Research · doi:10.1007/s10286-007-0444-x · Cited by 8 · see citing works
The Irritable Heart of Soldiers (Soldier's Heart).
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Self-citationNeurovesical dysfunction in postural tachycardia syndrome (POTS)
O’Leary, M. L., Smith, C. P., Erickson, J. R., Eidelman, B. H., & Chancellor, M. B. (2002) · International Urogynecology Journal · doi:10.1007/s001920200030 · Cited by 4 · see citing works
Holmgren, A. (1967) · PubMed Central · Cited by 4 · see citing works
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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 · see citing works
“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 · see citing works
“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 · see citing works
“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 · see citing works
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 · see citing works
The human ventilatory response to stress: rate or depth?
Tipton, M., Harper, A., Paton, J. F. R., & Costello, J. T. (2017) · The Journal of Physiology · doi:10.1113/jp274596 · Cited by 247 · see citing works
“Although equivocal, it appears that the onset of the syndrome can be linked to infection, trauma, surgery or stress (Mathias et al. 2011).”
BackgroundInfluentialPractical Instructions for the 2018 ESC Guidelines for the diagnosis and management of syncope
Brignole, M., Moya‐Mitjans, À., de Lange, F. J., Deharo, J., Elliot, P. M., Fanciulli, A., Fedorowski, A., Furlan, R., Kenny, R. A., Martı́n, A., et al. (2018) · European Heart Journal · doi:10.1093/eurheartj/ehy071 · Cited by 238 · see citing works
“The pathophysiology is debated and likely heterogeneous: deconditioning, immune-mediated processes, excessive venous pooling, and a hyperadrenergic state have been proposed.(28,29)”
BackgroundCastori, M. (2012) · ISRN Dermatology · doi:10.5402/2012/751768 · Cited by 222 · see citing works
Dysautonomia and its underlying mechanisms in the hypermobility type of Ehlers-Danlos syndrome
De Wandele, I., Rombaut, L., Leybaert, L., Van de Borne, P., De Backer, T., Malfait, F., De Paepe, A., & Calders, P. (2014) · Seminars in Arthritis and Rheumatism · doi:10.1016/j.semarthrit.2013.12.006 · Cited by 168 · see citing works
“Further, depression [47], deconditioning [6,44], and paininduced sympathetic arousal have been suggested to contribute to dysautonomia.”
BackgroundCastori, M., Morlino, S., Celletti, C., Ghibellini, G., Bruschini, M., Grammatico, P., Blundo, C., & Camerota, F. (2013) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.36315 · Cited by 159 · see citing works
“As fatigue, shortnessofbreath,and lethargyarecommonamong individuals suffering from postural orthostatic tachycardia syndrome [Mathias et al., 2011], it is likely that cardiovascular dysfunction has a role in fatigue-related disability seen in JHS/EDS-HT.”
BackgroundInfluentialCardiovascular autonomic dysfunction in Ehlers–Danlos syndrome—Hypermobile type
Hakim, A., O'Callaghan, C., De Wandele, I., Stiles, L., Pocinki, A., & Rowe, P. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31543 · Cited by 163 · see citing works
“Symptoms can be highly debilitating in hEDS [Rowe et al., 1999; Hakim and Grahame, 2004; Mathias et al., 2011; De Wandele et al., 2014b].”
BackgroundCaught in the thickness of brain fog: exploring the cognitive symptoms of Chronic Fatigue Syndrome
Ocon, A. J. (2013) · Frontiers in Physiology · doi:10.3389/fphys.2013.00063 · Cited by 162 · see citing works
“Of note, some individuals with CFS and/or POTS also suffer from Ehlers–Danlos Syndrome, a genetic disorder that causes a defect in the synthesis of collagen which affects connective tissue (Rowe et al., 1999; Mathias et al., 2012).”
BackgroundGastrointestinal involvement in the Ehlers–Danlos syndromes
Fikree, A., Chelimsky, G., Collins, H., Kovacic, K., & Aziz, Q. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31546 · Cited by 159 · see citing works
“…that dysautonomia was one mechanism by which this may occur [Gazit et al., 2003; Hakim and Grahame, 2004], and since then it has been shown that Postural Tachycardia Syndrome (PoTS) is associated with GI symptoms such as nausea, reflux, bloating, constipation, and diarrhea [Mathias et al., 2011].”
