Postural Orthostatic Tachycardia Syndrome (POTS)

Overview

Postural orthostatic tachycardia syndrome (POTS) is a form of autonomic dysfunction with orthostatic intolerance that affects up to 1% of adolescents with chronic fatigue, dizziness, and, often, gastrointestinal discomfort or other forms of chronic pain. With treatment, most patients can fully recover and return to normal life activities.

Other Names & Coding

Autonomic disorder (vague, but includes POTS)
Autonomic dysfunction (over-arching group of conditions of which POTS is a subset)
Chronic fatigue syndrome (CFS) (has diagnostic criteria for research use; over half of individuals with CFS likely have POTS or features thereof)
Dysautonomia (same as autonomic dysfunction)
Functional disorder (Lacking a specific diagnostic test, POTS is a functional disorder, as is migraine headache. Affected patients may have other functional disorders such as chronic pain or functional GI or neurologic disorder.)
Myalgic encephalopathy (ME) (British synonym for the American “CFS”)
Orthostatic intolerance (broad group of problems of which POTS is the form that is chronic and associated with excessive postural tachycardia)
Postural tachycardia syndrome (preferred in Great Britain and abbreviated “PoTS.”)

POTS has not been assigned a specific ICD-10 code; the following are commonly used:

ICD-10 coding

I49.8, Other specified cardiac dysrhythmia (POTS is listed as an example)

I95.1, Orthostatic hypotension (sometimes occurs with POTS)

G90.9, Disorder of the autonomic nervous system, unspecified

R42, Dizziness

R53.82, Chronic fatigue

For other specific symptoms, search ICD10Data.com.

Prevalence

By best estimates, there are more than a million American adolescents with POTS. While prevalence and incidence data are incompletely known, POTS likely affects more than 1% of American adolescents.

Fatigue is a nearly uniform feature of POTS. Overall, 31% of American adolescent girls experience bothersome fatigue more than once each week. [Ghandour: 2004] Studies from Holland suggest that 21% of girls and 7% of boys have had persistent tiredness for more than 3 months. [ter: 2006] A population-based community study in Britain showed that more than 1% of adolescents are disabled with fatigue and unable to participate in regular activities. [Rimes: 2007] Further, many patients with chronic fatigue, when subjected to autonomic testing, have evidence of autonomic dysfunction. [Silverman: 2010] How many of these have POTS? No one knows, but POTS accounts for a lot of disabling fatigue, and these data suggest that POTS could affect more than 1% of American adolescents. [Bhatia: 2016]

Many patients with POTS see many physicians and undergo numerous expensive tests (with normal results); a survey of nearly 5,000 patients with physician-diagnosed POTS revealed that the median time from symptom onset to diagnosis was 2 years. [Shaw: 2019] Without a clear diagnosis and initiation of effective treatment, medical expenses are significant as patients and families seek answers and improvement.

Genetics

The genetics of POTS are unknown. However, about 15% of patients with POTS have a first-degree relative with POTS, and POTS is much more common in whites than other races. [Shaw: 2019] Presumably, there are some genetic origins of these variations. [Johnson: 2010]

Many patients with POTS also have hypermobility. This has led to the speculation (as yet unconfirmed) that POTS is genetically linked to hypermobility and/or hypermobility syndromes.

Prognosis

There are limited data about outcomes of adolescent-onset POTS. A study including adolescents and adults found that approximately 37% no longer had POTS 1 year after starting treatment. [Kimpinski: 2012] A study of adolescents surveyed about 1½ years after starting treatment found that most were significantly improved, especially those treated with beta-blockers. [Lai: 2009] A longer (average 5 years) follow-up survey of adolescents with POTS demonstrated that symptoms of POTS were resolved or markedly reduced in about 86% of respondents. [Bhatia: 2016] Even a vast majority of adolescents with POTS and/or debilitating chronic pain can return to normal activities within months of completing an intensive recovery program. [Bruce: 2017] While not all adolescents with POTS fully recover, optimism for good recovery for the majority of affected patients is warranted.

Practice Guidelines

There are no official practice guidelines, mostly because of a lack of comparative studies of treatment options. However, these review articles focus on the management of POTS in adolescents:

Johnson JN, Mack KJ, Kuntz NL, Brands CK, Porter CJ, Fischer PR.
Postural orthostatic tachycardia syndrome: a clinical review.
Pediatr Neurol. 2010;42(2):77-85. PubMed abstract
Since this publication, the diagnostic criteria for adolescent POTS have been refined to include a postural tachycardia of at least 40 beats per minute change (instead of 30, as for adults). The suggested non-pharmacologic and pharmacologic treatment strategies remain the same.

