Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

all patients

1st line – avoidance of triggers

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ACUTE
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all patients
1st line – 

avoidance of triggers

A multidisciplinary approach is important to manage the widespread symptoms of POTS.[4]

Advise the patient to avoid triggers for POTS symptoms.[4]​ These triggers include:​

  • Exposure to excessive heat[2]

  • Prolonged standing[2]

  • Medications that exacerbate intravascular depletion (e.g., diuretics), afterload reduction (e.g., ACE inhibitors, angiotensin-II receptor antagonists), or sinus and orthostatic tachycardia (e.g., norepinephrine reuptake inhibitors such as atomoxetine)[1][2][4]

  • Excessive alcohol intake and exercise[1][2]

  • Recreational drugs (e.g., cocaine, methamphetamines).[61]

Plus – lifestyle modifications and management of comorbidities

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ACUTE
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all patients
Plus – 

lifestyle modifications and management of comorbidities

Treatment recommended for ALL patients in selected patient group

In addition to avoiding triggers, advise the patient to make the following lifestyle modifications:

  • Increase intake of water and salt to optimize intravascular volume.[1][2][4][59]​ Advise the patient to aim to consume at least 2 to 3 liters of water and up to 10 g of salt every day (1 teaspoon of salt is approximately equivalent to 2.3 g).[1][2][4][59]​​ However, in practice, increased salt intake is an inappropriate treatment for certain patients, such as those with hypertension, or kidney or cardiovascular disease.

  • Use waist-high compression stockings with an abdominal binder to reduce venous pooling in their lower extremities and splanchnic circulation.[1][2][60]​ If this is not tolerated, the patient could use waist-high compressions without abdominal binder or an abdominal binder alone.[1]​​[4][62]

  • Undertake graded exercise training.[1][2][4] Advise the patient to start with supine progressive aerobic and leg resistance training then progress to upright exercises (e.g., rowing machines and static exercise bikes).[1][2] However, be aware that some patients won’t tolerate graded exercise training until pharmacologic therapy has been started and adequate symptom control is achieved.[63]​ In practice, if a patient can’t tolerate graded exercise training as a first-line nonpharmacologic approach, reattempt this after starting pharmacologic treatment.

  • Physical counterpressure techniques (in children).[2]

Ensure any comorbidities are managed appropriately.[4] Treat any additional features, such as poor mental health, disturbed sleep, and headache, to improve the patient’s overall level of functioning and quality of life.

2nd line – pharmacologic treatment

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ACUTE
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all patients
2nd line – 

pharmacologic treatment

A multidisciplinary approach is important to manage the widespread symptoms of POTS.[4]

Start pharmacologic treatment if the patient’s symptoms persist despite nonpharmacologic measures.

  • Be aware that there are no medications that have been approved for the treatment of POTS.

  • Medication can be given as monotherapy, but a combination of medications is usually required.

  • In pregnancy, avoid pharmacologic therapy where possible and check the safety of specific drugs carefully.[53]​ In particular, note that ivabradine is not recommended in pregnancy as it may cause fetal harm.

  • It is reasonable to treat children with the same interventions that are recommended for adults, with a focus on promoting salt and fluid intake, physical counterpressure techniques, and reassurance to the patient and their families.[2] Pharmacologic treatment may be used. However, erythropoietin, modafinil, octreotide, and beta-blockers are only used on rare occasions under specialist guidance.

  • Non-orthostatic symptoms (e.g., gastrointestinal symptoms) do not usually improve with these pharmacologic treatments, so further symptomatic relief and involvement of other specialties as appropriate may also be required in practice.

Target pharmacologic treatment to manage the patient’s specific symptoms associated with POTS.[2] These include the following:

Intravascular volume expansion:

  • Options include fludrocortisone or desmopressin.​[2][4][64]​​[65][66]

  • Note that evidence for efficacy of fludrocortisone for POTS is limited, but some trials have shown it can be effective for vasovagal syncope.[1][2][64]​ Ensure that patients are on a high sodium diet and that their potassium level is monitored.[1] Pharmacodynamic effects of fludrocortisone may only last 1 to 2 days.[2]

  • A small randomized crossover study showed that desmopressin improved tachycardia and POTS symptoms in adult patients.[66]

Reduction of peripheral venous pooling:

  • Midodrine is an option.[2][56][57]​ Some small studies have shown benefit in patients with POTS, particularly if they have neuropathic POTS, and also for vasovagal syncope.[56][57][58]​​

Exertion of a negative chronotropic effect and lowering of heart rate:

  • Options include beta-blockers or ivabradine.[1][2][4][67]​​ These drugs are preferred in patients with hyperadrenergic POTS. 

