Postural orthostatic tachycardia syndrome: the Mayo clinic experience
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More than 75% of POTS patients are female.
“and more than 75% are female.7–9,11–14,16”
POTS symptoms occur with standing, are not tied to particular postures, and include systemic features such as fatigue, sleep disturbance, and migraine.
“The symptoms associated with POTS are those that occur with standing, such as lightheadedness and palpitations; not associated with particular postures, such as bloating, nausea, diarrhea, and abdominal pain; and systemic, such as fatigue, sleep disturbance, and migraine headaches.7”
Most POTS patients first present between ages 15 and 25 years.
“Most patients present with POTS between the ages of 15 and 25 years,7”
Upright plasma NE ≥600 pg/mL is a useful biomarker because it identifies patients who respond better to beta blockers.
“It is a useful biomarker in that it identifies patients who respond better to beta blockers.11”
From Hyperadrenergic Postural Tachycardia Syndrome: Clinical Biomarkers and Response to Guanfacine (2024) · cites this paper as [11]
In POTS patients, the mean age is 30.2 ± 10.3 years and 86% are women.
“The typical demographic is young women (mean age 30.2 Æ 10.3 years, 86% women)”
From Postural Orthostatic Tachycardia Syndrome (2019) · cites this paper as [6]
In POTS patients, the average HR increment from supine to upright is 44.2 ± 13.2 beats/min.
“with an average HR increment of 44.2 Æ 13.2 beats/min from supine to upright (6)”
From Postural Orthostatic Tachycardia Syndrome (2019) · cites this paper as [6]
POTS is frequently preceded by symptoms of a viral illness (42%) or may occur post-operatively (9.5%).
“It is frequently preceded by symptoms of a viral illness (in 42%) or may occur post-operatively (in 9.5%) (6).”
From Postural Orthostatic Tachycardia Syndrome (2019) · cites this paper as [6]
The onset of POTS can be subacute (13.8%), insidious (5.9%), or acute (12.5%).
“The onset of POTS can be subacute (13.8%), insidious (5.9%), or acute (12.5%).”
From Postural Orthostatic Tachycardia Syndrome (2019) · cites this paper as [6]
In one study, more than 50% of POTS patients were noted to have loss of sweat function in distal lower extremities, measured as impaired sweat volume on quantitative sudomotor axon reflex testing or reduced intraepidermal C-fiber density on skin biopsy.
“In one study, >50% of POTS patients (6) were noted to have loss of sweat function in distal lower extremities (32), measured as impaired sweat volume on quantitative sudomotor axon reflex testing (32) or reduced intraepidermal C-fiber density on skin biopsy (33).”
From Postural Orthostatic Tachycardia Syndrome (2019) · cites this paper as [6]
When sodium intake is controlled, approximately 30% of POTS patients have evidence of hypovolemia based on <100 mEq of urinary sodium excretion in 24 h.
“When sodium intake is controlled, urine sodium excretion can be used as a surrogate for hypovolemia, and approximately 30% of POTS patients have evidence of hypovolemia based on <100 mEq of urinary sodium excretion in 24 h (6).”
From Postural Orthostatic Tachycardia Syndrome (2019) · cites this paper as [6]
The hyperadrenergic subtype may correspond to 30% to 60% of POTS patients.
“Another large subgroup within the POTS population is the so-called hyperadrenergic subtype, with features that may correspond to 30% to 60% of POTS patients (6,36,37).”
From Postural Orthostatic Tachycardia Syndrome (2019) · cites this paper as [6]
Hyperadrenergic POTS patients have elevated standing plasma norepinephrine levels (>600 pg/ml), reflecting an exaggerated sympathetic response.
“These patients have elevated standing plasma norepinephrine (NE) levels (>600 pg/ml), reflecting an exaggerated sympathetic response (6,36),”
From Postural Orthostatic Tachycardia Syndrome (2019) · cites this paper as [6]
Women with POTS often report fluctuations in the severity of POTS symptoms throughout their menstrual cycle, with worsening during either the pre-menstrual or early follicular phase when both estrogen and progesterone levels are dropping or low.
“Women with POTS often report fluctuations in the severity of POTS symptoms throughout their menstrual cycle, with worsening during either the pre-menstrual or early follicular phase when both estrogen and progesterone levels are dropping or low (6,9,45,51).”