Kizilbash SJ, Ahrens SP, Bruce BK, Chelimsky G, Driscoll SW, Harbeck-Weber C, Lloyd RM, Mack KJ, Nelson DE, Ninis N, Pianosi PT, Stewart JM, Weiss KE, Fischer PR.
Adolescent fatigue, POTS, and recovery: a guide for clinicians.
Curr Probl Pediatr Adolesc Health Care. 2014;44(5):108-33. PubMed abstract / Full Text
This paper broadens the discussion of POTS treatment by including the management of patients with fatigue, even those without POTS, and emphasizing multidisciplinary functional restoration of debilitated patients.

Stewart JM, Boris JR, Chelimsky G, Fischer PR, Fortunato JE, Grubb BP, Heyer GL, Jarjour IT, Medow MS, Numan MT, Pianosi PT, Singer W, Tarbell S, Chelimsky TC.
Pediatric disorders of orthostatic intolerance.
Pediatrics. 2018;141(1). PubMed abstract / Full Text
This paper reviews standard management of adolescents with POTS and also mentions non-standard treatments that only have anecdotal support of efficacy.

Roles of the Medical Home

Patients with chronic functional disability and a variety of symptoms often seek lots of subspecialty care. With normal results of tests, patients tend to keep seeking new doctors and new tests. It is vitally important that an informed care provider coordinates the evaluation and management. With an accurate diagnosis of POTS and appropriate treatment, unnecessary consultation and “doctor shopping” can be avoided and the focus on functional restoration maintained.

Clinical Assessment

Overview

The clinical evaluation of tired, dizzy, uncomfortable adolescents needs to 1) consider the diagnosis of postural orthostatic tachycardia syndrome and 2) identify any relevant comorbidities. POTS is a real condition, even if it is a functional disorder with normal results on laboratory testing and imaging. Treatment is effective and recovery is likely.

Pearls & Alerts for Assessment

Measuring postural heart rate change

For patients with chronic fatigue and postural dizziness, determine the postural heart rate change. With the patient resting calmly supine for at least a few minutes, measure the resting heart rate and measure it again after the patient stands and remains still for at least 3 minutes. An increase in heart rate of more than 40 beats per minute would be considered excessive. Formal tilt table testing could corroborate the finding.

POTS diagnosis

The diagnosis of POTS is based on clinical history (>3 months of daily intolerance of upright position) coupled with postural tachycardia. Acute and intermittent symptoms do not qualify for the diagnosis. Similar symptoms without excessive postural tachycardia indicate orthostatic intolerance but not POTS; the treatment would be identical to the treatment of POTS except that medications are not needed for orthostatic intolerance when there is not excessive postural tachycardia.

Screening

No screening is recommended related to POTS.

Presentations

Orthostatic intolerance
Patients have upright dizziness and feel better lying down. Sometimes they also have other upright symptoms (headache, heaviness, fatigue, cloudy thinking) that are also at least partially abated by lying down. Typically, the dizziness is a sense of lightheadedness or instability, but sometimes patients report room-spinning vertigo.

Chronic fatigue
Almost all POTS patients have had bothersome fatigue for at least 3 months.

Fainting
Orthostatic hypotension sometimes develops after postural tachycardia when upright. If the blood pressure drops more than 20 mm Hg prior to an increase in heart rate, the problem is orthostatic hypotension rather than POTS. One way or the other, patients with POTS sometimes do faint.

Nausea and other gastrointestinal symptoms
Most POTS patients have at least some gastrointestinal distress along the lines of irritable bowel syndrome (functional gastrointestinal disorder). Of course, before assuming that POTS is the cause, celiac disease and inflammatory bowel disease should be considered.

Heat intolerance
Many patients with POTS have irregular temperature regulation, such as “wilting” outside on hot days, requiring different numbers of layers of clothes than peers, or sensing low-grade fevers.

Brain fog
Many POTS patients feel like they cannot think clearly or remember things. It is not clear whether this relates to altered cerebral blood flow or altered chemical activation of brain cells. However, cognitive testing does not usually reveal measurable deficits.

Diagnostic Criteria

Chronic (>3 months) orthostatic intolerance with daily symptoms impairing normal function. Symptoms, such as dizziness, come on when upright and abate, at least partially, when recumbent.

Excessive postural tachycardia. Research is based on 70-degree, head-up, tilt table testing, but some investigators find a bedside standing test to be adequate with several minutes resting calmly supine and them at least 3 minutes upright. For an adolescent, a sustained increase of more than 40 beats per minute is “excessive.”