  • A recent clinical trial found no difference in efficacy between the beta-blockers propranolol and bisoprolol.[68]​ In practice, labetalol is generally avoided as a standard treatment for POTS, except for patients with hyperadrenergic POTS. Labetalol is safe to use in pregnancy. 

  • Some evidence shows that ivabradine is an effective treatment for POTS, and lowers heart rate without affecting blood pressure.[54][69]​ However, note that it is not recommended in pregnancy as it may cause fetal harm.

Neuroenhancement:

  • Pyridostigmine is an option.[2][70][71]​​ In practice, this may also be useful if the patient is experiencing constipation as a predominant non-orthostatic symptom of POTS.

Treatment of prominent hyperadrenergic features:

  • Clonidine is an option.[2][4]

Treatment of fatigue and cognitive dysfunction:

  • Modafinil is an option.[2]

These drug options may be used alone or in combination depending on the clinical presentation. Concomitant use of beta-blockers and clonidine is usually avoided. If these drugs are used together, monitor heart rate and blood pressure.

Primary options

fludrocortisone: children: consult specialist for guidance on dose; adults: 0.1 to 0.2 mg orally once daily

or

desmopressin: children: consult specialist for guidance on dose; adults: 0.1 to 0.2 mg orally once daily

-- AND / OR --

midodrine: children: consult specialist for guidance on dose; adults: 2.5 to 15 mg orally once to three times daily

-- AND / OR --

bisoprolol: children: consult specialist for guidance on dose; adults: 2.5 to 5 mg orally once daily

or

propranolol hydrochloride: children: consult specialist for guidance on dose; adults: 10-20 mg orally (immediate-release) up to four times daily

or

labetalol: children: consult specialist for guidance on dose; adults: 100-200 mg orally twice daily

or

ivabradine: children: consult specialist for guidance on dose; adults: 2.5 to 7.5 mg orally twice daily

-- AND / OR --

pyridostigmine: children: consult specialist for guidance on dose; adults: 30-60 mg orally once to three times daily

-- AND / OR --

clonidine: children: consult specialist for guidance on dose; adults: 0.1 to 0.3 mg orally twice daily

-- AND / OR --

modafinil: children: consult specialist for guidance on dose; adults: 50-200 mg orally once or twice daily

Plus – avoidance of triggers

Back
ACUTE
|
all patients
Plus – 

avoidance of triggers

Treatment recommended for ALL patients in selected patient group

Advise the patient to avoid triggers for POTS symptoms.[4]​ These triggers include:​

  • Exposure to excessive heat[2]

  • Prolonged standing[2]

  • Medications that exacerbate intravascular depletion (e.g., diuretics), afterload reduction (e.g., ACE inhibitors, angiotensin-II receptor antagonists), or sinus and orthostatic tachycardia (e.g., norepinephrine reuptake inhibitors such as atomoxetine)[1][2][4]

  • Excessive alcohol intake and exercise[1][2]

  • Recreational drugs (e.g., cocaine, methamphetamines).[61]

Plus – lifestyle modifications and management of comorbidities

Back
ACUTE
|
all patients
Plus – 

lifestyle modifications and management of comorbidities

Treatment recommended for ALL patients in selected patient group

In addition to avoiding triggers, advise the patient to make the following lifestyle modifications:

  • Increase intake of water and salt to optimize intravascular volume.[1][2][4][59]​ Advise the patient to aim to consume at least 2 to 3 liters of water and up to 10 g of salt every day (1 teaspoon of salt is approximately equivalent to 2.3 g).[1][2][4][59]​​ However, in practice, increased salt intake is an inappropriate treatment for certain patients, such as those with hypertension, or kidney or cardiovascular disease.

  • Use waist-high compression stockings with an abdominal binder to reduce venous pooling in their lower extremities and splanchnic circulation.[1][2][60]​ If this is not tolerated, the patient could use waist-high compressions without abdominal binder or an abdominal binder alone.[1]​​[4][62]​​

  • Undertake graded exercise training.[1][2][4] Advise the patient to start with supine progressive aerobic and leg resistance training then progress to upright exercises (e.g., rowing machines and static exercise bikes).[1][2] However, be aware that some patients won’t tolerate graded exercise training until pharmacologic therapy has been started and adequate symptom control is achieved.[63]​ In practice, if a patient can’t tolerate graded exercise training as a first-line nonpharmacologic approach, reattempt this after starting pharmacologic treatment.

