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Postural Orthostatic Tachycardia Syndrome (POTS)

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Guidance for primary care clinicians diagnosing and managing children with POTS
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. The clinical evaluation of tired, dizzy, uncomfortable adolescents needs to 1) consider the diagnosis of postural orthostatic tachycardia syndrome and/or orthostatic intolerance and 2) identify any relevant comorbidities. POTS is a real condition, even if it is a functional disorder with normal laboratory testing and imaging results. Most patients can fully recover and return to normal life activities with treatment.

Other Names

Postural tachycardia syndrome (preferred in Great Britain and abbreviated “PoTS.”)
Autonomic disorder or autonomic dysfunction (over-arching group of conditions of which POTS is a subset)
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.)
Orthostatic intolerance (broad group of problems characterized by bothersome symptoms when upright that improve when lying down; POTS is the form that is chronic and associated with excessive postural tachycardia)

Key Points

Importance of diagnosis
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.
Diagnosis criteria
The diagnosis of POTS is based on clinical history (>3 months of daily intolerance of upright position) coupled with postural tachycardia (>40 beats per minute increase). 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.
Management of POTS symptoms
Advise patients to drink so much that their urine looks clear, like water. Eat as much salt as you can tolerate; some patients prefer to take salt tablets/capsules. 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.
Importance of healthy lifestyle
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. 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.
Infections and POTS
Patients with POTS tend to get sicker with common illnesses than do other patients. Patients with POTS feel terrible in just about every way with just about any symptom. It is vitally important that they even adhere to and even exaggerate their baseline fluid/salt intake and exercise/activity plan when they are ill.
Failure to improve
Your patient needs to keep going forward, 1 little step at a time. Failure to improve only rarely relates to medication. Here are things to emphasize:
  • Your patient will most likely need to get more aggressive about increasing fluid and salt intake.
  • It is critical to get daily aerobic exercise. If the person is too tired to exercise, 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.
  • Cognitive behavioral therapy is very important
  • Family and friends supporting the recovery of normal function (as opposed to comfort with disability) is vital.

Practice Guidelines

There are no official practice guidelines, mostly because of a lack of comparative studies of treatment options. See Helpful Articles in the Resources section for supportive literature.

Diagnosis

Nearly all individuals with POTS have postural dizziness and chronic fatigue; about ⅔ of individuals also have headache and abdominal discomfort. 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. 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. POTS has not been associated with any early life features.
Diagnosis is based on demonstration of excessive postural tachycardia. While POTS research is based on 70-degree, head-up, tilt table testing, 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.”

Presentations

POTS patients typically look completely well. 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.
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 systolic blood pressure drops more than 20 mm Hg and/or the diastolic pressure drops >10 mmHg within the first 3 min of upright position, 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 and nausea (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 and Classifications

POTS is chronic (>3 months) orthostatic intolerance with tachycardia, with daily symptoms impairing normal function. For an adolescent, a sustained increase of more than 40 beats per minute when upright is “excessive.” Symptoms, such as tachycardia and dizziness, come on when upright and abate when recumbent.
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 usually does not require medications.
Prior to the onset of POTS, ⅔ of adolescent report a significant illness (e.g., mononucleosis) or injury (e.g., 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. For researchers, there are sometimes distinctions among categorizations of POTS – hyperadrenergic, post-infectious, and neuropathic. These distinctions are not associated with a different treatment or prognosis; categorizing individual patients offers no value.

Screening & Diagnostic Testing

No screening is recommended for asymptomatic individuals.
Testing is useful only when needed to confirm postural tachycardia, narrow the differential diagnosis, or evaluate for comorbidities. For patients with chronic fatigue and postural dizziness, determine the postural heart rate change with standing gravitational challenge or tilt table testing. With the patient resting calmly supine for at least a few minutes, measure the resting heart rate and blood pressure and measure them 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.

Laboratory Testing

No routine diagnostic labs. Test with CBC, thyroid studies, vitamin D levels, celiac screening, ESR, or additional tests only if needed to narrow the differential or determine any suspected comorbidities based on clinical suspicion.

Testing Family Members

Only if symptomatic. About 15% of those with POTS have a first-degree relative with a history consistent with POTS.

Genetics & Inheritance

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 linked to hypermobility and/or hypermobility syndromes.

