Postural orthostatic tachycardia syndrome

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Postural orthostatic tachycardia syndrome (POTS, or postural tachycardia syndrome) is a condition in which a change from the supine position (lying horizontally) to an upright position causes an abnormally large increase in heart rate, called tachycardia. It is a form of orthostatic intolerance (OI), a type of dysautonomia. Other symptoms of an orthostatic nature — occurring in response to upright posture — may accompany the tachycardia.[1] These include headaches, fatigue, sweating, nausea; fainting and dizziness may occur. The autonomic nervous system (ANS) is in charge of heart rate and blood pressure but it has become dysregulated. The female to male ratio of patients with POTS is 4:1.[2][3]

Onset[edit | edit source]

Onset may be linked to infection including viruses like EBV or enteroviruses, trauma, surgery or stress.[4][5]

Signs and symptoms[edit | edit source]

Acrocyanosis in POTS. These images shows the legs of two patients with POTS, immediately after standing up and after standing for several minutes. The reddish-purple discoloration in the legs is very notable, due to poor circulation in the extremities, which returns to normal upon returning to a reclined position.Source: Abou-Diab, J., Moubayed, D., Taddeo, D., Jamoulle, O., & Stheneur, C. (2018). Acrocyanosis Presentation in Postural Orthostatic Tachycardia Syndrome. International Journal Of Clinical Pediatrics, 7(1-2), 13-16. License: CC-BY-NC 4.0

The main symptom of POTS is an abnormal increase in heart rate upon standing. The specific diagnostic criteria for POTS is an increase in heart rate from the lying to upright position of greater than 30 beats per minute, or a heart rate of greater than 120 beats per minute within 10 minutes of standing. Patients with POTS usually present with other symptoms, commonly occurring in the upright position. These include:

Common stimuli in daily life, such as modest exertion, food ingestion and heat, can exacerbate symptoms.[9]

Potential mechanisms[edit | edit source]

Autoimmunity is thought to play a role in many cases of POTS: adrenergic[10], muscarinic[11] and other autoantibodies[12][13][14] have been found. A small study of postural orthostatic tachycardia syndrome in children found that 24.39% of patients had acetylcholine receptor autoantibodies.[15]A small study of adult patients found elevated α1, β1 and β2 adrenergic receptor autoantibodies.[16]

Lax vasculature has been though to play a role in the development of POTS in people with Ehlers-Danlos Syndrome, a connective tissue disorder.[17]

Testing[edit | edit source]

This interactive video explains what you can expect during the tilt table test. By eMedTV

Assessing orthostatic blood pressure can be done in a physician's office by measuring the patient's blood pressure while lying down, sitting, and standing at standardized time increments. Dr. Lucinda Bateman uses a modified orthostatic blood pressure assessment called the NASA 10-minute Lean Test, a variant of a test used by NASA researchers to test for orthostatic intolerance following space flight. The NASA 10-minute Lean Test is less taxing on the patient and can be done in any physician's office. Instructions are available for printout for both healthcare providers and patients.[18]

If the results of the standard orthostatic blood pressure assessment are inconclusive, a tilt table test can be used for diagnosis.[19]

Treatment[edit | edit source]

A 2012 study Diagnosis and management of postural orthostatic tachycardia syndrome: A brief review[20] concluded:

"The pathophysiology of POTS is complex and the result of a number of separate mechanisms producing a common pattern of symptoms. The large number of clinical manifestations that characterize this disorder and the wide range of medications available, plus the clear evidence that certain medications and treatment strategies work in some, but not all POTS patients, demonstrates that POTS is a range of disorders requiring comprehensive investigation and characterisation to guide selection of the most appropriate treatment. The recent consensus statement will help to direct further research into the underlying conditions that lead to POTS."
When the cause of POTS is able to be identified and treated in certain individuals, their POTS symptoms may subside. However, there is currently no cure for POTS on the broader level. The following treatments have been identified to improve symptoms and quality of life:

A small randomized crossover design trial found that patients with postural orthostatic tachychardia improved with Mestinon.[21]

Conditions associated with POTS[edit | edit source]

POTS is not only comorbid with a range of diseases, but its phenotype also resembles that of other disorders (e.g., ME, Ehlers-Danlos Syndrome).

ME/CFS[edit | edit source]

POTS can be a co-morbid condition in ME/CFS patients.[22][23] Estimates on the prevalence of POTS among ME/CFS patients varies widely, from 11% to 70%. In a 2008 study done in the UK by the Northern CFS/ME Clinical Network, using the Fukuda criteria, 27% of the study population had POTS compared with 9% in the control population.[24]

Prevalence of POTS in ME/CFS population samples
Study: number of

ME/CFS patients

%

POTS

Stewart et al. 1999 25 70%
Schondorf et al. 1999 75 40%
Hoad et al. 2008 59 27%
Van Campen et al. 2018 627 25%
Dowsett & Ramsay, 1990[25] 420 21%
Lewis et al. 2013 179 13%
Reynolds et al. 2014 306 11%
Roerink et al. 2017 419 5,7%

