History and exam

Key diagnostic factors

common

orthostatic tachycardia

Check the patient’s heart rate and blood pressure while they are supine and then standing.​[2] This is known as the 10-minute standing test.​​

  • The patient’s heart rate will typically increase by ≥30 bpm (or ≥40 bpm in patients ages 12 to 19 years old) after changing position from supine to standing, with no orthostatic hypotension (sustained drop in systolic blood pressure by ≥20 mmHg).[1]​​[2][3]​​​[4]

  • Allow at least 5 minutes of a supine position and at least 1 minute of standing before checking orthostatic vital signs.[40]​ If there is no significant variation in the patient’s heart rate after 1 minute of standing, repeat the standing heart rate and blood pressure check at 3, 5, and 10 minutes.[40]

  • Note that changes in heart rate are often not apparent after 1 minute of standing in practice. A common mistake is to focus on the changes in heart rate after 1 minute of standing only, rather than the full 10 minutes, which can lead to misdiagnosis.

  • Be aware that some patients have hyperadrenergic POTS, and have increased sympathetic response and excess circulating catecholamine.[1][2]​​ Patients with hyperadrenergic POTS will have orthostatic hypertension (increase in systolic blood pressure ≥10 mmHg after standing for 10 minutes).​[2]

orthostatic palpitations

A key orthostatic symptom of POTS.[1][2][3]​​​​[4]​ Orthostatic symptoms of POTS occur when standing and improve rapidly when the patient returns to a supine position.[1][2] A patient with POTS may report palpitations even if they have a normal heart rate, particularly if they are taking negative chronotropic medications (e.g., beta-blockers, ivabradine). For a diagnosis of POTS, orthostatic symptoms should occur frequently and have lasted for at least 3 months.[1]

presence of risk factors

Consider POTS if a patient presents with relevant symptoms and signs and particularly if they have any of the following risk factors:

  • Age 15 to 25 years​[2]

  • Female sex[1][2]

  • Recent viral infection[16]

  • Pregnancy[18]

  • Relevant associated comorbidities, such as migraine headaches, irritable bowel syndrome, Ehlers-Danlos syndrome, chronic fatigue syndrome, or autoimmune diseases (particularly Hashimoto thyroiditis and celiac disease).[1]

Other diagnostic factors

common

orthostatic lightheadedness

An orthostatic symptom of POTS.[1][3]​​​​[4]​ It occurs due to rapid heart rate and hypoperfusion on standing. Orthostatic symptoms of POTS occur when standing and improve rapidly when the patient returns to a supine position.[1][2] For a diagnosis of POTS, the patient should also have an increase in heart rate by ≥30 bpm within 10 minutes of standing from a supine position or head-up tilt, without orthostatic hypotension, and orthostatic symptoms should occur frequently and have lasted for at least 3 months.[1][2]​​​​​​[4]

orthostatic blurred vision

An orthostatic symptom of POTS.[1] Orthostatic symptoms of POTS occur when standing and improve rapidly when the patient returns to a supine position.[1][2] For a diagnosis of POTS, the patient should also have an increase in heart rate by ≥30 bpm within 10 minutes of standing from a supine position or head-up tilt, without orthostatic hypotension, and orthostatic symptoms should occur frequently and have lasted for at least 3 months.[1][2]​​​​​​[4]

exercise intolerance

Can be an orthostatic or non-orthostatic feature of POTS.[1][2]​​​​​[4]​ Postexercise malaise is a frequently reported symptom. Orthostatic symptoms of POTS occur when standing and improve rapidly when the patient returns to a supine position.[1][2] For a diagnosis of POTS, the patient should also have an increase in heart rate by ≥30 bpm within 10 minutes of standing from a supine position or head-up tilt, without orthostatic hypotension, and orthostatic symptoms should occur frequently and have lasted for at least 3 months.[1][2]​​​​​​[4]

orthostatic presyncope

Presyncope is an orthostatic symptom of POTS.​[2] It occurs due to rapid heart rate and hypoperfusion while standing. Orthostatic symptoms of POTS occur when standing and improve rapidly when the patient returns to a supine position.[1][2] For a diagnosis of POTS, the patient should also have an increase in heart rate by ≥30 bpm within 10 minutes of standing from a supine position or head-up tilt, without orthostatic hypotension, and orthostatic symptoms should occur frequently and have lasted for at least 3 months.[1][2]​​​​​​[4]

orthostatic tremor

Tremor is an orthostatic symptom of POTS.[1]​​[2]​​​​​​​​[4]​ Orthostatic symptoms of POTS occur when standing and improve rapidly when the patient returns to a supine position.[1][2] For a diagnosis of POTS, the patient should also have an increase in heart rate by ≥30 bpm within 10 minutes of standing from a supine position or head-up tilt, without orthostatic hypotension, and orthostatic symptoms should occur frequently and have lasted for at least 3 months.[1][2]​​​​​​​[4]

orthostatic generalized weakness

Generalized weakness is an orthostatic symptom of POTS.[1][2] Orthostatic symptoms of POTS occur when standing and improve rapidly when the patient returns to a supine position.[1][2] For a diagnosis of POTS, the patient should also have an increase in heart rate by ≥30 bpm within 10 minutes of standing from a supine position or head-up tilt, without orthostatic hypotension, and orthostatic symptoms should occur frequently and have lasted for at least 3 months.[1][2]​​​​​​​[4]

