Postural Orthostatic Tachycardia Syndrome (POTS)

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This clinical tool supports primary care clinicians in recognizing, assessing, diagnosing and managing Postural Orthostatic Tachycardia Syndrome (POTS). POTS is a form of dysautonomia characterized by excessive orthostatic tachycardia and orthostatic intolerance, resulting in chronic and sometimes debilitating symptoms that may significantly impact quality of life. While there is no cure, reduced symptom severity and improved quality of life can often be achieved through accurate diagnosis, detailed assessment and targeted management.1,2

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Clinical presentation

Presentation of POTS symptoms can vary between patients; however, symptoms of orthostatic intolerance must be dominant in the clinical presentation.1

Practice point: It is more important to understand symptom presentation and behaviour (e.g., if some symptoms are improved upon recumbency) rather than requiring a specific cluster or number of symptoms.1,2 

Common symptoms1-3
Symptoms improved with recumbence (orthostatic intolerance symptoms):
  • Light-headedness/dizziness 
  • Palpitation (“heart racing”)  
  • Tremulousness 
  • Atypical chest discomfort/dyspnea  
  • Shortness of breath 
  • Peripheral acrocyanosis (“POTS feet”) 
Symptoms that may or may not be associated with changes in position include:
  • Chronic fatigue 
  • Chronic pain 
  • Exercise intolerance 
  • Heat intolerance 
  • Sleep disturbances 
  • Cognitive dysfunction (e.g., brain fog) 
  • Headaches 
  • Frequent nausea 
  • Constipation, mild diarrhea, bloating, unspecific abdominal pain (e.g., IBS, GERD, early satiety) 

Assessment New

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Carry out a detailed history, a physical examination and a 12-lead ECG during the initial assessment for POTS. This approach is often sufficient to diagnose and begin treatment; however, given the heterogeneity of POTS, an individualized approach is warranted.1  

Clinical history

Once a list of orthostatic and commonly associated symptoms has been identified, proceed with inquiring about the following:1,2 

  • Potential triggers and precipitating events (can be present in up to 50% of patients and commonly include, infections, trauma, surgery and pregnancy)  
  • Severity of symptoms  
  • Effect on quality of life 
  • Diet (salt and fluid intake) 
  • Exercise (functional capacity) 
  • Features of autonomic neuropathy 
  • Autonomic review of symptoms 
  • Medication history (some medications may exacerbate symptoms) see non-pharmacological management for more information 
Questions to ask that may indicate presence of dysautonomia
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Questions to ask that may indicate presence of dysautonomia2

  • Do you have pain, burning or numbness in your hands and/or feet?
  • Do you have heat intolerance and/or abnormal sweating (increased or decreased) compared to your peers? 
  • Do you have severe constipation, episodes of diarrhea or early fullness/vomiting after eating? 
  • Do you have trouble controlling or emptying your bladder? 
  • Do you have severe dry eyes and/or mouth? 
  • Do your feet change colour when standing (acrocyanosis)? 

Physical examination1,2

Series of blood pressure and heart rate readings taken during the 10-minute active stand test, easily performed in a clinic setting.

Active stand test

  • While in the supine position for at least five minutes, measure the patient’s supine heart rate (HR) and blood pressure (BP). Then, have the patient stand upright for up to 10 minutes, measuring their HR and BP throughout those 10 minutes. 
  • Recording measurements may be stopped early if the patient meets hemodynamic criteria for a diagnosis of POTS before the end of 10 minutes or if the patient is having difficulty continuing the test due to significant symptoms (e.g., syncope).
  • Orthostatic tachycardia (heart rate increase of at least 30 bpm) must be sustained for at least two consecutive recordings (separated by at least one minute) after the first minute, for a POTS diagnosis.

Routine use of ancillary cardiac testing (e.g., echocardiograms and Holter monitors) is not recommended; however, these might be considered when:

  • Ruling out conditions that can mimic POTS.
  • Concerned about structural heart disease (e.g., cardiomyopathy, valvuopathy, past myocarditis, rheumatic fever).
  • Arrhythmia not coming from the sinus node is suspected.

Consider specialist consultation/referral for patients who show clear neurological deficits during the patient history review.