A 2011 study of 58 POTS patients by the Vanderbilt Autonomic Dysfunction Center (Vanderbilt University School of Medicine, Nashville, TN, U.S.A.), reported that 64% of also met the Centers for Disease Control & Prevention (CDC) criteria for chronic fatigue syndrome.[26]

The SEID criteria requires either orthostatic intolerance (of which POTS is one type) or cognitive dysfunction for a diagnosis.[27] POTS is also a symptom of the Canadian Consensus Criteria (CCC) which diagnoses ME/CFS,[28] and the International Consensus Criteria (ICC) for diagnosing myalgic encephalomyelitis (ME).[29] However, the diagnosis of POTS alone does not automatically support a ME/CFS diagnosis and cannot be used as a diagnostic biomarker to determine ME/CFS. POTS can occur independent from ME/CFS, and, likewise, ME/CFS can occur without the symptomatology of POTS.

A September 2016 study in the Netherlands by Roerink et al., found that patients with CFS who fulfilled the SEID criteria did not have a prevalence of POTS different from that in the overall ME/CFS population. In adults with ME/CFS, the prevalence of POTS was low, between 6% - 18% (depending on age), was not different from the rate in non-ME/CFS fatigued patients, and was not related to disease severity or treatment outcome.[30]

ME patients with POTS can experience impaired neurocognitive abilities (such as working memory, information processing) under increased orthostatic stress (i.e., standing, tilt table test).[31]

Other conditions associated with POTS[edit | edit source]

Notable research[edit | edit source]

Learn more[edit | edit source]

See also[edit | edit source]

References[edit | edit source]