BackgroundPostural tachycardia syndrome: beyond orthostatic intolerance
Garland, E. M., Celedonio, J. E., & Raj, S. R. (2015) · Current Neurology and Neuroscience Reports · doi:10.1007/s11910-015-0583-8 · Cited by 160 · see citing works
Antiadrenergic autoimmunity in postural tachycardia syndrome
Fedorowski, A., Li, H., Yu, X., Koelsch, K. A., Harris, V. M., Liles, C., Murphy, T. A., Quadri, S. M. S., Scofield, R. H., Sutton, R., et al. (2017) · EP Europace · doi:10.1093/europace/euw154 · Cited by 151 · see citing works
“There has long been speculation that the predilection for POTS in younger females and occasional association with a viral/bacterial infection, vaccination or a stress event would support an autoimmune component in its aetiology.(6,16,17) Recognition that circulating autoantibodies interact with G protein-coupled recep…”
BackgroundSyndromes of orthostatic intolerance: a hidden danger
Fedorowski, A., & Melander, O. (2013) · Journal of Internal Medicine · doi:10.1111/joim.12021 · Cited by 151 · see citing works
Castori, M., Morlino, S., Celletti, C., Celli, M., Morrone, A., Colombi, M., Camerota, F., & Grammatico, P. (2012) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.35483 · Cited by 151 · see citing works
“Finally, a recent review outlines that postural tachycardia syndrome seems to be the most common presentation of disautonomia in JHS/EDS-HT [Mathias et al., 2011].”
BackgroundCardiovascular autonomic dysfunction in post-COVID-19 syndrome: a major health-care burden
Fedorowski, A., Fanciulli, A., Raj, S. R., Sheldon, R. S., Shibao, C. A., & Sutton, R. (2024) · Nature Reviews Cardiology · doi:10.1038/s41569-023-00962-3 · Cited by 128 · see citing works
“These signs include pupillary abnormalities, venous pooling, cold violaceous hands or feet 118 , marbled skin 119 or joint hypermobility 119 (Fig.”
George, S. A., Bivens, T. B., Howden, E. J., Saleem, Y., Galbreath, M. M., Hendrickson, D., Fu, Q., & Levine, B. D. (2016) · Heart Rhythm · doi:10.1016/j.hrthm.2015.12.012 · Cited by 127 · see citing works
De Wandele, I., Calders, P., Peersman, W., Rimbaut, S., De Backer, T., Malfait, F., De Paepe, A., & Rombaut, L. (2014) · Seminars in Arthritis and Rheumatism · doi:10.1016/j.semarthrit.2014.05.013 · Cited by 119 · see citing works
“Similarly, in POTS and CFS, it has also been suggested that deconditioning is probably not the primary cause of OI, but merely a secondary contributing factor or even a consequence [37,38].”
BackgroundWiesmann, T., Castori, M., Malfait, F., & Wulf, H. (2014) · Orphanet Journal of Rare Diseases · doi:10.1186/s13023-014-0109-5 · Cited by 117 · see citing works
“Postural orthostatic tachycardia syndrome (POTS) is sometimes a feature in EDS, especially those with the hypermobile variant (or joint hypermobility syndrome) [20].”
BackgroundCastori, M., Morlino, S., Dordoni, C., Celletti, C., Camerota, F., Ritelli, M., Morrone, A., Venturini, M., Grammatico, P., & Colombi, M. (2012) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.35506 · Cited by 106 · see citing works
Joint Hypermobility Links Neurodivergence to Dysautonomia and Pain
Csecs, J. L. L., Iodice, V., Rae, C. L., Brooke, A., Simmons, R., Quadt, L., Savage, G. K., Dowell, N. G., Prowse, F., Themelis, K., et al. (2022) · Frontiers in Psychiatry · doi:10.3389/fpsyt.2021.786916 · Cited by 98 · see citing works
“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.”