Clinical Classification

There is no clinically useful classification of POTS – either the patient has it or not. A distinction is that similar symptoms without excessive postural tachycardia count as “orthostatic intolerance” instead of POTS. Orthostatic intolerance is treated similarly to POTS (increased fluid and salt intake, daily aerobic exercise, adequate sleep, cognitive behavioral therapy) but does not require medications.

For researchers, there are sometimes distinctions among categorizations of POTS – hyperadrenergic, post-infectious, and neuropathic. These descriptions are not associated with a different treatment or prognosis; categorizing individual patients offers no value.

Differential Diagnosis

First, differentiate between orthostatic intolerance in general and the subset of orthostatic intolerance that includes excessive postural tachycardia (POTS).

Second, identify major comorbidities that must concurrently be treated, such as iron deficiency, hypovitaminosis D, and anxiety/depression.

Third, some chronic pain syndromes and debilitation from other causes result in postural tachycardia due to deconditioning (“bedrest postural tachycardia”), but these conditions do not usually include persistent upright symptoms that abate with recumbency.

Fourth, nausea and abdominal pain are common with POTS but can also be caused by sugar malabsorption, celiac disease, and inflammatory bowel disease. Median arcuate ligament syndrome is a rare cause of abdominal pain but has (rarely) been reported in association with POTS.

Fifth, severe headaches and abdominal pain occur with POTS, but brain tumors and appendicitis can also coincidentally occur with POTS; the accurate diagnosis of POTS does not preclude the concurrent diagnosis of a separate condition.

Medical Conditions Causing Condition

Typically, POTS has no known cause, but there are associations. POTS is most common in high-achievers and is more common in whites than in other races and in girls than in boys (~2:1 prevalence). The onset is usually within 1 or 2 years of the onset of puberty. Hypermobility is common. These are associations but not causal factors.

About ⅔ of patients with POTS experience onset of orthostatic intolerance following an infection (such as mononucleosis) or an injury (such as a concussion). The illness or injury seems not to have caused POTS but, rather, to have triggered its onset in a predisposed individual. There is no evidence of an ongoing infection causing the POTS, and only in very rare cases are neurologically relevant antibodies associated with POTS.

Comorbid & Secondary Conditions

Patients with POTS often suffer from comorbid illnesses, including:
  • Iron deficiency: Nearly ½ of patients with POTS have ferritin levels <20 ng/dL and benefit some from iron supplementation.
  • Vitamin D deficiency: About ⅓ of POTS patients have 25-OH-vitamin D levels < 20 ng/dL and benefit to some degree from vitamin D supplementation.
  • Anxiety and depression: Approximately ⅓ of patients with POTS have significant anxiety and/or depression, some of which preceded the onset of POTS symptoms. Similar neurotransmitters are involved in the regulation of both the autonomic nervous system and mood, which may be a factor. Chronic debilitation due to POTS could also trigger depression.
  • Headache, other forms of chronic pain: Seen in the majority of those with POTS
  • Motion sickness: Also common in POTS
Other co-morbidities are purportedly linked to POTS, but the nature of the association is unproven. Nonetheless, hypermobility, mast cell activation disorder, and other comorbid pathologies are treated similarly whether the patient also has POTS or not. See also [Ojha: 2011].

History & Examination

Current & Past Medical History

Nearly all individuals with POTS have postural dizziness and chronic fatigue; about ⅔ of individuals also have headache and abdominal discomfort.

POTS almost never occurs prior to the onset of puberty (menarche in girls, growth spurt in boys).

Prior to the onset of POTS, ⅔ of adolescent report a significant illness (e.g., mononucleosis) or injury (e.g., concussion).

Family History

Is there a history of family members with POTS-like symptoms? (About 15% of those with POTS have a first-degree relative with a history consistent with POTS.)

Pregnancy/Perinatal History

POTS has not been associated with any early life features.

Developmental & Educational Progress

Most patients who develop POTS are high-achievers with a history of academic and extracurricular success. This makes the fall to fatigue and debilitation even more challenging.

Maturational Progress

POTS almost never occurs in pre-pubertal children.

Social & Family Functioning

With POTS, maintaining academic and extra-curricular success (or even participation) is difficult. Loving parents tend to do whatever they can to help the patient succeed. However, individuals typically link 2 activities with their recovery – staying in school and exercising regularly, the very activities that are most challenging with POTS. A clear diagnosis and treatment plan help patients and families support activity and recovery rather than inactivity and illness. Parents need to facilitate normal activities rather than helping the patient to stay comfortable with increasing debilitation.

Physical Exam

General

POTS patients typically look completely well.