  1. Mar, Philip L.; Raj, Satish R. (2014). "Neuronal and hormonal perturbations in postural tachycardia syndrome". Frontiers in Physiology. 5: 220. doi:10.3389/fphys.2014.00220. ISSN 1664-042X. PMID 24982638. 
  2. "PoTS - Postural Tachycardia Syndrome". PoTS UK. Retrieved Aug 16, 2018. 
  3. "PoTS - Postural Tachycardia Syndrome - What is POTS?". PoTS UK. Retrieved Aug 16, 2018. 
  4. Grahame, Rodney; Kirbis, Mojca; Andrew P. Owens; Iodice, Valeria; Low, David A.; Mathias, Christopher J. (Jan 2012). "Postural tachycardia syndrome—current experience and concepts". Nature Reviews Neurology. 8 (1): 22–34. doi:10.1038/nrneurol.2011.187. ISSN 1759-4766. 
  5. 5.05.15.2 Gunning, William T.; Kvale, Heather; Kramer, Paula M.; Karabin, Beverly L.; Grubb, Blair P. (Sep 17, 2019). "Postural Orthostatic Tachycardia Syndrome Is Associated With Elevated G‐Protein Coupled Receptor Autoantibodies". Journal of the American Heart Association. 8 (18): e013602. doi:10.1161/JAHA.119.013602. 
  6. "Symptoms of Postural Orthostatic Tachycardia Syndrome (POTS)". Standing Up to POTS. Jul 9, 2018. 
  7. Raj, Satish R (Apr 1, 2006). "The Postural Tachycardia Syndrome (POTS): Pathophysiology, Diagnosis & Management". Indian Pacing and Electrophysiology Journal. 6 (2): 84–99. ISSN 0972-6292. PMC 1501099Freely accessible. PMID 16943900. 
  8. 8.08.18.2 "Dysautonomia International: Postural Orthostatic Tachycardia Syndrome". www.dysautonomiainternational.org. Retrieved Oct 25, 2018. 
  9. 9.09.1 Grahame, Rodney; Kirbis, Mojca; Andrew P. Owens; Iodice, Valeria; Low, David A.; Mathias, Christopher J. (Jan 2012). "Postural tachycardia syndrome—current experience and concepts". Nature Reviews Neurology. 8 (1): 22–34. doi:10.1038/nrneurol.2011.187. ISSN 1759-4766. 
  10. Kem, David C.; Melander, Olle; Sutton, Richard; Scofield, Robert Hal; Quadri, Syed M. S.; Murphy, Taylor A.; Liles, Campbell; Harris, Valerie M.; Koelsch, Kristi A. (Jul 1, 2017). "Antiadrenergic autoimmunity in postural tachycardia syndrome". EP Europace. 19 (7): 1211–1219. doi:10.1093/europace/euw154. ISSN 1099-5129. 
  11. Yu, Xichun; Stavrakis, Stavros; Hill, Michael A.; Huang, Shijun; Reim, Sean; Li, Hongliang; Khan, Muneer; Hamlett, Sean; Cunningham, Madeleine W. (Jan 1, 2012). "Autoantibody activation of beta-adrenergic and muscarinic receptors contributes to an "autoimmune" orthostatic hypotension". Journal of the American Society of Hypertension. 6 (1): 40–47. doi:10.1016/j.jash.2011.10.003. ISSN 1933-1711. 
  12. Thieben, Mark J.; Sandroni, Paola; Sletten, David M.; Benrud-Larson, Lisa M.; Fealey, Robert D.; Vernino, Steven; Low, Phillip A.; Lennon, Vanda A.; Shen, Win-Kuang (Mar 1, 2007). "Postural Orthostatic Tachycardia Syndrome: The Mayo Clinic Experience". Mayo Clinic Proceedings. 82 (3): 308–313. doi:10.4065/82.3.308. ISSN 0025-6196. 
  13. Li Hongliang; Yu Xichun; Liles Campbell; Khan Muneer; Vanderlinde‐Wood Megan; Galloway Allison; Zillner Caitlin; Benbrook Alexandria; Reim Sean. "Autoimmune Basis for Postural Tachycardia Syndrome". Journal of the American Heart Association. 3 (1): e000755. doi:10.1161/JAHA.113.000755. PMC 3959717Freely accessible. PMID 24572257. 
  14. Lennon, Vanda; Low, Phillip; Klein, Christopher; Singer, Wolfgang (Apr 6, 2015). "Autoantibodies in the Postural Tachycardia Syndrome (P1.272)". Neurology. 84 (14 Supplement): P1.272. ISSN 0028-3878. 
  15. Li, Jiawei; Zhang, Qingyou; Liao, Ying; Zhang, Chunyu; Hao, Hongjun; Du, Junbao (Aug 3, 2014). "The Value of Acetylcholine Receptor Antibody in Children with Postural Tachycardia Syndrome". Pediatric Cardiology. 36 (1): 165–170. doi:10.1007/s00246-014-0981-8. ISSN 0172-0643. 
  16. Li, Hongliang; Yu, Xichun; Liles, Campbell; Khan, Muneer; Vanderlinde‐Wood, Megan; Galloway, Allison; Zillner, Caitlin; Benbrook, Alexandria; Reim, Sean (Jan 27, 2014). "Autoimmune Basis for Postural Tachycardia Syndrome". Journal of the American Heart Association. 3 (1). doi:10.1161/jaha.113.000755. ISSN 2047-9980. PMC 3959717Freely accessible. PMID 24572257. 
  17. Hakim, Alan; O'Callaghan, Chris; Wandele, Inge De; Stiles, Lauren; Pocinki, Alan; Rowe, Peter (2017). "Cardiovascular autonomic dysfunction in Ehlers–Danlos syndrome—Hypermobile type". American Journal of Medical Genetics Part C: Seminars in Medical Genetics. 175 (1): 168–174. doi:10.1002/ajmg.c.31543. ISSN 1552-4876. 
  18. Bateman, Lucinda (Sep 27, 2016). "Simple Way to Assess Orthostatic Intolerance - Bateman Horne Center". Bateman Horne Center Newsletter. Retrieved Aug 16, 2018. 
  19. "How Doctors Diagnose & Test for POTS • MyHeart". MyHeart. Retrieved Aug 16, 2018. 
  20. 20.020.1 Abed, Howraa; Ball, Patrick A; Wang, Le-Xin (2012). "Diagnosis and management of postural orthostatic tachycardia syndrome: A brief review". Journal of Geriatric Cardiology : JGC. 9 (1): 61–67. doi:10.3724/SP.J.1263.2012.00061. ISSN 1671-5411. PMID 22783324. 
  21. Raj, S. R. (May 31, 2005). "Acetylcholinesterase Inhibition Improves Tachycardia in Postural Tachycardia Syndrome". Circulation. 111 (21): 2734–2740. doi:10.1161/circulationaha.104.497594. ISSN 0009-7322. 
  22. "POTS in ME/CFS". Freedom From ME - The Optimum Health Clinic on YouTube. Aug 2, 2014. 
  23. "Overlapping Conditions – American ME and CFS Society". ammes.org. Retrieved Aug 12, 2018. 
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  31. Ocon, Anthony J.; Messer, Zachary R.; Medow, Marvin S.; Stewart, Julian M. (Mar 2012). "Increasing orthostatic stress impairs neurocognitive functioning in chronic fatigue syndrome with postural tachycardia syndrome". Clinical Science (London, England : 1979). 122 (5): 227–238. doi:10.1042/CS20110241. ISSN 0143-5221. PMC 3368269Freely accessible. PMID 21919887. 
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  33. Yun, Dong Joo; Choi, Han Na; Oh, Gun-Sei (2013). "A Case of Postural Orthostatic Tachycardia Syndrome Associated with Migraine and Fibromyalgia". The Korean Journal of Pain. 26 (3): 303–306. doi:10.3344/kjp.2013.26.3.303. ISSN 2005-9159. PMID 23862007. 
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Myalgic encephalomyelitis or M.E. has different diagnostic criteria to chronic fatigue syndrome; neurological symptoms are required but fatigue is an optional symptom.<ref name="ICP2011primer">{{Citation

Myalgic encephalomyelitis or chronic fatigue syndrome

Myalgic encephalomyelitis or M.E. has different diagnostic criteria to chronic fatigue syndrome; neurological symptoms are required but fatigue is an optional symptom.<ref name="ICP2011primer">{{Citation

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