BackgroundSelf-citationMallorquÃ-Bagué, N., Garfinkel, S. N., Engels, M., Eccles, J. A., Pailhez, G., Bulbena, A., & Critchley, H. D. (2014) · Frontiers in Psychology · doi:10.3389/fpsyg.2014.01162 · Cited by 97 · see citing works
Mind-Body Interactions in Anxiety and Somatic Symptoms
Mallorquí-Bagué, N., Bulbena, A., Pailhez, G., Garfinkel, S. N., & Critchley, H. D. (2016) · Harvard Review of Psychiatry · doi:10.1097/hrp.0000000000000085 · Cited by 96 · see citing works
Brain structure and joint hypermobility: Relevance to the expression of psychiatric symptoms
Eccles, J. A., Beacher, F. D. C., Gray, M. A., Jones, C. L., Minati, L., Harrison, N. A., & Critchley, H. D. (2012) · British Journal of Psychiatry · doi:10.1192/bjp.bp.111.092460 · Cited by 96 · see citing works
“Additionally, individuals with hypermobility often exhibit autonomic abnormalities, typically postural tachycardia syndrome, where there is enhanced cardiovascular reactivity and a phenomenological overlap with anxiety disorders.(3) Thus, direct and indirect evidence links hypermobility to anxiety and stress-sensitive…”
Ehlers–Danlos Syndrome—Hypermobility Type: A Much Neglected Multisystemic Disorder
Gazit, Y., Jacob, G., & Grahame, R. (2016) · Rambam Maimonides Medical Journal · doi:10.5041/rmmj.10261 · Cited by 94 · see citing works
Self-citationPostural Orthostatic Tachycardia Syndrome: Prevalence, Pathophysiology, and Management
Zadourian, A., Doherty, T. A., Swiatkiewicz, I., & Taub, P. R. (2018) · Drugs · doi:10.1007/s40265-018-0931-5 · Cited by 90 · see citing works
“Though the mechanism is unclear, it may be attributable to physical deconditioning as the body is weakened and unable to tolerate stress [11, 26, 57].”
BackgroundBeckers, A. B., Keszthelyi, D., Fikree, A., Vork, L., Masclee, A., Farmer, A. D., & Aziz, Q. (2017) · Neurogastroenterology & Motility · doi:10.1111/nmo.13013 · Cited by 90 · see citing works
Adolescent Fatigue, POTS, and Recovery: A Guide for Clinicians
Kizilbash, S. J., Ahrens, S. P., Bruce, B. K., Chelimsky, G., Driscoll, S. W., Harbeck-Weber, C., Lloyd, R. M., Mack, K. J., Nelson, D. E., Ninis, N., et al. (2014) · Current Problems in Pediatric and Adolescent Health Care · doi:10.1016/j.cppeds.2013.12.014 · Cited by 120 · see citing works
Koike, H., Watanabe, H., & Sobue, G. (2012) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp-2012-302833 · Cited by 83 · see citing works
“The onset of symptoms may be acute and can occur following infection, trauma or surgery.(84) 86 Therefore, some cases of POTS may be considered as a milder form of AAG.”
BackgroundNeurological manifestations of Ehlers-Danlos syndrome(s): A review.
Castori, M., & Voermans, N. C. (2014) · Europe PMC (PubMed Central) · Cited by 82 · see citing works
Castori, M., Dordoni, C., Valiante, M., Sperduti, I., Ritelli, M., Morlino, S., Chiarelli, N., Celletti, C., Venturini, M., Camerota, F., et al. (2014) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.36805 · Cited by 81 · see citing works
“A few patients also needed a head-up tilt-test [Mathias et al., 2011].”
BackgroundEvaluation of postural tachycardia syndrome (POTS)
Goodman, B. P. (2018) · Autonomic Neuroscience · doi:10.1016/j.autneu.2018.04.004 · Cited by 77 · see citing works
Owens, A. P., Friston, K. J., Low, D. A., Mathias, C. J., & Critchley, H. (2018) · Autonomic Neuroscience · doi:10.1016/j.autneu.2018.01.001 · Cited by 79 · see citing works
Self-citationOrthostatic intolerance and fatigue in the hypermobility type of Ehlers-Danlos syndrome
De Wandele, I., Rombaut, L., De Backer, T., Peersman, W., Da Silva, H., De Mits, S., De Paepe, A., Calders, P., & Malfait, F. (2016) · Rheumatology · doi:10.1093/rheumatology/kew032 · Cited by 72 · see citing works
Scheper, M. C., Rombaut, L., de Vries, J., Wandele, I. D., van der Esch, M., Visser, B., Malfait, F., Calders, P., & Engelbert, R. (2016) · Disability and Rehabilitation · doi:10.1080/09638288.2016.1196396 · Cited by 71 · see citing works
“Patients diagnosed with Ehlers–Danlos syndrome, are characterized by an altered structural integrity of connective tissue [1,2] resulting in frailty [3] and multi-systemic manifestations like orthostatic intolerance,[4] hyper-elastic skin,[5] organ dysfunction [6] and joint instability.”
