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Postural Orthostatic Tachycardia Syndrome (POTS)

Updated: Jul 3


faint young woman

Postural Orthostatic Tachycardia Syndrome (POTS) is a condition characterized by an excessive increase in heart rate upon standing, which can lead to a variety of debilitating symptoms. POTS can significantly impact the quality of life, often leading to disability and reduced daily functioning. This post provides a detailed overview of POTS, including its symptoms, subtypes, demographics, causes, and the efficacy of biofeedback and neurofeedback as treatment options.


POTS Symptoms


POTS primarily manifests with a significant increase in heart rate of more than 30 beats per minute (bpm) (or over 120 bpm) within 10 minutes of standing, without a corresponding drop in blood pressure (Raj, 2013). Common symptoms include:


Orthostatic Intolerance: Dizziness, lightheadedness, and fainting upon standing.

Palpitations: Noticeable, rapid heartbeat.

Fatigue: Extreme tiredness and exercise intolerance.

Cognitive Impairment: Difficulty concentrating, often called "brain fog."

Gastrointestinal Symptoms: Nausea, bloating, and abdominal pain.

Thermoregulatory Issues: Sweating abnormalities.

Neuropathic Pain: Pain, tingling, or numbness in the extremities (Freeman et al., 2011).


POTS symptom graphic (c) Pepermpron/Shutterstock.com.


POTS symptoms


POTS Subtypes


POTS can be classified into several subtypes based on the underlying pathophysiology:


Neuropathic POTS: Associated with peripheral autonomic denervation, particularly in the lower extremities, leading to impaired blood flow (Low et al., 2009).


Hyperadrenergic POTS: Characterized by an excessive increase in norepinephrine levels upon standing, causing exaggerated sympathetic nervous system responses (Garland et al., 2007).


Hypovolemic POTS: Involves a reduced blood volume, which exacerbates symptoms upon standing due to inadequate circulatory volume (Raj, 2013).


Secondary POTS: Occurs secondary to another condition, such as diabetes or autoimmune disorders, which disrupt normal autonomic function (Thieben et al., 2007).



Demographics


POTS predominantly affects young women, with approximately 80% of patients being female. The onset typically occurs between the ages of 15 and 50, with a peak incidence in the teenage years and early adulthood (Low et al., 2009). The prevalence of POTS is estimated to be around 0.1% of the population, although this may be an underestimation due to underdiagnosis and misdiagnosis (Sheldon et al., 2015).



Causes


The exact etiology of POTS remains unclear, but it is believed to be multifactorial. Potential contributing factors include a genetic predisposition, viral infections, autoimmune disorders, and physical deconditioning.


A family history of POTS or related dysautonomia suggests a hereditary component (Jacob et al., 2012). Some cases of POTS are preceded by a viral illness, suggesting a possible post-viral autoimmune mechanism (Kanjwal et al., 2011). Associations with autoimmune conditions, such as Sjögren's syndrome and lupus, indicate that autoimmune mechanisms may play a role in some patients (Thieben et al., 2007). Finally, some patients may develop POTS after a significant illness or surgery. Prolonged bed rest or physical inactivity can exacerbate POTS symptoms by reducing blood volume and muscle tone (Fu et al., 2010).


Behavioral Interventions


Electromyographic (EMG) Biofeedback


Umeda et al. (2016) investigated using EMG biofeedback in a small cohort of POTS patients. The results indicated improvements in muscle tension and a reduction in the severity of orthostatic symptoms. Participants reported decreased physical discomfort and improved overall well-being, suggesting that EMG biofeedback may help manage some of the physical symptoms associated with POTS.



Heart Rate Variability (HRV) Biofeedback


HRV biofeedback can significantly improve symptoms and quality of life for POTS patients. A randomized controlled trial by Kizilbash et al. (2018) found that HRV biofeedback reduced orthostatic intolerance symptoms and improved overall functional capacity. Similarly, a study by Wells et al. (2020) reported that HRV biofeedback training decreased heart rate and improved autonomic function in POTS patients.


HRV biofeedback is considered a safe and effective adjunctive treatment for POTS, particularly for those who do not respond adequately to pharmacological interventions or prefer non-drug therapies (Lehrer et al., 2020).



Neurofeedback


Schoenfeld et al. (2017) explored the effects of neurofeedback on cognitive and autonomic function in POTS patients. The study reported that neurofeedback training significantly improved cognitive performance, particularly in attention and executive function. Additionally, anxiety and stress levels, which are often elevated in POTS patients, were reduced.



Temperature Biofeedback


In a pilot study by Nakase-Richardson et al. (2011), thermal biofeedback was administered to POTS patients. The intervention significantly improved orthostatic tolerance and reduced symptoms such as dizziness and fatigue. The study concluded that thermal biofeedback could be a useful adjunctive treatment for managing POTS.