dyspnea

A common non-orthostatic feature of POTS, which is mostly associated with dysfunctional breathing.[32]​ Cohort studies suggest that dyspnea may be present in at least 65% of patients with POTS.[11][33]

uncommon

orthostatic syncope

Syncope is an orthostatic symptom of POTS.​[2] It may occur due to excessive tachycardia and transient hypoperfusion while standing. Orthostatic symptoms of POTS occur when standing and improve rapidly when the patient returns to a supine position.[1][2] For a diagnosis of POTS, the patient should also have an increase in heart rate by ≥30 bpm within 10 minutes of standing from a supine position or head-up tilt, without orthostatic hypotension, and orthostatic symptoms should occur frequently and have lasted for at least 3 months.[1][2]​​​​​​[4]

bloating

A gastrointestinal feature of POTS.​[2][3]​​​​​​​[4] Gastrointestinal symptoms are occasionally reported by people with POTS and may be due to autonomic dysfunction, or associated irritable bowel disease or celiac disease.[34]​ For a diagnosis of POTS, symptoms should have lasted for at least 3 months.[1]

nausea

A gastrointestinal feature of POTS, which can be accompanied by fainting and migraines.​[2][3]​​​​​​​​[4] Gastrointestinal symptoms are occasionally reported by people with POTS and may be due to autonomic dysfunction, or associated irritable bowel disease or celiac disease. For a diagnosis of POTS, symptoms should have lasted for at least 3 months.[1]

diarrhea

A gastrointestinal feature of POTS.​[2][3]​​​​​​​​[4] Gastrointestinal symptoms are occasionally reported by people with POTS and may be due to autonomic dysfunction, or associated irritable bowel disease or celiac disease. For a diagnosis of POTS, symptoms should have lasted for at least 3 months.[1]

constipation

A gastrointestinal feature of POTS.​​​​​​​[4]​​​[34]​​ Gastrointestinal symptoms are occasionally reported by people with POTS and may be due to autonomic dysfunction, or associated irritable bowel disease or celiac disease. For a diagnosis of POTS, symptoms should have lasted for at least 3 months.[1]

abdominal pain

A gastrointestinal feature of POTS.​[2][3]​​​​​​​​[4] Gastrointestinal symptoms are occasionally reported by people with POTS and may be due to autonomic dysfunction, or associated irritable bowel disease or celiac disease. For a diagnosis of POTS, symptoms should have lasted for at least 3 months.[1]

fatigue

Can be an orthostatic or non-orthostatic feature of POTS.[1][2][3]​​​​​​​​[4]​ The patient may describe muscle fatigue and low energy. Orthostatic symptoms of POTS occur when standing and improve rapidly when the patient returns to a supine position.[1][2]​ For a diagnosis of POTS, symptoms should have lasted for at least 3 months.[1]

headache

A non-orthostatic symptom of POTS.[1][2][3]​​​​​​​​[4]​ For a diagnosis of POTS, symptoms should have lasted for at least 3 months.[1]

sleep disturbance

A non-orthostatic symptom of POTS.​[2][3]​​​​​​​​[4] For a diagnosis of POTS, symptoms should have lasted for at least 3 months.[1]

cognitive impairment

A non-orthostatic symptom of POTS.[1]​​​​​​​​​​[4]​ For a diagnosis of POTS, symptoms should have lasted for at least 3 months.[1]

chest pain

Can be an orthostatic or non-orthostatic symptom of POTS.[1]​​​​​​​​​[4] For a diagnosis of POTS, symptoms should have lasted for at least 3 months.[1]

bladder disturbance

Can be a non-orthostatic feature of POTS.[35][36]​​ Symptoms can include nocturia and urgency.[36]

Risk factors

strong

young age (15 to 25 years)

Most patients who present with POTS are typically young women ages 15 to 25 years.[2][10]​​​ However, POTS can occur outside of this age range, although new onset POTS is rare after age 50.[2]​ Patients <12 years may present with similar symptoms to older patients, particularly if there is a family history of POTS.[1]​ However, POTS remains undefined in patients <12 years and the sensitivity and specificity of the heart rate criteria in this age group is not established.[1]

female sex

POTS is more common in women than men and has a female to male ratio of 4:1.[1][2]

recent viral infection

Many patients with POTS report recent febrile illness, which suggests an autoimmune process may be responsible for causing orthostatic symptoms.[1]

Pathogens commonly linked to POTS include SARS-CoV-2 and Epstein Barr virus.[16]

pregnancy

Hormonal changes during pregnancy affect peripheral vascular resistance and blood volume, which may trigger orthostatic intolerance, particularly early in pregnancy (i.e., the first trimester).[18]

associated comorbidities

The most commonly associated comorbidities are:[1]

  • Migraine headaches (40%)[4]

  • Irritable bowel syndrome (30%)

  • Ehlers-Danlos syndrome (25%)[4]

  • Chronic fatigue syndrome (21%)[4]

  • Autoimmune diseases (20%), particularly Hashimoto thyroiditis and celiac disease.

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