The challenges of chronic pain and fatigue
Eccles, J., & Davies, K. A. (2021) · Clinical Medicine · doi:10.7861/clinmed.2020-1009 · Cited by 70 · see citing works
The Postural Tachycardia Syndrome (PoTS)
Mathias, C. J., Owens, A. P., & Iodice, V. (2021) · Springer eBooks · doi:10.1007/978-3-030-62263-3_20 · Cited by 70 · see citing works
Self-citationSerotoninergic dysfunction in the 47, XYY syndrome.
Bioulac, B. H., Bénézech, M., Renaud, B., Noël, B., & Roche, D. (1980) · PubMed · Cited by 70 · see citing works
Postural tachycardia syndrome: current perspectives
Wells, R. M., Spurrier, A. J., Linz, D., Gallagher, C., Mahajan, R., Sanders, P., Page, A. J., & Lau, D. H. (2017) · Vascular Health and Risk Management · doi:10.2147/vhrm.s127393 · Cited by 69 · see citing works
“Concurrent clinical syndromes Irritable bowel syndrome (IBS) Symptoms of IBS, food intolerance and allergic sinusitis, are commonly reported in individuals with POTS.(7) Although rarely performed, plethysmography may demonstrate splanchnic venous pooling in patients with IBS.”
Efficacy of Therapies for Postural Tachycardia Syndrome: A Systematic Review and Meta-analysis
Wells, R., Elliott, A. D., Mahajan, R., Page, A., Iodice, V., Sanders, P., & Lau, D. H. (2018) · Mayo Clinic Proceedings · doi:10.1016/j.mayocp.2018.01.025 · Cited by 67 · see citing works
Self-citationMathias, C. J., Owens, A., Iodice, V., & Hakim, A. (2021) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31951 · Cited by 65 · see citing works
“Within the dysautonomia population, and often applicable to hypermobile EDS (hEDS) and HSD, a large group consist of those with intermittent autonomic malfunction, where between episodes there appear to be no obvious autonomic abnormalities, unlike in patients with autonomic failure (Mathias et al., 2011).”
BackgroundInfluentialSelf-citationConnective tissue, Ehlers–Danlos syndrome(s), and head and cervical pain
Castori, M., Morlino, S., Ghibellini, G., Celletti, C., Camerota, F., & Grammatico, P. (2015) · American Journal of Medical Genetics Part C Seminars in Medical Genetics · doi:10.1002/ajmg.c.31426 · Cited by 67 · see citing works
“In the clinical practice, adhesion to such criteria help in selecting patients for confirmatory laboratory tests, which are now available for all major EDS subtypes except EDS-HT [Mayer et al., 2013].”
BackgroundAssociation of mast-cell-related conditions with hypermobile syndromes: a review of the literature
Monaco, A., Choi, D., Uzun, S., Maitland, A. L., & Riley, B. A. (2022) · Immunologic Research · doi:10.1007/s12026-022-09280-1 · Cited by 64 · see citing works
Fikree, A., Aziz, Q., & Grahame, R. (2013) · Rheumatic Disease Clinics of North America · doi:10.1016/j.rdc.2013.03.003 · Cited by 64 · see citing works
Self-citationA Case of Postural Orthostatic Tachycardia Syndrome Secondary to the Messenger RNA COVID-19 Vaccine
Reddy, S., Reddy, S., & Arora, M. (2021) · Cureus · doi:10.7759/cureus.14837 · Cited by 62 · see citing works
“Some of the common causes of the disorder include the hypothalamus-pituitary-adrenal axis dysfunction, mitochondrial and mast cell disorders, cardiac sympathetic dysautonomia, irregular activation of the renin-angiotensin system, or a baroreflex dysfunction [3,4].”
BackgroundNon-invasive brain stimulation and the autonomic nervous system
Schestatsky, P., Simis, M., Freeman, R. L., Pascual‐Leone, Á., & Fregni, F. (2013) · Clinical Neurophysiology · doi:10.1016/j.clinph.2013.03.020 · Cited by 62 · see citing works
“…2011), parkinsonian symptoms (Iodice et al., 2011), vasovagal syncope (Kanjwal et al., 2011), septic shock (Pancoto et al., 2008), primary palmar hyperhidrosis (Schestatsky et al., 2011), complex regional pain syndrome (Marinus et al., 2011) and postural tachycardia syndrome (Mathias et al., 2011).”