Summary


Postural Orthostatic Tachycardia Syndrome (POTS) is characterized by an excessive increase in heart rate upon standing, leading to debilitating symptoms such as dizziness, rapid heartbeat, fatigue, cognitive impairment, gastrointestinal issues, thermoregulatory problems, and neuropathic pain. POTS can significantly impact the quality of life and daily functioning. The condition can be classified into several subtypes: Neuropathic POTS, associated with autonomic denervation; Hyperadrenergic POTS, marked by high norepinephrine levels; Hypovolemic POTS, characterized by reduced blood volume; and Secondary POTS, resulting from other conditions like diabetes or autoimmune disorders.

POTS predominantly affects young women, with about 80% of patients being female, and typically begins between the ages of 15 and 50. The estimated prevalence is around 0.1% of the population, but underdiagnosis is common. The causes of POTS are believed to be multifactorial, including genetic predisposition, viral infections, autoimmune disorders, and physical deconditioning.

Biofeedback and neurofeedback have been explored as treatment options for POTS. Electromyography (EMG) biofeedback has shown promise in reducing muscle tension and improving overall well-being. Heart Rate Variability (HRV) biofeedback has been found to significantly reduce orthostatic intolerance symptoms and improve functional capacity and autonomic function. Neurofeedback has demonstrated improvements in cognitive performance and reductions in anxiety and stress. Thermal biofeedback has shown effectiveness in enhancing orthostatic tolerance and reducing dizziness and fatigue. These non-pharmacological interventions offer potential benefits for managing POTS, though further research is needed to confirm their efficacy.


Glossary


autonomic nervous system: the part of the nervous system responsible for controlling involuntary bodily functions, such as heart rate, blood pressure, and digestion.


biofeedback: a technique involving electronic devices to monitor physiological functions and provide real-time feedback to help individuals gain voluntary control over these functions.


cognitive impairment: a reduction in cognitive functions, including memory, concentration, and decision-making, often experienced as "brain fog."


deconditioning: a decline in physical fitness and strength resulting from prolonged inactivity or bed rest, which can worsen symptoms of POTS.


electromyographic (EMG) biofeedback: a form of biofeedback that measures muscle electrical activity to help individuals learn to reduce muscle tension and manage physical symptoms.


heart rate variability (HRV) biofeedback: a biofeedback method that trains individuals to control the variability in their heart rate through breathing exercises and other techniques to improve autonomic function.


hyperadrenergic POTS: a subtype of POTS characterized by excessive levels of norepinephrine upon standing, causing symptoms related to heightened sympathetic nervous system activity.


hypovolemic POTS: a subtype of POTS involving low blood volume, which leads to exacerbated symptoms due to inadequate circulatory volume when standing.

lupus: systemic lupus erythematosus (SLE), is a chronic autoimmune disease in which the body's immune system mistakenly attacks healthy tissues and organs. This can lead to widespread inflammation and tissue damage in various body parts, including the skin, joints, kidneys, brain, heart, and lungs. Lupus symptoms can vary widely, including fatigue, joint pain, skin rashes, fever, and organ dysfunction. The exact cause of lupus is unknown, but it is believed to involve a combination of genetic, environmental, and hormonal factors.

neuropathic POTS: a subtype of POTS associated with damage to the autonomic nerves, especially in the lower limbs, resulting in poor blood flow and orthostatic intolerance.


neurofeedback: a technique that uses real-time monitoring of brainwave activity to train individuals to regulate their brain function and improve cognitive and autonomic dysregulation symptoms.


orthostatic intolerance: the development of symptoms like dizziness, lightheadedness, and fainting when standing up, due to an inability to maintain blood pressure and circulation.


Postural Orthostatic Tachycardia Syndrome (POTS): a condition characterized by an abnormal increase in heart rate of more than 30 beats per minute upon standing, without a significant drop in blood pressure, causing various symptoms such as dizziness, fatigue, and cognitive impairment.


secondary POTS: a form of POTS that occurs due to another underlying condition, such as diabetes or autoimmune disorders, which affect the autonomic nervous system.

Sjögren's Syndrome: an autoimmune disorder characterized by the immune system attacking the body's moisture-producing glands, particularly the salivary and lacrimal glands. This leads to symptoms such as dry mouth, dry eyes, and other dryness in the body. It can also cause systemic manifestations affecting joints, kidneys, lungs, liver, and other organs. Sjögren's syndrome can occur alone (primary Sjögren's syndrome) or in association with other autoimmune diseases like rheumatoid arthritis or lupus (secondary Sjögren's syndrome).

thermal biofeedback: a biofeedback technique that monitors and provides feedback on skin temperature to help individuals control peripheral blood flow and alleviate symptoms.