Vital Signs

Resting vital signs are typically normal with, perhaps, blood pressures tending to be on the lower side of the normal range.

Postural tachycardia is a key to diagnosis.

With chronic symptoms, deconditioning intervenes and there can be some resting tachycardia.

Growth Parameters

Ht | Wt | BMI Is the patient having difficulty maintaining or gaining weight?

Skin

Normal - perhaps coolness and duskiness of peripheral extremities when extremities are dependent

Heart

Normal - only the postural tachycardia is unique to POTS patients

Abdomen

Normal - sometimes bloating in those with predominant gastrointestinal symptoms

Neurologic Exam

Normal - perhaps increased sweating

Testing

Testing is useful only when needed to confirm postural tachycardia, narrow the differential diagnosis, or evaluate for comorbidities.

Other Testing

Standing gravitational challenge or tilt table testing to identify postural tachycardia

Specialty Collaborations & Other Services

Depending on the experience of the primary provider, it might help for the patient to see someone experienced in managing POTS, be that another general pediatrician, a cardiologist, or a neurologist.

Pediatric Cardiology (see UT providers [7])

May be helpful in evaluation if sufficiently experienced with POTS

Pediatric Neurology (see UT providers [5])

May be helpful in evaluation if sufficiently experienced with POTS

Treatment & Management

Overview

Management of individuals with POTS requires patience, persistence, and positivity. Non-pharmacologic treatment (fluids, salt, exercise, therapy) may be sufficient and should be used for all patients; pharmacologic treatment (corticosteroids, beta-blockers, antidepressants) may be very helpful for select patients. Of those who need medications, most can discontinue them after 1-3 years.

Although detailed outcome data are not available, survey studies of POTS patients suggest that the vast majority make great improvements during the months following initiation of treatment. [Lai: 2009] Over a few years, nearly 90% recover or are greatly improved; yet, some patients do not fully recover. [Bhatia: 2016] [Bhatia: 2016]

Helping the patient and the family understand the condition, implement and maintain needed lifestyle and dietary changes, and sustain optimism and goals for the future are key.

Pearls & Alerts for Treatment & Management

Behavioral choices are critical

Instruct patients to increase fluid and salt intake, exercise daily, get adequate/appropriate sleep, and engage in cognitive behavioral therapy techniques. Prescription medications are most useful when the patient is fully engaged in these non-pharmacologic treatment modalities.

Patient advice

Drink so much that your urine looks clear like water. Eat as much salt as you can tolerate. Get aerobic exercise every day (working up to 30 minutes in a single daily session). Get enough sleep every night and don’t take naps. Get help from a good psychologist - and take your medication, too.

How should common problems be managed differently in children with Postural Orthostatic Tachycardia Syndrome (POTS)?

Growth or Weight Gain

Maintain normal weight and eat regular “healthy” meals; avoid weight loss or inappropriate weight gain.

Development (Cognitive, Motor, Language, Social-Emotional)

A good mental attitude (focusing on working for recovery rather than adjusting to debility) and cognitive behavioral therapy are very helpful. Support of the recovery (not the debility) from family and friends helps. Sometimes, a brief, temporary reduction in the school load is necessary.

Viral Infections

Patients with POTS tend to get sicker with common illnesses than do other patients. So, it is vitally important that they even exaggerate their baseline fluid/salt intake and exercise/activity plan when they are ill.

Bacterial Infections

Similar to viral infections

Over the Counter Medications

Caffeine can stall recovery. Antihistamines might slightly hinder recovery, so they should only be used if truly needed.

Common Complaints

Everything - patients with POTS feel terrible in just about every way with just about any symptom.

Other

Patients with POTS are further compromised when they get behind on regular healthy lifestyle activities, such as stress management, weight management, exercise, regular meals, and sleep. They must keep their bodies in balance.

Systems

For management of many patients with POTS, a physician who is familiar with the condition and a psychologist skilled in cognitive behavioral therapy are the only specialists needed. If the primary care provider or medical home physician is not familiar with POTS, involvement of a POTS-aware physician can help – be that a generalist, a neurologist, or a cardiologist. Some patients are more likely to continue daily exercise if they have a physical therapist or coach involved.