From Postural Orthostatic Tachycardia Syndrome (2019) · cites this paper as [6]
A frequently described comorbidity in POTS patients is migraine, as well as other chronic headache types.
“A frequently described comorbidity in POTS patients is migraine, as well as other chronic headache types (6,65,72,73).”
From Postural Orthostatic Tachycardia Syndrome (2019) · cites this paper as [6]
Chronic fatigue has been cited in up to 48% of POTS patients, and patients with CFS often have POTS as well.
“Chronic fatigue has been cited in up to 48% of POTS patients (6), and patients with CFS often have POTS as well (81).”
From Postural Orthostatic Tachycardia Syndrome (2019) · cites this paper as [6]
The presentation of POTS appears to follow two patterns: acute onset after a trigger or slowly progressive symptoms over a longer period.
“The presentation seems to have two patterns – acute onset after one of the above triggers or with slowly progressive symptoms over a longer period of time (Thieben et al., 2007).”
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 Thieben et al., 2007
A low titer of antibody to the ganglionic acetylcholine receptor was reported in 15% of adult POTS patients evaluated at the Mayo Clinic between 1993 and 2003.
“A low titer of an antibody to the ganglionic acetylcholine receptor (nAChR) was reported in 15% of adult POTS patients evaluated at the Mayo Clinic between 1993 and 2003 [26].”
From Postural tachycardia syndrome: beyond orthostatic intolerance (2015) · cites this paper as [26]
POTS patients frequently report symptom onset following acute stressors such as pregnancy, trauma, surgery, or viral illness.
“Patients frequently report symptom onset following acute stressors (e.g., pregnancy, a traumatic event, surgery, or a viral illness) [2;26].”
From Postural tachycardia syndrome: beyond orthostatic intolerance (2015) · cites this paper as [26]
Symptoms in POTS can be exacerbated by standing still, exercise, food or alcohol ingestion, or heat exposure.
“Symptoms can be exacerbated by simple activities of daily life like standing still, exercise, food or alcohol ingestion, or heat exposure [26].”
From Postural tachycardia syndrome: beyond orthostatic intolerance (2015) · cites this paper as [26]
Thieben et al. did not observe that POTS subtype affected how well off-label medications worked.
“Although the successful off-label use of different medications might be expected to be influenced by the POTS subtype, Thieben et al. did not find this to be true with a variety of medications [26].”
From Postural tachycardia syndrome: beyond orthostatic intolerance (2015) · cites this paper as [26]
Autonomic problems in POTS may include gastrointestinal complaints, bladder symptoms, and abnormal sudomotor regulation.
“Autonomic problems in POTS might include gastrointestinal complaints, such as abdominal pain, nausea and irritable bowel syndrome, as well as bladder symptoms and abnormal sudomotor regulation [19;26].”
From Postural tachycardia syndrome: beyond orthostatic intolerance (2015) · cites this paper as [26]
The prevalence of anti-gAChR antibodies in this study was higher than that in a previous study.
“The prevalence of anti-gAChR antibodies in this study was higher than that in previous study.17”
From Autoimmune postural orthostatic tachycardia syndrome (2018) · cites this paper as [17]
Approximately 50% of POTS patients evaluated at tertiary care centers exhibit a neuropathic phenotype associated with partial sympathetic denervation, usually due to a distal small fiber neuropathy producing sympathetic denervation of the lower limbs.
“approximately 50% of POTS patients evaluated at tertiary care centers exhibit a neuropathic phenotype associated with partial sympathetic denervation, usually due to a distal small fiber neuropathy producing sympathetic denervation of the lower limbs”
From Postural tachycardia syndrome – Diagnosis, physiology, and prognosis (2018) · cites this paper as Thieben et al., 2007
In an initial study, approximately 15% of POTS patients had low serum titers of ganglionic acetylcholine receptor antibodies.
“An initial study reported a low titer of ganglionic acetylcholine receptor (AChR) antibodies in the serum of approximately 15% of POTS patients (Thieben et al., 2007).”
From Postural tachycardia syndrome – Diagnosis, physiology, and prognosis (2018) · cites this paper as Thieben et al., 2007
A hyperadrenergic state was identified in 29% of POTS cases from a general cohort, though hEDS cases were not specifically identified.