Orthostatic vital signs considerations
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Orthostatic vital signs considerations1,2

Active stand test
  • A patient’s very low resting heart rate climbing to the normal range with standing does not warrant a POTS diagnosis. In adults, a minimum standing heart rate of 90 bpm is required for diagnosis.  
  • Hemodynamic criteria do not need to be met at every visit (e.g., if a patient’s heart rate decreases to < 30 bpm in a single visit after diagnosis, POTS status should not be altered). 
  • If a patient shows some evidence of orthostatic intolerance but does not meet orthostatic tachycardia criteria during initial testing, reassess at a later date (in the morning if possible) as test results can vary depending on time of day, hydration status, etc.  

Practice point: Patients with symptoms of orthostatic intolerance, in the absence of a confirmed diagnosis of POTS, may still benefit from conservative management strategies.2

Differential diagnosis and comorbid conditions1,2

Assess for possible secondary causes of orthostatic tachycardia through a 12-lead ECG and routine blood tests, including complete blood count, electrolytes (Na+, K+, and Cl–), renal function (creatinine, and urea), ferritin, thyroid stimulating hormone and morning cortisol level.  

It is important to screen for comorbid or underlying conditions as these conditions might alter management and treatment response and prognosis over time. Possible primary causes and associated comorbid conditions may include: 

Diagnosis New

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Diagnosis of POTS is based on the presence of:1,3

> 3 months duration of symptoms of orthostatic intolerance that are at least partially relieved by recumbency

Sustained increase in heart rate (30 bpm in adults 19+) from supine position to upright within 10 minutes of standing. See Orthostatic vital signs

Absence of orthostatic hypotension (decrease in systolic blood pressure > 20 mm Hg or diastolic blood pressure > 10 mm Hg)

Many individuals experience a transient increase in heart rate upon standing. This is a potential manifestation of initial orthostatic hypotension but it is not a part of the POTS diagnosis.1  

Practice point: Overall, POTS is a clinical diagnosis which includes patient history, physical exam, and evaluation of orthostatic vitals. Additional investigations such as tilt table testing, autonomic nervous system testing, or plasma catecholamine testing may not be routinely needed. However, these tests can help identify POTS subtypes to direct management further if needed or provide clarity in cases of diagnostic uncertainty. These investigations are best ordered by a specialist familiar with dysautonomia.1,2

Specialist referral

Consider referral to a specialty clinic for POTS in cases of diagnostic uncertainty or when a patient experiences poor response to nonpharmacological and initial pharmacological treatments. POTS management can be provided by multidisciplinary teams where appropriate, including physicians and allied health professionals.1,2 

Management New

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Practice point: There is no single treatment that has been demonstrated to be effective in all POTS patients. POTS management will often require a combination of strategies, beginning with non-pharmacological interventions either on their own or in combination with pharmacological therapies, depending on symptom severity.1

Overarching management goals:1

    • Provide patient education (consider referring to the Patient and Caregiver Resources for examples of educational materials suggested by persons with lived experience)
    • Reduce symptoms
    • Enhance quality of life
    • Improve physical conditioning
    • Achieve symptom remission (if possible)

Proposed 2-step POTS management algorithm:1,2 

Adapted from CCS Position Statement on POTS and Related Disorders of Chronic Orthostatic Intolerance, 2020

Clinical accommodation and disability support  

Beyond the non-pharmacological and pharmacological management strategies outlined below, patients whose symptoms significantly affect their ability to work may require supplementary disability income support. Some patients with POTS will need some form of disability support and will need their clinician’s assistance in obtaining it. The presentation of symptoms can look different from patient to patient and can present on different timelines. It is important to keep this in mind during assessment and when providing corresponding documentation for disability claims. 

Non-pharmacological management  

First-line therapy for POTS should always include non-pharmacological strategies. Consider comorbid conditions in patients, as this might alter non-pharmacological treatment approaches. Strategies include:1,2 

Discontinuation of symptom-exacerbating medications and substances
  • First therapeutic step: Withdrawal of any medications/substances that may mimic or exacerbate symptoms of POTS: ADHD medications, ⍺- adrenergic blockers, excessive b-adrenergic blockers (whereas low dose b-blockers can be beneficial), calcium channel blockers, serotonin-norepinephrine reuptake inhibitors, monoamine oxidase inhibitors, tricyclic antidepressants and phenothiazine.