Mobility/Function/ADLs/Adaptive

Focus management on the interventions known to be helpful: oral fluids, salt, exercise, and cognitive behavioral therapy (CBT). Most of the non-pharmacologic treatment can become lifelong habits.
  • Fluids: Advise patients with POTS to drink enough that the urine is clear.
  • Salt: Eat as much salt as tolerated; reduce salt intake once the dizziness has been fully resolved without POTS medication for about 6 months.
  • Compression stockings may be helpful for problematic dizziness; they can be discontinued when the dizziness is tolerable.
  • Exercise: Adaptation to fatigue and other symptoms of POTS often leads to deconditioning, which requires reconditioning exercise to reverse. Find the amount of upright exercise the patient can do – even if just 2 minutes of light walking. Make the exercise intense enough to gradually increase tolerance aiming for at least 30 minutes of aerobic exercise every day. Sometimes, patients comply better with exercise regimens if they are followed and guided by a physical therapist.
With POTS, maintaining academic and extra-curricular success (or even participation) is difficult. Loving parents tend to do whatever they can to help the patient succeed. However, individuals typically link 2 activities with their recovery – staying in school and exercising regularly, the very activities that are most challenging. Parents need to facilitate normal activities rather than merely helping the patient to stay comfortable with increasing debilitation. Although sometimes a brief, temporary reduction in school load is necessary, support (of recovery, not of debility) from family and friends helps. There are sometimes ups and downs, with the downs correlating to intercurrent illnesses, even after POTS seems to have resolved. Aggressive attention to non-pharmacologic therapies is usually adequate.

Specialty Collaborations & Other Services

Physical Therapy (see UT providers [62])

May be helpful in designing and monitoring response to a reconditioning program

Mental Health/Behavior

Cognitive behavioral therapy (CBT) is vitally important in the management of POTS-related symptoms. Up to ⅓ of those with POTS have concurrent struggles with anxiety and/or depression and might require counseling or medication management for those problems. Standard medications, such as SSRIs, and dosing are appropriate and may also help with POTS symptoms. See the Portal’s Depression and Anxiety Disorders for more information.

Specialty Collaborations & Other Services

Behavioral Therapies (see UT providers [30])

Referral for cognitive behavioral therapy (CBT) is likely appropriate for all those diagnosed with POTS.

Therapy/Counseling > … (see UT providers [777])

Other mental health providers may be helpful for assessment and treatment of depression and/or anxiety, or if no CBT providers are available. (This is a parent category with up to 30 subcategories.)

Psychiatry/Medication Management (see UT providers [62])

May be helpful if medications are needed for treatment of depression or anxiety

Pharmacy & Medications

When symptoms are severe and non-pharmacologic interventions have not adequately reduced symptoms, medication can be used.

Fludrocortisone: Some authorities start with fludrocortisone 0.1 mg daily to help the body retain fluid and salt, but other authorities find that generous oral fluid and salt intake obviates the need for fludrocortisone.

Beta-blocker: A beta-blocker can facilitate peripheral blood flow; metoprolol 25 mg on awakening in the morning and again 4-6 hours later is usually adequate. (Rarely, a beta-blocker can lead to more fatigue, and a different beta-blocker, such as atenolol, can be tried.) If the beta-blocker is not adequate, midodrine can be substituted or added – 2.5 mg, 3 times daily, increasing to 5 mg or even 7.5 or 10 per dose if needed. Going up too quickly on the dose causes bothersome scalp sensations. Medications are usually continued for 1-2 years. Adjustments in dose are based on symptoms and symptom resolution. New signs of different conditions or lack of improvement should prompt consideration of a different approach.

Other considerations include:
  • Limit beta-blockers if there is excessive resting bradycardia.
  • Absent excessive postural tachycardia, medications are not indicated for orthostatic intolerance.
  • If the patient still has excessive postural tachycardia an hour after taking a beta-blocker dose, the dose could be increased.
  • Beta-blockers can cause rare worsening of asthma, rare difficulty recognizing symptoms of hypoglycemia in patients with diabetes, and occasional increased fatigue (especially with propranolol). Midodrine can cause a “creepy-crawly” sensation of the scalp with too high a dose or too rapidly increasing a dose.

Specialty Collaborations & Other Services

Psychiatry/Medication Management (see UT providers [62])

May be helpful if medications are needed for treatment of depression or anxiety

Neurology

In some centers, neurologists serve as the primary managers of POTS-specific situations. Neurologists may also be helpful in the management of otherwise recalcitrant headaches.

Specialty Collaborations & Other Services

Pediatric Neurology (see UT providers [5])

Refer if local expertise is concentrated among neurologists or for specific problems in which neurology has expertise, such as persistent headaches.

Gastro-Intestinal & Bowel Function

Nausea and gastro-intestinal symptoms are common in POTS. Medication management of delayed gastric emptying and the dysmotility associated with irritable bowel syndrome (functional gastrointestinal disorder) are commonly experienced with POTS.