“In support of the hypothesis that this is one mechanism in hEDS, a study by Thieben et al. [2007] identified a hyperadrenergic state in 29% of cases of POTS from a general cohort though cases of hEDS were not specifically identified.”
From Cardiovascular autonomic dysfunction in Ehlers–Danlos syndrome—Hypermobile type (2017) · cites this paper as Thieben et al. [2007]
These findings confirm prior findings, but now establish them in a cohort many times larger than prior studies.
“These confirm prior findings, but now establish them in a cohort that is many times larger than prior studies that have detailed the clinical characteristics of POTS patients 17, 18, 22, 36.”
From The face of postural tachycardia syndrome – insights from a large cross‐sectional online community‐based survey (2019) · cites this paper as [17]
Other studies have reported similar findings.
“Other studies have reported similar findings 17, 18.”
From The face of postural tachycardia syndrome – insights from a large cross‐sectional online community‐based survey (2019) · cites this paper as [17]
A typical POTS patient is a young woman around 25 years old whose first symptoms appear near age 15, although onset can extend into the fifth decade and about one-fifth of patients are men.
“A characteristic patient with POTS is a young woman about 25 years old, reporting first symptoms at age around 15 years, although the range of disease onset may extend until fifth decade of life and one-fifth are men [21,22].”
From Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management (2019) · cites this paper as [21]
Many POTS patients report no triggering event and instead have slowly progressive or stationary symptoms over a long period.
“However, a substantial number of POTS patients do not report any triggering event and experience rather slowly progressing or even stationary symptoms over a long period of time [21].”
From Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management (2019) · cites this paper as [21]
POTS has been shown to have a prevalence of 0.2% in cohort studies and predominately affects females at a 5:1 ratio.
“POTS has been shown to have a prevalence of 0.2% in cohort studies31–33 and predominately affects females at a 5:1 ratio.31,34–36”
From Sinus Tachycardia: a Multidisciplinary Expert Focused Review (2022) · cites this paper as [34]
The vast majority of POTS patients are young, with a mean age of presentation between 15-30.
“The vast majority are young, with a mean age of presentation between 15–3034,36.”
From Sinus Tachycardia: a Multidisciplinary Expert Focused Review (2022) · cites this paper as [34]
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“The symptoms associated with POTS are those that occur with standing, such as lightheadedness and palpitations; not associated with particular postures, such as bloating, nausea, diarrhea, and abdominal pain; and systemic, such as fatigue, sleep disturbance, and migraine headaches.(7) The standing (or orthostatic) hea…”
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Deconditioning in patients with orthostatic intolerance
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Autoimmunity in postural orthostatic tachycardia syndrome: Current understanding
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Self-citationOrthostatic hypotension: A review
Joseph, A., Wanono, R., Flamant, M., & Vidal‐Petiot, E. (2017) · Néphrologie & Thérapeutique · doi:10.1016/j.nephro.2017.01.003 · Cited by 109 · see citing works
Japanese clinical guidelines for juvenile orthostatic dysregulation version 1
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Single centre experience of ivabradine in postural orthostatic tachycardia syndrome
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Migraine and Autonomic Dysfunction: Which Is the Horse and Which Is the Jockey?
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Structural and Functional Small Fiber Abnormalities in the Neuropathic Postural Tachycardia Syndrome
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“In tertiary referral center studies, a neuropathic etiology underlies POTS in 33 to 50% of individuals [6,8].”
BackgroundResultInfluentialPostural Orthostatic Tachycardia Syndrome (POTS): A critical assessment
Olshansky, B., Cannom, D. S., Fedorowski, A., Stewart, J. M., Gibbons, C. H., Sutton, R., Shen, W.-K., Muldowney, J. A. S., Chung, T., Feigofsky, S., et al. (2020) · Progress in Cardiovascular Diseases · doi:10.1016/j.pcad.2020.03.010 · Cited by 102 · see citing works
Self-citationProspective Evaluation of Autonomic Dysfunction in Post-Acute Sequela of COVID-19
Jamal, S. M., Landers, D. B., Hollenberg, S. M., Turi, Z. G., Glotzer, T. V., Tancredi, J., & Parrillo, J. E. (2022) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2022.03.357 · Cited by 100 · see citing works
IVIg for apparently autoimmune small-fiber polyneuropathy: first analysis of efficacy and safety
Liu, X., Treister, R., Lang, M., & Oaklander, A. L. (2018) · Therapeutic Advances in Neurological Disorders · doi:10.1177/1756285617744484 · Cited by 99 · see citing works
Autoimmune postural orthostatic tachycardia syndrome
Watari, M., Nakane, S., Mukaino, A., Nakajima, M., Mori, Y., Maeda, Y., Masuda, T., Takamatsu, K., Kouzaki, Y., Higuchi, O., et al. (2018) · Annals of Clinical and Translational Neurology · doi:10.1002/acn3.524 · Cited by 94 · see citing works
“The prevalence of anti-gAChR antibodies in this study was higher than that in previous study.(17) Anti-gAChR antibodies may impair autonomic ganglionic synaptic transmission, and antibodies that interfere with ganglionic transmission may contribute to dysautonomia in patients with POTS.”