Postural Tachycardia Syndrome in Children and Adolescents
Jarjour, I. T. (2013) · Seminars in Pediatric Neurology · doi:10.1016/j.spen.2013.01.001 · Cited by 60 · see citing works
Simmonds, J. V., Herbland, A., Hakim, A., Ninis, N., Lever, W., Aziz, Q., & Cairns, M. (2019) · Disability and Rehabilitation · doi:10.1080/09638288.2017.1398278 · Cited by 60 · see citing works
“Given the known overlap between JHS/ EDS-HT with fibromyalgia [48], depression and anxiety [49] and Postural Tachycardia Syndrome [17] these forms of exercise may be of significant benefit to this patient group.”
BackgroundInfluentialDemirdas, S., Dulfer, E., Robert, L., Kempers, M., van Beek, D., Micha, D., van Engelen, B. G., Hamel, B., Schalkwijk, J., Loeys, B., et al. (2017) · Clinical Genetics · doi:10.1111/cge.12853 · Cited by 60 · see citing works
“Peripheral blood pooling as part of postural tachycardia syndrome or venous insufficiency related to subcutaneous tissue laxity might play a role (20).”
BackgroundPostural Tachycardia Syndrome (POTS) and the GI Tract: A Primer for the Gastroenterologist
DiBaise, J. K., Harris, L. A., & Goodman, B. (2018) · American Journal of Gastroenterology · doi:10.1038/s41395-018-0215-4 · Cited by 57 · see citing works
“EDS appears to be common in patients with POTS, suggesting that the connective tissue matrix in the gut, which is responsible for the passive mechanical properties of the gut, might be impaired in POTS patients with EDS [31].”
BackgroundKrahe, A. M., Adams, R. D., & Nicholson, L. L. (2018) · Disability and Rehabilitation · doi:10.1080/09638288.2017.1323022 · Cited by 54 · see citing works
“Orthostatic intolerance is a potentially modifiable condition, both pharmacologically and non-pharmacologically [33].”
BackgroundAutonomic Dysfunction in Autism Spectrum Disorder
Owens, A. P., Mathias, C. J., & Iodice, V. (2021) · Frontiers in Integrative Neuroscience · doi:10.3389/fnint.2021.787037 · Cited by 55 · see citing works
Self-citationRisk Factors for Postural Tachycardia Syndrome in Children and Adolescents
Lin, J., Han, Z., Li, X., Ochs, T., Zhao, J., Zhang, X., Yang, J., Liu, P., Xiong, Z., Gai, Y., et al. (2014) · PLoS ONE · doi:10.1371/journal.pone.0113625 · Cited by 54 · see citing works
Mallien, J., Isenmann, S., Mrazek, A., & Haensch, C. (2014) · Frontiers in Neurology · doi:10.3389/fneur.2014.00118 · Cited by 52 · see citing works
Pasquini, M., Celletti, C., Berardelli, I., Roselli, V., Mastroeni, S., Castori, M., Biondi, M., & Camerota, F. (2014) · Rheumatology International · doi:10.1007/s00296-013-2901-2 · Cited by 51 · see citing works
“In particular, this feature, usually in the form of postural tachycardia syndrome in JHS/EDS-HT, often presents with dizziness and light-headedness, palpitations, visual disturbances, clamminess, loss of consciousness, nausea, headache, pain (chest or upper abdomen), shortness of breath, and nonspecific symptoms, incl…”
BackgroundZhao, J., Han, Z., Zhang, X., Du, S., Liu, A. D., Holmberg, L., Li, X., Lin, J., Xiong, Z., Gai, Y., et al. (2015) · BMJ Open · doi:10.1136/bmjopen-2014-007356 · Cited by 48 · see citing works
Pain in Ehlers-Danlos syndromes: manifestations, therapeutic strategies and future perspectives
Castori, M. (2016) · Expert Opinion on Orphan Drugs · doi:10.1080/21678707.2016.1238302 · Cited by 46 · see citing works
“thoracic cage and respiratory muscles) should be first excluded, thoracic pain may be a presenting feature of postural orthostatic tachycardia syndrome, a common complication of EDS [18,98].”
BackgroundTai, F. W. D., Palsson, O. S., Lam, C. Y., Whitehead, W. E., Sperber, A. D., Tornblom, H., Simren, M., & Aziz, I. (2020) · Neurogastroenterology & Motility · doi:10.1111/nmo.13975 · Cited by 44 · see citing works
“20,21 The PHQ-9 categorizes symptoms of depression as none (score: 0-4), mild (5-9), moderate (10-14), moderately severe (1519), and severe (20-27).”