Specialty Collaborations & Other Services

Pediatric Gastroenterology (see UT providers [4])

May be helpful in managing GI problems not responsive to primary care measures

Endocrine/Metabolism

Absent the coincidental occurrence of hypothyroidism or adrenal insufficiency, endocrine disorders are not a part of POTS. However, some patients have been over-treated with steroids before recognizing POTS as the primary diagnosis; an endocrinologist can facilitate weaning off steroids.

Specialty Collaborations & Other Services

Pediatric Endocrinology (see UT providers [7])

Refer when there are concerns about endocrinopathy or if help is needed weaning from steroid dependency.

Cardiology

Although the symptoms of POTS are not cardiac in origin, cardiologists are often consulted, and some have developed substantial expertise. Evaluation for other causes of intermittent tachycardia may be needed in some patients who do not respond to standard management of POTS.

Specialty Collaborations & Other Services

Pediatric Cardiology (see UT providers [7])

Refer if needed to a cardiologist who has expertise/experience in the diagnosis and management of POTS or for specific cardiac concerns.

Musculoskeletal

Some patients with POTS will have excessive musculoskeletal pain with or without hypermobility. Physical therapy can help with assessing and managing pain.

Specialty Collaborations & Other Services

Physical Therapy (see UT providers [62])

Refer for evaluation of excessive pain and for designing and monitoring an exercise program.

Maturation/Sexual/Reproductive

Many female patients with POTS have very bothersome menstrual periods, and POTS symptoms are often worse during menstruation. Effective hormonal therapy to modulate, minimize, or temporarily stop menstruation may be very useful.

Specialty Collaborations & Other Services

Gynecology: Pediatric/Adolescent; Special Needs (see UT providers [21])

Refer if assistance in management of menstrual problems is needed.

Sleep

Disordered or inadequate sleep can exacerbate fatigue and further complicate recovery from POTS. Rigorous sleep hygiene is key with at least 8-9 hours of sleep at night; avoid naps.

Specialty Collaborations & Other Services

Pediatric Sleep Medicine (see UT providers [3])

Consultation with sleep medicine may be warranted if primary care and family measures are not successful in attaining adequate sleep.

Complementary & Alternative Medicine

Both before and after the diagnosis of POTS, patients and families often seek solutions to symptoms wherever they may be found. No interventions beyond those discussed above have been proven effective in treating the autonomic dysregulation that underlies POTS, though some interventions may provide temporary symptomatic relief. Examples include massage for achiness and stress reduction and magnesium and riboflavin for headaches.

It is important to avoid interventions with side effects, such as autonomic destabilization from caffeine use and dietary impacts of avoiding gluten or other components of foods. Absent evidence of benefit, cost should be a consideration (e.g., carnitine or coenzyme Q10 use). Focus management on interventions known to be helpful: oral fluids, salt, exercise, and cognitive behavioral therapy.
No Related Issues were found for this diagnosis.

Ask the Specialist

What’s wrong with my patient? She’s tired and dropping out of school, and all the test results are normal.

Is she dizzy when she stands up? If so, check postural heart rate changes. This might be POTS, and treatment can help.

My patient with POTS is not getting better. What other medication can I try?

Failure to improve only rarely relates to medication. Your patient will most likely need to get more aggressive about increasing fluid and salt intake and getting daily aerobic exercise.

My patient is too tired to exercise. What can we do?

Find the amount of upright exercise the patient can do – even if just 2 minutes of light walking. Make the exercise intense enough so the patient is breathing a bit faster than normal. Then, increase the duration of that daily exercise by 1-2 minutes every 5 days. Gradually, the tolerance for exercise will increase. Keep going until the patient can continue with 30 minutes of aerobic exercise every day.

My patient is too sick and just can’t do all this?

True. No patient can do all this alone. Cognitive behavioral therapy is vital. Your patient needs to keep going forward, 1 little step at a time. Engage family and friends in supporting the recovery of normal function (as opposed to comfort with disability).

Resources for Clinicians

On the Web

Postural Orthostatic Tachycardia Syndrome (GARD)
Symptoms, causes, inheritance, treatment, prognosis, and research related to POTS; Genetic and Rare Diseases Information Center.

Adolescent POTS – North American Pediatric Guidelines (PDF Document 5.7 MB)
PDF of slides with information about prevalence, presentations, comorbidities, diagnosis, treatment, and outcome (including parents' role); by Phil Fischer, MD (module author) for the "London Masterclass" sponsored by POTS UK.

Postural Orthostatic Tachycardia Syndrome - Grand Rounds Lecture
One-hour video of a Grand Rounds lecture at Primary Children's Hospital by Phillip Fischer, MD (2014), includes patient accounts of the condition.