ResultPreparing for the long-haul: Autonomic complications of COVID-19
Larsen, N. W., Stiles, L. E., & Miglis, M. G. (2021) · Autonomic Neuroscience · doi:10.1016/j.autneu.2021.102841 · Cited by 90 · see citing works
Postural Orthostatic Tachycardia Syndrome: Prevalence, Pathophysiology, and Management
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P ostural tachycardia syndrome following human papillomavirus vaccination
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Neurohumoral and haemodynamic profile in postural tachycardia and chronic fatigue syndromes
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Angiotensin II Type 1 Receptor Autoantibodies in Postural Tachycardia Syndrome
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A prospective, 1-year follow-up study of postural tachycardia syndrome
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Small-fiber neuropathy with cardiac denervation in postural tachycardia syndrome
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Long COVID-19 and Postural Orthostatic Tachycardia Syndrome- Is Dysautonomia to Be Blamed?
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Evaluation of postural tachycardia syndrome (POTS)
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The role of autoantibodies in the syndromes of orthostatic intolerance: a systematic review
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Autonomic dysfunction in fibromyalgia syndrome: Postural orthostatic tachycardia
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A review of postural orthostatic tachycardia syndrome
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Distal sudomotor findings in postural tachycardia syndrome
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Twenty-four-hour urinary sodium excretion and postural orthostatic tachycardia syndrome
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Other autonomic neuropathies associated with ganglionic antibody
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A Case of Postural Orthostatic Tachycardia Syndrome Secondary to the Messenger RNA COVID-19 Vaccine
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Cardiac Arrhythmias in Post-COVID Syndrome: Prevalence, Pathology, Diagnosis, and Treatment
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Sinus Tachycardia: a Multidisciplinary Expert Focused Review
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A Survey-Based Analysis of Symptoms in Patients with Postural Orthostatic Tachycardia Syndrome
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- Authors
- 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.
- Affiliations
- Mayo Clinic (US)
- Corresponding author
- Low, Phillip A.
- Venue
- Mayo Clinic Proceedings
- Publisher
- Elsevier BV
- Published
- 2007-03
- Volume
- 82
- Issue
- 3
- Pages
- 308-313
- ISSN
- 0025-6196 (ISSN-L)
- OpenAlex
- W2113993848
- Cited by
- 516
- References
- 23
- Citations by year
- 2026: 16 · 2025: 28 · 2024: 31 · 2023: 33 · 2022: 36 · 2021: 34 · 2020: 41 · 2019: 29 · 2018: 38 · 2017: 34 · 2016: 26 · 2015: 27 · 2014: 29 · 2013: 30 · 2012: 17
- FWCI
- 16.13
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Mayo Clinic Proceedings (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institute of Diabetes and Digestive and Kidney Diseases (DK068055, P01DK068055)
- National Institute of Neurological Disorders and Stroke (NS3 2352, P01NS044233, P50NS032352, NS-44233, NS4 3364, S11NS043364)
- National Center for Research Resources (M01-RR00858)
- 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 › Psychosomatic Disorders and Their Treatments
- Keywords
- postural orthostatic tachycardia syndrome, autonomic dysfunction, autoimmune etiology, hyperadrenergic state, norepinephrine, hypovolemia, urinary sodium, quantitative sudomotor axon reflex test, thermoregulatory sweat test, pathogenesis
- MeSH terms
- Adult, Autonomic Nervous System Diseases, Female, Humans, Hypotension, Orthostatic, Male, Minnesota, Retrospective Studies, Syndrome, Chi-Square Distribution
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