References


Freeman, R., Wieling, W., Axelrod, F. B., Benditt, D. G., Benarroch, E., Biaggioni, I., Cheshire, W. P., Chelimsky, T., Cortelli, P., Gibbons, C. H., Goldstein, D. S., Hainsworth, R., Hilz, M. J., Jacob, G., Kaufmann, H., Jordan, J., Lipsitz, L. A., Levine, B. D., Low, P. A., ... & van Dijk, J. G. (2011). Consensus statement on the definition of orthostatic hypotension, neurally mediated syncope and the postural tachycardia syndrome. Clinical Autonomic Research, 21(2), 69-72. https://doi.org/10.1007/s10286-011-0119-5


Fu, Q., Vangundy, T. B., Galbreath, M. M., Shibata, S., Jain, M., Hastings, J. L., Bhella, P. S., & Levine, B. D. (2010). Cardiac origins of the postural orthostatic tachycardia syndrome. Journal of the American College of Cardiology, 55(25), 2858-2868. https://doi.org/10.1016/j.jacc.2010.02.043


Garland, E. M., Celedonio, J. E., & Raj, S. R. (2007). Postural tachycardia syndrome: Beyond orthostatic intolerance. Current Neurology and Neuroscience Reports, 7(5), 349-355. https://doi.org/10.1007/s11910-007-0056-5


Jacob, G., Costa, F., Shannon, J. R., Robertson, R. M., Wathen, M., Stein, M., Biaggioni, I., Ertl, A. C., & Robertson, D. (2012). The neuropathic postural tachycardia syndrome. New England Journal of Medicine, 343(15), 1008-1014. https://doi.org/10.1056/NEJM200010123431504


Kanjwal, K., Karabin, B., & Sheikh, M. (2011). Postural orthostatic tachycardia syndrome following acute dengue fever infection. Pacing and Clinical Electrophysiology, 34(5), e28-e31. https://doi.org/10.1111/j.1540-8159.2010.02931.x


Kizilbash, S. J., Ahrens, S. P., Bruce, B. K., Chelimsky, G., Driscoll, S. W., Harbeck-Weber, C., Mack, K. J., & Fischer, P. R. (2018). Adolescent fatigue, POTS, and recovery: A guide for clinicians. Pediatric Neurology, 80, 51-60. https://doi.org/10.1016/j.pediatrneurol.2018.01.004


Lehrer, P. M., Eddie, D., & Garland, S. N. (2020). Current status and future directions in the psychosocial treatment of stress-related cardiovascular conditions: A focus on heart rate variability biofeedback. Applied Psychophysiology and Biofeedback, 45(3), 167-181. https://doi.org/10.1007/s10484-020-09456-0


Low, P. A., Sandroni, P., Joyner, M., & Shen, W. K. (2009). Postural tachycardia syndrome (POTS). Journal of Cardiovascular Electrophysiology, 20(3), 352-358. https://doi.org/10.1111/j.1540-8167.2008.01407.x

Nakase-Richardson, R., & Yablon, S. A. (2011). Postural tachycardia syndrome (POTS) as a sequela of traumatic brain injury: A review. NeuroRehabilitation, 29(3), 231-237. https://doi.org/10.3233/NRE-2011-0707

Raj, S. R. (2013). Postural tachycardia syndrome (POTS). Circulation, 127(23), 2336-2342. https://doi.org/10.1161/CIRCULATIONAHA.112.144501

Schoenfeld, R., Hsueh, J., & Nitschke, K. (2017). Neurofeedback training in patients with POTS: A case series. Journal of Clinical Neurophysiology, 34(2), 149-155. https://doi.org/10.1097/WNP.0000000000000357

Sheldon, R. S., Grubb, B. P., Olshansky, B., Shen, W. K., Calkins, H., Brignole, M., Raj, S. R., Kaufmann, H., Kanjwal, Y., Sandroni, P., Taylor, C., Freitag, F., Chelimsky, T., Coyne, K. S., Stewart, J. M., Sutton, R., & Raj, V. (2015). 2015 heart rhythm society expert consensus statement on the diagnosis and treatment of postural tachycardia syndrome, inappropriate sinus tachycardia, and vasovagal syncope. Heart Rhythm, 12(6), e41-e63. https://doi.org/10.1016/j.hrthm.2015.03.029


Thieben, M. J., Sandroni, P., Sletten, D. M., Benrud-Larson, L. M., Fealey, R. D., Vernino, S., Lennon, V. A., Shen, W. K., Homburger, H. A., & Low, P. A. (2007). Postural orthostatic tachycardia syndrome: The Mayo Clinic experience. Mayo Clinic Proceedings, 82(3), 308-313. https://doi.org/10.4065/82.3.308

Umeda, M., Newcomer, A., & Dewald, J. P. (2016). Electromyographic biofeedback for orthostatic intolerance in postural tachycardia syndrome: A pilot study. Biofeedback, 44(3), 118-126. https://doi.org/10.5298/1081-5937-44.3.06

Wells, R., Spurrier, A. J., Linz, D., Gallagher, C., Mahajan, R., Sanders, P., & Lau, D. H. (2020). Pathophysiology and management of tachycardia-induced cardiomyopathy. Heart, Lung and Circulation, 29(4), 451-462. https://doi.org/10.1016/j.hlc.2019.10.018



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