Helpful Articles

PubMed search for articles about postural orthostatic tachycardia syndrome published in the last 3 years

Bhatia R, Kizilbash SJ, Ahrens SP, Killian JM, Kimmes SA, Knoebel EE, Muppa P, Weaver AL, Fischer PR.
Outcomes of Adolescent-Onset Postural Orthostatic Tachycardia Syndrome.
J Pediatr. 2016;173:149-53. PubMed abstract
Over a few years, 86% or more of patients with adolescent-onset POTS report recovery or significant improvement.

Johnson JN, Mack KJ, Kuntz NL, Brands CK, Porter CJ, Fischer PR.
Postural orthostatic tachycardia syndrome: a clinical review.
Pediatr Neurol. 2010;42(2):77-85. PubMed abstract
Since this publication, the diagnostic criteria for adolescent POTS have been refined to include a postural tachycardia of at least 40 beats per minute change (instead of 30, as for adults). The suggested non-pharmacologic and pharmacologic treatment strategies remain the same.

Kizilbash SJ, Ahrens SP, Bruce BK, Chelimsky G, Driscoll SW, Harbeck-Weber C, Lloyd RM, Mack KJ, Nelson DE, Ninis N, Pianosi PT, Stewart JM, Weiss KE, Fischer PR.
Adolescent fatigue, POTS, and recovery: a guide for clinicians.
Curr Probl Pediatr Adolesc Health Care. 2014;44(5):108-33. PubMed abstract / Full Text
This paper broadens the discussion of POTS treatment by including the management of patients with fatigue, even those without POTS, and emphasizing multidisciplinary functional restoration of debilitated patients.

Lai CC, Fischer PR, Brands CK, Fisher JL, Porter CB, Driscoll SW, Graner KK.
Outcomes in adolescents with postural orthostatic tachycardia syndrome treated with midodrine and beta-blockers.
Pacing Clin Electrophysiol. 2009;32(2):234-8. PubMed abstract
Even over the months of initial treatment, most patients receiving a beta-blocker improve and credit the medication with their improvement.

Stewart JM, Boris JR, Chelimsky G, Fischer PR, Fortunato JE, Grubb BP, Heyer GL, Jarjour IT, Medow MS, Numan MT, Pianosi PT, Singer W, Tarbell S, Chelimsky TC.
Pediatric disorders of orthostatic intolerance.
Pediatrics. 2018;141(1). PubMed abstract / Full Text
This paper reviews standard management of adolescents with POTS and also mentions non-standard treatments that only have anecdotal support of efficacy.

Clinical Tools

Approach to Treatment of POTS
Care Process for POTS
Figure 2 from Postural orthostatic tachycardia syndrome: a clinical review [Johnson: 2010]
Click image to access the abstract (article requires a subscription to Pediatric Neurology).

Medications Management of POTS
Medications Used in Managing POTS

Table 2 from Pediatric disorders of orthostatic intolerance [Stewart: 2018]
Click image for free access to table.

Patient Education & Instructions

Postural Tachycardia Syndrome (POTS) (Mayo Clinic)
Five-minute video for patients and families of Phil Fischer, MD (author of this module) explaining POTS.

Resources for Patients & Families

Information on the Web

Postural Orthostatic Tachycardia Syndrome (KidsHealth)
Summary of key facts to know about POTS for those first learning about the condition.

Standing Up To POTS
Focussed on providing original research and educating those learning about, living with, or being treated for POTS.

National & Local Support

Dysautonomia International
In-depth information for patients (salty recipes, exercise tips, crisis hotlines), family (caregiver tips, connect with others, treatment options), and clinicians (patient education, expert recommendations, journal articles).

Dysautonomia Youth Network of America, Inc. (DYNA)
Dedicated to providing supports, information, and programs about childhood/adolescent/young adult onset dysautonomia conditions.

PoTS UK
Patient- and family-focused organization in the United Kingdom with information about POTS (called Postural Tachycardia Syndrome there); includes a list of doctors in the UK, Canada, Australia, Sweden, and US with experience treating PoTS.

Studies/Registries

Clinical Studies of POTS (clincaltrials.gov)
Comprehensive list of studies registered with the National Institutes of Health.

Services for Patients & Families in Utah (UT)

For services not listed above, browse our Services categories or search our database.

* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.

Authors & Reviewers

Initial publication: November 2019
Current Authors and Reviewers:
Author: Philip (Phil) R. Fischer, MD

Bibliography

Bhatia R, Kizilbash SJ, Ahrens SP, Killian JM, Kimmes SA, Knoebel EE, Muppa P, Weaver AL, Fischer PR.
Outcomes of Adolescent-Onset Postural Orthostatic Tachycardia Syndrome.
J Pediatr. 2016;173:149-53. PubMed abstract
Over a few years, 86% or more of patients with adolescent-onset POTS report recovery or significant improvement.

