Approach
The goal of therapeutic interventions for POTS is to improve symptoms by:[1]
Expanding intravascular volume
Reducing peripheral venous pooling
Exerting a negative chronotropic effect and lowering heart rate.
A multidisciplinary approach is important to manage the widespread symptoms of POTS.[4]
Avoidance of triggers and lifestyle modifications is the first management step for all patients, before pharmacological therapy if symptoms persist.[1][2] Note that these treatments are for management of orthostatic symptoms of POTS. Offer symptomatic relief of any associated non-orthostatic symptoms (e.g., gastrointestinal symptoms) and involve other specialties as necessary, because these will not usually respond to the standard treatments for POTS.
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] However, erythropoietin, modafinil, octreotide, and beta-blockers are only used on rare occasions under specialist guidance, and bupropion and droxidopa are not recommended in children.
In pregnancy, avoid pharmacological therapy where possible and check the safety of specific drugs carefully.[52] In particular, note that ivabradine is not recommended in pregnancy as it may cause fetal harm.
Managing the symptoms of POTS is key because they can significantly compromise a patient’s quality of life, and negatively impact their ability to advance in their education and career.[1][8] In general, there is a lack of medium- or long-term evidence for effective treatments for patients with POTS, particularly children, and managing symptoms can be challenging.[2][4] However, some evidence is emerging for certain treatments, particularly ivabradine, increasing salt intake, and compression.[53][54][55][56][57][58][59]
Avoidance of triggers
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., noradrenaline reuptake inhibitors such as atomoxetine)[1][2][4]
Recreational drugs (e.g., cocaine, methamphetamines).[60]
Lifestyle modifications and management of comorbidities
In addition to avoiding triggers, advise the patient to make the following lifestyle modifications:
Increase intake of water and salt to optimise intravascular volume.[1][2][4][58]
Advise the patient to aim to consume at least 2 to 3 litres 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][58] 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][59]
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 pharmacological therapy has been started and adequate symptom control is achieved.[62] In practice, if a patient can’t tolerate graded exercise training as a first-line non-pharmacological approach, reattempt this after starting pharmacological 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.
Initial pharmacological treatment
Start pharmacological treatment if the patient’s symptoms persist despite non-pharmacological 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. Concomitant use of beta-blockers and clonidine is usually avoided. If these drugs are used together, monitor heart rate and blood pressure.
Non-orthostatic symptoms (e.g., gastrointestinal symptoms) do not usually improve with these pharmacological treatments, so further symptomatic relief and involvement of other specialties as appropriate may also be required in practice.
Target pharmacological treatment to manage the patient’s specific symptoms associated with POTS.[2] These include:
Intravascular volume expansion:
Options include fludrocortisone or desmopressin.[2][4][63][64][65]
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][63] 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 randomised crossover study showed that desmopressin improved tachycardia and POTS symptoms in adult patients.[65]
Reduction of peripheral venous pooling:
Exertion of a negative chronotropic effect and lowering of heart rate:
Options include beta-blockers or ivabradine.[1][2][4][66] 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.[67] 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.[53][68] However, note that it is not recommended in pregnancy as it may cause fetal harm.
Neuroenhancement:
Treatment of prominent hyperadrenergic features:
Treatment of fatigue and cognitive dysfunction:
Modafinil is an option.[2]
Salvage therapy for refractory symptoms
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)[71]
Intermittent intravenous saline infusion[2][4][76]
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]
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