BackgroundDroxidopa in the Treatment of Postural Orthostatic Tachycardia Syndrome
Ruzieh, M., Dasa, O., Pacenta, A., Karabin, B., & Grubb, B. (2017) · American Journal of Therapeutics · doi:10.1097/mjt.0000000000000468 · Cited by 42 · see citing works
Bonamichi-Santos, R., Yoshimi-Kanamori, K., Giavina-Bianchi, P., & Aun, M. V. (2018) · Immunology and Allergy Clinics of North America · doi:10.1016/j.iac.2018.04.004 · Cited by 42 · see citing works
“25 The exact relationship between POTS and hEDS is still unclear.”
Moon, J., Kim, D.-Y., Lee, W.-J., Lee, H. S., Lim, J.-A., Kim, T.-J., Jun, J.-S., Park, B., Byun, J.-I., Sunwoo, J.-S., et al. (2018) · Neurotherapeutics · doi:10.1007/s13311-018-0612-9 · Cited by 40 · see citing works
Coupal, K. E., Heeney, N. D., Hockin, B. C. D., Ronsley, R. J., Armstrong, K. R., Sanatani, S., & Claydon, V. E. (2019) · Frontiers in Neuroscience · doi:10.3389/fnins.2019.01197 · Cited by 42 · see citing works
Postural tachycardia syndrome: multiple symptoms, but easily missed
Kavi, L., Gammage, M. D., Grubb, B. P., & Karabin, B. L. (2012) · British Journal of General Practice · doi:10.3399/bjgp12x648963 · Cited by 40 · see citing works
Boris, J. R., Huang, J., & Bernadzikowski, T. (2019) · Clinical Autonomic Research · doi:10.1007/s10286-019-00622-y · Cited by 40 · see citing works
Li, H., Wang, Y., Liu, P., Chen, Y., Feng, X., Tang, C., Du, J., & Jin, H. (2016) · Pediatric Cardiology · doi:10.1007/s00246-016-1436-1 · Cited by 40 · see citing works
Mast cell activation disease and the modern epidemic of chronic inflammatory disease
Afrin, L. B. (2016) · Translational Research · doi:10.1016/j.trsl.2016.01.003 · Cited by 39 · see citing works
Ehlers-Danlos syndrome, hypermobility type
INSERM (2020) · Definitions · doi:10.32388/0ekfj6 · Cited by 39 · see citing works
Palmer, S., Terry, R. H., Rimes, K. A., Clark, C., Simmonds, J. V., & Horwood, J. P. (2015) · Physiotherapy · doi:10.1016/j.physio.2015.05.001 · Cited by 37 · see citing works
“severity and duration of pain symptoms; the degree of joint hypermobility and 499 instability; psychological dysfunction [15]; and concurrent conditions such as postural 500 tachycardia syndrome [28], dysautonomia [29] and gastro-intestinal dysfunction 501 [30]).”
BackgroundStructural brain abnormalities in postural tachycardia syndrome: A VBM-DARTEL study
Umeda, S., Harrison, N. A., Gray, M. A., Mathias, C. J., & Critchley, H. (2015) · Frontiers in Neuroscience · doi:10.3389/fnins.2015.00034 · Cited by 36 · see citing works
“Eleven patients (9 female; mean age 32.2 years; range 21–50 years) with established diagnoses of PoTS (Freeman et al., 2011; Mathias et al., 2012) and 23 age-matched healthy controls (17 female; mean age 29.0 years; range 18–55 years) participated in this study.”
BackgroundMethodInfluentialSelf-citationHypermobile Ehlers–Danlos syndrome and pregnancy
Karthikeyan, A., & Venkat-Raman, N. (2018) · Obstetric Medicine · doi:10.1177/1753495x18754577 · Cited by 37 · see citing works
Postural Orthostatic Tachycardia Syndrome (POTS): An Update for Clinical Practice.
Sebastian, S. A., Co, E. L., Panthangi, V., Jain, E., Ishak, A., Shah, Y., Vasavada, A., & Padda, I. (2022) · Current problems in cardiology · doi:10.1016/j.cpcardiol.2022.101384 · Cited by 33 · see citing works
Autoimmunoreactive IgGs from patients with postural orthostatic tachycardia syndrome
Wang, X.-L., Chai, Q., Charlesworth, M., Figueroa, J. J., Low, P., Shen, W., & Lee, H.-C. (2012) · PROTEOMICS - Clinical Applications · doi:10.1002/prca.201200049 · Cited by 34 · see citing works
“Postural orthostatic tachycardia syndrome (POTS) is a disorder of unknown etiology but is an important cause of orthostatic intolerance resulting from cardiovascular autonomic dysfunction [5].”
