History and exam

Key diagnostic factors

common

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: aged 15 to 25 years; female sex; recent viral infection; pregnancy.[1][2][16][18]​ Also consider POTS if your patient has relevant associated comorbidities, such as: migraine headaches; irritable bowel syndrome; Ehlers-Danlos syndrome; chronic fatigue syndrome; or autoimmune diseases (particularly Hashimoto's thyroiditis and coeliac disease).[1]

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 aged 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 drugs (e.g., beta-blockers, ivabradine). For a diagnosis of POTS, orthostatic symptoms should occur frequently and have lasted for at least 3 months.[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] ​Post-exercise 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 generalised weakness

Generalised 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]

dyspnoea

A common non-orthostatic feature of POTS, which is mostly associated with dysfunctional breathing.[32]​ Cohort studies suggest that dyspnoea 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 coeliac 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 coeliac disease.​ For a diagnosis of POTS, symptoms should have lasted for at least 3 months.[1]​

diarrhoea

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 coeliac 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 coeliac 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 coeliac 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 aged 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: migraine headaches (40%); irritable bowel syndrome (30%); Ehlers-Danlos syndrome (25%); chronic fatigue syndrome (21%); and autoimmune diseases (20%), particularly Hashimoto's thyroiditis and coeliac disease.[1][4]​

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