    Practice point: It can be very difficult for a patient to stop these medications. It may be possible to lower doses but difficult to withdraw completely. A risk-benefit discussion and assessment should be considered before implementing changes.2

Increase blood volume
  • Blood volume can be increased through dietary interventions such as: 
    • Daily oral fluid intake of at least 3 L.
    • Daily oral intake of ~10 g of NaCl via high-salt foods, NaCl sachets, salt sticks or salt tablets (if patient is not hypertensive). Salt intake should be introduced gradually. The method of salt intake will depend on patient preference and budget.  

    Practice point: For patients who do not respond to treatment or where supplemental salt intake is suspected to be suboptimal, 24-hour urine excretion can be done to provide an estimate of dietary salt intake (recommended urinary sodium target is >170 mmol/d).1

Exercise training
  • Exercise training (semi-recumbent exercise, at least initially) might alleviate symptoms, improve quality of life, and achieve remission in some POTS patients. The approach to exercise should consider post-exertional malaise (PEM) and the functional and exercise limitations of comorbid conditions such as ME/CFS.  Current exercise rehabilitation protocols for patients with POTS may not consider these. 
Compression garments
  • Use of lower body compression garments (e.g., waist-high compression stockings or abdominal binders) can be used to reduce venous polling and enhance venous return, which may help reduce symptoms of orthostatic intolerance. 
Exercise tips and considerations
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Tips and considerations1,2

Do NOT provide the following guidance if the patient has been diagnosed with ME/CFS or other comorbid conditions that can be made worse with exercise.

 

  • Consider the patient’s baseline level of function, severity of orthostatic intolerance symptoms and activity level capacity without symptom exacerbation before implementing exercise strategies.  
  • Exercise programs featuring aerobic training with additional leg resistance training have demonstrated improvements in patients. 
  • Exercises such as rowing, recumbent cycling or swimming are usually better tolerated than upright exercise. 
  • Consistent exercise at least four times per week, with sessions lasting at least 30 minutes, has been shown to contribute to success (if tolerated by the patient). It may take 4-6 weeks for some patients to notice improvements. 
  • If patients diagnosed with POTS are interested in support to engage in exercise, it is important to obtain that support from healthcare providers (physiotherapists, rehabilitation experts) who are knowledgeable and experienced in working with patients with both POTS and other comorbid conditions.  
  • Caution in patients with Hypermobile Ehlers-Danlos Syndrome (often comorbid with POTS) due to risk of injury.

 

For more information on how to manage ME/CFS, see Management of ME/CFS.  

Compression tips and considerations
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Tips and considerations1,2

  • Patients are advised to avoid warm environments and prolonged standing.  
  • Some patients may not tolerate compression garments for various reasons (e.g., gastrointestinal symptoms due to sensitivity to abdominal compression, skin sensitivity/bruising or heat intolerance). 
  • Start with compression of 20-30 mmHg and increase pressure as tolerated. 
  • Compression only below the thigh is unlikely to be effective.
  • Consider cheaper alternatives to compression garments for those unable to access prescription compression (e.g., high–waisted sports leggings, shapewear, compression sleeves, biker shorts).     

Pharmacological management

If patients have disabling symptoms of POTS or continue to experience debilitating symptoms after implementing non-pharmacologic strategies, consider pharmacologic therapies. These therapies aim to mitigate symptoms by improving central blood volume, enhancing vasoconstriction and reducing sinus tachycardia.1  

Pharmacologic strategies have the potential to support patients, particularly those at the beginning stages of exercise training.1

Practice point: Start with a low dose and up-titrate slowly to efficacy based on tolerance limitations. Achieving efficacy at low doses may help avoid side effects and medication burden. As needed dosing (“PRN” dosing) or two-drug combinations are often required to keep lower doses and to minimize side effects.2  

First-line pharmacological therapies1-15

References

  • [1]

    Raj SR, Guzman JC, Harvey P, Richer L, Schondorf R, Seifer C, et al. Canadian Cardiovascular Society Position Statement on Postural Orthostatic Tachycardia Syndrome (POTS) and Related Disorders of Chronic Orthostatic Intolerance. Can J Cardiol. 2020;36(3):357–72.

  • [2]

    Expert Opinion.

  • [3]

    Hasan B, Almasri J, Marwa B, Klaas KM, Fischer PR. Treatment of Postural Orthostatic Tachycardia Syndrome With Medication: A Systematic Review. J Child Neurol. 2020 Dec;35(14):1004–16.

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