BackgroundClinical challenges in the diagnosis and management of postural tachycardia syndrome
Jones, P., Shaw, B., & Raj, S. (2016) · Practical Neurology · doi:10.1136/practneurol-2016-001405 · Cited by 34 · see citing works
Williams, E. L., Raj, S. R., Schondorf, R., Shen, W.-K., Wieling, W., & Claydon, V. E. (2021) · Autonomic Neuroscience · doi:10.1016/j.autneu.2021.102906 · Cited by 36 · see citing works
“…and Hainsworth, 1998a; Cooper and Hainsworth, 2002), and this is not surprising considering the known higher susceptibility to orthostatic intolerance in females than males (Deveau et al., 2020; Protheroe et al., 2013; Hainsworth and Claydon, 2013; Mathias et al., 2011; Ruwald et al., 2012).”
BackgroundNeurovisceral phenotypes in the expression of psychiatric symptoms
Eccles, J., Owens, A. P., Mathias, C. J., Umeda, S., & Critchley, H. (2015) · Frontiers in Neuroscience · doi:10.3389/fnins.2015.00004 · Cited by 36 · see citing works
“Below we discuss the relevance of three of these “neurovisceral phenotypes” (Joint Hypermobility, Postural Tachycardia Syndrome and Vasovagal Syncope) to psychiatric symptoms.”
InfluentialSelf-citationTransient loss of consciousness and syncope
Bassetti, C. L. (2013) · Handbook of clinical neurology · doi:10.1016/b978-0-7020-4086-3.00013-8 · Cited by 35 · see citing works
Lost among the trees? The autonomic nervous system and paediatrics
Rees, C. (2014) · Archives of Disease in Childhood · doi:10.1136/archdischild-2012-301863 · Cited by 34 · see citing works
Fikree, A., Aktar, R., Morris, J. K., Grahame, R., Knowles, C. H., & Aziz, Q. (2017) · Neurogastroenterology & Motility · doi:10.1111/nmo.12942 · Cited by 33 · see citing works
Self-citationAbnormal gastric myoelectrical activity in postural tachycardia syndrome
Seligman, W. H., Low, D. A., Asahina, M., & Mathias, C. J. (2013) · Clinical Autonomic Research · doi:10.1007/s10286-012-0185-3 · Cited by 33 · see citing works
Self-citationSharp, H. E. C., Critchley, H. D., & Eccles, J. A. (2021) · World Journal of Psychiatry · doi:10.5498/wjp.v11.i10.805 · Cited by 32 · see citing works
Protheroe, C. L., Ravensbergen, H. J. C., Inskip, J. A., & Claydon, V. E. (2013) · Journal of Visualized Experiments · doi:10.3791/4315 · Cited by 33 · see citing works
Joint Hypermobility and Headache: Understanding the Glue That Binds the Two Together – Part 1
Neilson, D., & Martin, V. T. (2014) · Headache The Journal of Head and Face Pain · doi:10.1111/head.12418 · Cited by 31 · see citing works
“Dysautonomia frequently manifests in EDS-HT as variations of POTS.(51) In a study of adults with joint hypermobility, 21 of 27 hypermobile adults demonstrated variations of orthostatic intolerance, compared with 2 of 21 controls.”
ResultKirbiš, M., Grad, A., Meglič, B., & Bajrović, F. F. (2013) · Europe PMC (PubMed Central) · Cited by 31 · see citing works
Fikree, A., Aziz, Q., & Sifrim, D. (2017) · Neurogastroenterology & Motility · doi:10.1111/nmo.13029 · Cited by 30 · see citing works
Is there a relationship between gluten sensitivity and postural tachycardia syndrome?
