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 prevalence of POTS has increased after COVID-19 with 2%-14% of COVID-19 survivors developing POTS and 9%-61% experiencing POTS-like symptoms. While evaluating tired, dizzy, uncomfortable adolescents, consider the diagnosis of postural orthostatic tachycardia syndrome and/or orthostatic intolerance and 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.

POTS can be especially challenging for young athletes used to strenuous physical activity. Managing expectations in this particular population can go a long way in treatment and recovery.

Other Names

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

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 for patients 12-19 years and >30 beats per minute increase for patients over 19 years) within 10 minutes of upright posture. See Diagnosis below for specific criteria. 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 no excessive postural tachycardia. Please see Diagnosis section for detailed criteria.

Management of POTS symptoms
Advise patients to drink so much that their urine looks clear, like water. Eat as much salt as tolerated; some patients prefer salt tablets/capsules. Get aerobic exercise daily (working up to 30 minutes in a single daily session). Get enough sleep every night and do not take naps. If this is not possible, limit naps to once a day and no longer than 30 minutes - avoid napping after 2 pm. Get help from a good psychologist and consider medication, too. Please see Treatment and Management Section for more information.

Importance of healthy lifestyle
Patients with POTS are further compromised when they get behind on exercise, regular meals, sleep, and other healthy habits that help with stress management and weight gain. 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.

Physical therapy
Working with a physical therapist who is knowledgeable in POTS can be very beneficial. Also, adjusting expectations in athletes is important so they do not set themselves up to fail by having high standards for physical activity. If there is joint hypermobility, work with a physical therapist familiar with hypermobility to teach patients how to strengthen and protect their joints. Please see the Exercise Section below to find more details on an Exercise Program for patients with POTS.

Importance of healthy nutrition
Iron deficiency and vitamin D deficiencies can worsen POTS symptoms. A balanced diet and taking a daily multivitamin can be helpful. Evaluating for nutritional deficiencies can help guide management if there are concerns about poor diet. If iron deficiency is suspected, an actual treatment dose would be indicated (daily multivitamins might not have enough iron to treat iron deficiency).

Infections and POTS
Patients with POTS tend to get sicker with common illnesses than others. Patients with POTS feel terrible in just about every way with just about any symptom. It is vitally important that they adhere to and even exaggerate their baseline fluid/salt intake and exercise/activity plan when they are ill.

Failure to improve
Patients need to keep going forward, 1 little step at a time. Failure to improve only rarely relates to medication. Here are things to emphasize:

• Become more aggressive about increasing fluid and salt intake.
• Focus on 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 breathes a bit faster than normal. Then, increase the duration of that daily exercise by 1-2 minutes every 5 days. Gradually, exercise tolerance will increase. Keep going until the patient can continue with 30 minutes of aerobic exercise daily.
• Participating in cognitive behavioral therapy is very important.

Family and friends supporting the recovery of normal function (as opposed to comfort with disability) is vital.

Mostly because of a lack of comparative studies of treatment options, there are no official Practice Guidelines for POTS. Below is a summary of what is known so far about pediatric POTS:

Boris JR, Moak JP.
Pediatric Postural Orthostatic Tachycardia Syndrome: Where We Stand.
Pediatrics. 2022;150(1). PubMed abstract / Full Text

See Helpful Articles in the Resources section for supportive literature.

• Sustained heart rate increase of ≥ 30 beats/min (or ≥ 40 beats/min if patient is 12–19 yr within 10 minutes of upright posture.
• Absence of significant orthostatic hypotension (blood pressure drop ≥ 20/10 mm Hg).
• Very frequent symptoms of orthostatic intolerance that are worse while upright, with rapid improvement upon return to a supine position. Symptoms vary among individuals but often include lightheadedness, palpitations, tremulousness, generalized weakness, blurred vision, and fatigue.
• Symptom duration ≥ 3 months.
• Absence of other conditions that could explain sinus tachycardia on standing
  • Acute hypovolemia (from dehydration or blood loss)
  • Anemia
  • Orthostatic hypotension
  • Endocrinopathy
  • Adrenal insufficiency
  • Carcinoid tumor
  • Hyperthyroidism
  • Pheochromocytoma
  • Adverse effects from medication or polypharmacy.
  • Panic attacks and severe anxiety
  • Prolonged or sustained bed rest
  • Recreational drug effects

History

Family History

Presentations

Figure 1 from Pediatric Postural Orthostatic Tachycardia Syndrome: Where We Stand (AAP)

Diagnostic Criteria and Classifications

Genetics & Inheritance

Screening & Diagnostic Testing

Genetics & Inheritance

Prevalence

Differential Diagnosis

Co-Occurring Conditions

There are limited data about the 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 a good recovery for most affected patients is warranted.

Instruct patients to increase fluid and salt intake, exercise daily, get adequate sleep, and engage in cognitive behavioral therapy techniques. Prescription medications are most useful when the patient fully engages in non-pharmacologic treatment modalities. [Lai: 2009]

In 1 study, nearly 90% recovered or greatly improved over a few years. However, some patients do not fully recover. [Bhatia: 2016] Management of individuals with POTS requires patience, persistence, and positivity.

Non-pharmacologic treatment (fluids, salt, exercise, compression garments, elevating the head of bed, therapy) may be sufficient and should be used for all patients.

Pharmacologic treatment (fludrocortisone, beta-blockers, antidepressants) may be very helpful for select patients. Most of those needing medications 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 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 unfamiliar with POTS, 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. Some institutions have multi-disciplinary teams for POTS so patients can see a POTS-aware physician, a psychologist, and a physical therapist on the same day. It prevents repeated hospital visits and bills and allows for care coordination.

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.)

Mental Health / Behavior

Medication

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

Neurology

Gastro-Intestinal & Bowel Function

Nutrition

Endocrine/Metabolism

Cardiology

Musculoskeletal

Maturation/Sexual/Reproductive

Sleep

Education & School

Complementary & Alternative Medicine

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, the involvement of a POTS-aware physician can help – be that a generalist, a neurologist, or a cardiologist.

When to refer a patient with POTS

• No improvement noted with non-pharmacologic measures
• If the primary care clinician is not comfortable prescribing medications for POTS, or the patient does not improve with first-line medications like beta-blockers, or after targeting specific issues like anxiety and depression with SSRIs
• If symptoms are worsening
• If there are associated comorbidities complicating diagnostic picture and management

The Center for Autonomic Dysfunction and Research is a multi-disciplinary clinic for patients with POTS and dysautonomia. The team involves POTS-aware physicians, psychologists, and physical therapists.

Additional specialists can be helpful in the diagnosis and management of various aspects of care or comorbid conditions:

Pediatric Cardiology (see RI providers [17])
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 RI providers [18])
May be helpful in evaluation if sufficiently experienced with POTS.

Physical Therapy (see RI providers [6])
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 RI providers [32])
Referral for cognitive behavioral therapy (CBT) is likely appropriate for all those diagnosed with POTS.

Individual Counseling (see RI providers [36])
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 RI 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 RI providers [18])
May be helpful in managing GI problems not responsive to primary care measures.

G90. A is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. ICD-10-CM G90. A is a new 2023 ICD-10-CM code that became effective on October 1, 2022.