Bruce BK, Weiss KE, Ale CM, Harrison TE, Fischer PR.
Development of an Interdisciplinary Pediatric Pain Rehabilitation Program: The First 1000 Consecutive Patients.
Mayo Clin Proc Innov Qual Outcomes. 2017;1(2):141-149. PubMed abstract / Full Text

Ghandour RM, Overpeck MD, Huang ZJ, Kogan MD, Scheidt PC.
Headache, stomachache, backache, and morning fatigue among adolescent girls in the United States: associations with behavioral, sociodemographic, and environmental factors.
Arch Pediatr Adolesc Med. 2004;158(8):797-803. PubMed abstract

Johnson JN, Mack KJ, Kuntz NL, Brands CK, Porter CJ, Fischer PR.
Postural orthostatic tachycardia syndrome: a clinical review.
Pediatr Neurol. 2010;42(2):77-85. PubMed abstract
Since this publication, the diagnostic criteria for adolescent POTS have been refined to include a postural tachycardia of at least 40 beats per minute change (instead of 30, as for adults). The suggested non-pharmacologic and pharmacologic treatment strategies remain the same.

Kimpinski K, Figueroa JJ, Singer W, Sletten DM, Iodice V, Sandroni P, Fischer PR, Opfer-Gehrking TL, Gehrking JA, Low PA.
A prospective, 1-year follow-up study of postural tachycardia syndrome.
Mayo Clin Proc. 2012;87(8):746-52. PubMed abstract / Full Text

Kizilbash SJ, Ahrens SP, Bruce BK, Chelimsky G, Driscoll SW, Harbeck-Weber C, Lloyd RM, Mack KJ, Nelson DE, Ninis N, Pianosi PT, Stewart JM, Weiss KE, Fischer PR.
Adolescent fatigue, POTS, and recovery: a guide for clinicians.
Curr Probl Pediatr Adolesc Health Care. 2014;44(5):108-33. PubMed abstract / Full Text
This paper broadens the discussion of POTS treatment by including the management of patients with fatigue, even those without POTS, and emphasizing multidisciplinary functional restoration of debilitated patients.

Lai CC, Fischer PR, Brands CK, Fisher JL, Porter CB, Driscoll SW, Graner KK.
Outcomes in adolescents with postural orthostatic tachycardia syndrome treated with midodrine and beta-blockers.
Pacing Clin Electrophysiol. 2009;32(2):234-8. PubMed abstract
Even over the months of initial treatment, most patients receiving a beta-blocker improve and credit the medication with their improvement.

Ojha A, Chelimsky TC, Chelimsky G.
Comorbidities in pediatric patients with postural orthostatic tachycardia syndrome.
J Pediatr. 2011;158(1):20-3. PubMed abstract

Rimes KA, Goodman R, Hotopf M, Wessely S, Meltzer H, Chalder T.
Incidence, prognosis, and risk factors for fatigue and chronic fatigue syndrome in adolescents: a prospective community study.
Pediatrics. 2007;119(3):e603-9. PubMed abstract

Shaw BH, Stiles LE, Bourne K, Green EA, Shibao CA, Okamoto LE, Garland EM, Gamboa A, Diedrich A, Raj V, Sheldon RS, Biaggioni I, Robertson D, Raj SR.
The face of postural tachycardia syndrome - insights from a large cross-sectional online community-based survey.
J Intern Med. 2019;286(4):438-448. PubMed abstract / Full Text

Silverman MN, Heim CM, Nater UM, Marques AH, Sternberg EM.
Neuroendocrine and immune contributors to fatigue.
PM R. 2010;2(5):338-46. PubMed abstract / Full Text

Stewart JM, Boris JR, Chelimsky G, Fischer PR, Fortunato JE, Grubb BP, Heyer GL, Jarjour IT, Medow MS, Numan MT, Pianosi PT, Singer W, Tarbell S, Chelimsky TC.
Pediatric disorders of orthostatic intolerance.
Pediatrics. 2018;141(1). PubMed abstract / Full Text
This paper reviews standard management of adolescents with POTS and also mentions non-standard treatments that only have anecdotal support of efficacy.

ter Wolbeek M, van Doornen LJ, Kavelaars A, Heijnen CJ.
Severe fatigue in adolescents: a common phenomenon?.
Pediatrics. 2006;117(6):e1078-86. PubMed abstract