Prevalence

POTS likely affects more than 1% of American adolescents. [Bhatia: 2016] 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. POTS almost never occurs prior to the onset of puberty (menarche in girls, growth spurt in boys).
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]

Differential Diagnosis

Differentiate between orthostatic intolerance in general and the subset of orthostatic intolerance that includes excessive postural tachycardia (POTS).
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.
Other causes of tachycardia, dizziness, and fatigue can include anemia, thyroid disorders, anxiety/depression, and effects of certain medications. Nausea and abdominal pain are common with POTS but can also be caused by sugar malabsorption, celiac disease, migraines, and inflammatory bowel disease. Median arcuate ligament syndrome is a rare cause of abdominal pain but has (rarely) been reported in association with POTS.
Severe headaches, common with POTS, are often caused by Headache (Migraine & Chronic) and other headache disorders. Also consider brain tumors, increased intracranial pressure, and vision disorders.

Co-Occurring Conditions

The accurate diagnosis of POTS does not preclude the concurrent diagnosis of a separate condition.
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 comorbidities 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].

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.

Treatment & Management

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.
Although detailed outcome data are not available, survey studies of POTS patients suggest that the majority of individuals 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] 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 (fludrocortisone, beta-blockers, antidepressants) may be very helpful for select patients. Of those who need medications, most can discontinue them after 1-3 years. 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. 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.
Approach to Treatment of 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.)
Care Process for POTS

Table 2 from Pediatric disorders of orthostatic intolerance [Stewart: 2018]
Click image for free access to table.
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 (perhaps as capsules or tablets). 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.

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.
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.

Medication

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 may not be 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.
  • Caffeine can stall recovery.
  • Antihistamines might slightly hinder recovery, so they should only be used if truly needed.
Medications to Manage Pots (Table 2 from Pediatric disorders of orthostatic intolerance [Stewart: 2018] -
Click image for free access to table.)

Medications Used in Managing POTS

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

Neurology

Expect a normal neurological exam. Increased sweating may be present in some individuals. In some centers, neurologists serve as the primary managers of POTS-specific situations. Neurologists may also be helpful in the management of otherwise recalcitrant Headache (Migraine & Chronic).

Gastro-Intestinal & Bowel Function

Some bloating may be present although the gastrointestinal exam is usually normal. 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.

Nutrition

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

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.

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.

Musculoskeletal

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

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. See Contraception & Menstrual Management.

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. See:

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. See Integrative Medicine for CYSHCN and Apps to Help Kids and Teens with Anxiety.

Services & Referrals

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.
Additional specialists can be helpful in diagnosis and management of aspects of care or comorbid conditions:
Pediatric Cardiology (see NW providers [0])
May be helpful in evaluation and management if sufficiently experienced with POTS and to rule out concerning cardiac causes of syncope and palpitations, if associated, or for specific cardiac concerns.
Pediatric Neurology (see NW providers [0])
May be helpful in evaluation if sufficiently experienced with POTS.
Physical Therapy (see NW providers [0])
May be helpful in designing and monitoring response to a reconditioning program and for evaluation and management of excessive pain. Some patients are more likely to continue daily exercise if they have a physical therapist or coach involved.
Behavioral Therapies (see NW providers [1])
Referral for cognitive behavioral therapy (CBT) is likely appropriate for all those diagnosed with POTS.
Individual Counseling (see NW providers [0])
Other mental health providers may be helpful for assessment and treatment of depression and/or anxiety, or if no CBT providers are available.
Pediatric Integrative Medicine (see NW providers [0])
May be helpful to direct components of management, including traditional and complementary modalities in a safe and evidence-based manner.
Pediatric Gastroenterology (see NW providers [1])
May be helpful in managing GI problems not responsive to primary care measures.

ICD-10 Coding

POTS has not been assigned a specific ICD-10 code; the following are commonly used:
  • FI49.8, 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.

Resources

Information & Support

For Professionals

Postural Orthostatic Tachycardia Syndrome (GARD)
Includes information about symptoms, inheritance, diagnosis, finding a specialist, related diseases, and support organizations; Genetic and Rare Diseases Information Center of the National Center for Advancing Translational Sciences.

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.

Patient Education

POTS (Postural Orthostatic Tachycardia Syndrome) (FAQ)
Answers to questions that families may have about POTS.

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

Tools

Table of Medications to Manage POTS
Medication, Dosing, Side Effects, and comments; from 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

Services for Patients & Families Nationwide (NW)

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.

Studies

Clinical Studies of POTS (clincaltrials.gov)
Studies looking at better understanding, diagnosing, and treating this condition; from the National Library of Medicine.

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.

Authors & Reviewers

Initial publication: November 2019; last update/revision: January 2020
Current Authors and Reviewers:
Author: Phil Fischer, MD
Reviewer: Gisela G. Chelimsky, MD

Page 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

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