Penny, H. A., Aziz, I., Ferrar, M., Atkinson, J., Hoggard, N., Hadjivassiliou, M., West, J. N., & Sanders, D. S. (2016) · European Journal of Gastroenterology & Hepatology · doi:10.1097/meg.0000000000000740 · Cited by 30 · see citing works
Ehlers-Danlos Syndrome, Hypermobility Type -- GeneReviews(®)
Ra, P., Adam, M. P., Hh, A., Se, W., Amemiya, A., Ljh, B., Bird, T. D., Ct, F., Mefford, H. C., Rjh, S., et al. (2016) · Europe PMC (PubMed Central) · Cited by 29 · see citing works
Miklovic, T., & Sieg, V. (2019) · StatPearls · Cited by 29 · see citing works
Knuttinen, M.-G., Zurcher, K. S., Khurana, N., Patel, I. J., Foxx–Orenstein, A. E., Harris, L. A., Lawrence, A., Aguilar, F., Sichlau, M. J., Smith, B. H., et al. (2020) · Phlebology The Journal of Venous Disease · doi:10.1177/0268355520947610 · Cited by 28 · see citing works
“Connective tissue abnormalities are also being considered in investigations, as an association with Ehlers Danlos Syndrome has been suggested.(2) In recently presented small case studies, an association between POTS and vascular compression syndromes such as median arcuate ligament syndrome and pelvic compression synd…”
BackgroundZhao, J., Tang, C., Jin, H., & Du, J. (2014) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2014.04.032 · Cited by 27 · see citing works
Postural Heart Rate Changes in Young Patients With Vasovagal Syncope
Medow, M. S., Merchant, S., Suggs, M., Terilli, C., O’Donnell-Smith, B., & Stewart, J. M. (2017) · Pediatrics · doi:10.1542/peds.2016-3189 · Cited by 27 · see citing works
“29, 30 Thus, the excessive tachycardia preceding hypotension and bradycardia is often part of the vasovagal response in young patients, as described here in our cohort of patients with VVS.”
BackgroundPrevalence of joint hypermobility syndromes in pediatric postural orthostatic tachycardia syndrome
Boris, J. R., & Bernadzikowski, T. (2021) · Autonomic Neuroscience · doi:10.1016/j.autneu.2020.102770 · Cited by 27 · see citing works
Pediatric-Onset Postural Orthostatic Tachycardia Syndrome in a Single Tertiary Care Center
Staples, A., Thompson, N. R., & Moodley, M. (2020) · Journal of Child Neurology · doi:10.1177/0883073820916260 · Cited by 26 · see citing works
“Currently, the prevalence of POTS in adult patients is estimated to be 170 in 100 000, but the true prevalence is likely unknown secondary to under-recognition.(1,2) POTS patients are predominantly female at an estimated 3.”
BackgroundInfluentialMaya, T. R., Fettig, V. M., Mehta, L., Gelb, B. D., & Kontorovich, A. (2021) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.62446 · Cited by 26 · see citing works
“, 1999), neuropathy, connective tissue laxity, alpha and beta adrenergic hyper-responsiveness (De Wandele et al., 2014; Mathias et al., 2011), as well as potentially pathogenic adrenergic, muscarinic and other neural autoantibodies (A.”
BackgroundA novel role for the extracellular matrix glycoprotein‐Tenascin‐X in gastric function
Aktar, R., Peiris, M., Fikree, A., Eaton, S., Kritas, S., Kentish, S. J., Araujo, E. J. A., Bacarin, C., Page, A. J., Voermans, N. C., et al. (2019) · The Journal of Physiology · doi:10.1113/jp277195 · Cited by 25 · see citing works
“The most common upper GI symptoms include bloating, reflux, disordered gastric emptying of a meal, nausea and indigestion (Castori et al. 2010; Mathias et al. 2012; Fikree et al. 2014).”
BackgroundAdamec, I., Junaković, A., Skorić, M. K., & Habek, M. (2018) · European Neurology · doi:10.1159/000490628 · Cited by 25 · see citing works
“Namely, OH is a result of inadequate return of blood from the lower extremities and splanchnic circulation due to impaired vasoconstriction [16].”
BackgroundCamerota, F., Castori, M., Celletti, C., Colotto, M., A, S. A., Colella, A., Curione, M., & Danese, C. (2014) · Clinical Rheumatology · doi:10.1007/s10067-014-2618-y · Cited by 25 · see citing works
“Now, it is known that, in association with structural and “primary” functional heart anomalies, JHS/EDS patients may also present postural orthostatic tachycardia syndrome [17].”
BackgroundMethodInfluentialOwens, A. P., Low, D. A., Critchley, H., & Mathias, C. J. (2018) · Autonomic Neuroscience · doi:10.1016/j.autneu.2018.01.004 · Cited by 25 · see citing works
“Symptoms include dizziness and palpitations when upright; some have orthostatic headache, fatigue, bladder and gastrointestinal (GI) symptoms (11).”
BackgroundSelf-citationDeng, X., Zhang, Y., Liao, Y., & Du, J. (2019) · Frontiers in Pediatrics · doi:10.3389/fped.2019.00460 · Cited by 23 · see citing works
- 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 › Surgery › Cardiovascular Syncope and Autonomic Disorders
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Heart Rate Variability and Autonomic Control
- Health Sciences › Medicine › 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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