  • Physical counterpressure techniques (in children).[2]

Ensure any comorbidities are managed appropriately.[4] Treat any additional features, such as poor mental health, disturbed sleep, and headache, to improve the patient’s overall level of functioning and quality of life.

3rd line – salvage therapy

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ACUTE
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all patients
3rd line – 

salvage therapy

A multidisciplinary approach is important to manage the widespread symptoms of POTS.[4]

If a patient’s symptoms do not improve with initial pharmacotherapy, they may benefit from adding one of the following treatments for salvage therapy:

  • Bupropion (but be aware that this should not be used in children because it can increase the risk of suicidal thoughts)[72]

  • Erythropoietin (epoetin alfa) (only used on rare occasions in children and under specialist guidance)[73][74]

  • Octreotide (only used on rare occasions in children and under specialist guidance)[74][75]

  • Droxidopa (not recommended in children)[74][76]

  • Intermittent intravenous saline infusion.[2][4][77]​​ Intravenous saline may be useful for patients who are clinically decompensated with worsening symptoms.[2] However, regular or long-term infusions are not recommended, because insertion of a chronic central venous catheter is usually required, which puts the patient at risk of catheter-related complications.[2]

Patients should continue on their initial pharmacologic treatment (see above).

Primary options

bupropion hydrochloride: adults: 150-300 mg orally (extended-release) once daily

OR

epoetin alfa: children: consult specialist for guidance on dose; adults: 10,000 to 20,000 units subcutaneously once weekly

OR

octreotide: children: consult specialist for guidance on dose; adults: 50-200 micrograms subcutaneously three times daily

OR

droxidopa: adults: 100-600 mg orally three times daily

Plus – avoidance of triggers

Back
ACUTE
|
all patients
Plus – 

avoidance of triggers

Treatment recommended for ALL patients in selected patient group

Advise the patient to avoid triggers for POTS symptoms.[4]​ These triggers include:​

  • Exposure to excessive heat[2]

  • Prolonged standing[2]

  • Medications that exacerbate intravascular depletion (e.g., diuretics), afterload reduction (e.g., ACE inhibitors, angiotensin-II receptor antagonists), or sinus and orthostatic tachycardia (e.g., norepinephrine reuptake inhibitors such as atomoxetine)[1][2][4]

  • Excessive alcohol intake and exercise[1][2]

  • Recreational drugs (e.g., cocaine, methamphetamines).[61]

Plus – lifestyle modifications and management of comorbidities

Back
ACUTE
|
all patients
Plus – 

lifestyle modifications and management of comorbidities

Treatment recommended for ALL patients in selected patient group

In addition to avoiding triggers, advise the patient to make the following lifestyle modifications:

  • Increase intake of water and salt to optimize intravascular volume.[1][2][4][59]​ Advise the patient to aim to consume at least 2 to 3 liters of water and up to 10 g of salt every day (1 teaspoon of salt is approximately equivalent to 2.3 g).[1][2][4][59]​​ However, in practice, increased salt intake is an inappropriate treatment for certain patients, such as those with hypertension, or kidney or cardiovascular disease.

  • Use waist-high compression stockings with an abdominal binder to reduce venous pooling in their lower extremities and splanchnic circulation.[1][2][60]​ If this is not tolerated, the patient could use waist-high compressions without abdominal binder or an abdominal binder alone.[1]​​[4][62]​​

  • Undertake graded exercise training.[1][2][4] Advise the patient to start with supine progressive aerobic and leg resistance training then progress to upright exercises (e.g., rowing machines and static exercise bikes).[1][2] However, be aware that some patients won’t tolerate graded exercise training until pharmacologic therapy has been started and adequate symptom control is achieved.[63]​ In practice, if a patient can’t tolerate graded exercise training as a first-line nonpharmacologic approach, reattempt this after starting pharmacologic treatment.

  • Physical counterpressure techniques (in children).[2]

Ensure any comorbidities are managed appropriately.[4] Treat any additional features, such as poor mental health, disturbed sleep, and headache, to improve the patient’s overall level of functioning and quality of life.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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