Postural Orthostatic Tachycardia Syndrome (PoTS)

Overview

The following guide on PoTS has been put together by the Remedy team with support from local cardiologists in BNSSG.

Local cardiologists advise that PoTS should not be considered a `heart condition`  but is on the spectrum of (usually temporary) autonomic dysfunction (such as Vasovagal Syncope and it is also a comorbidity in a significant proportion of cases of ME/CFS). Once other causes of symptoms have been excluded, which can usually be done in primary care,  then reassuring patients to that effect is very important. Most patients can be then managed in primary care as described below, with specialist involvement reserved for diagnositic uncertainty or consideration of second line medication.

Postural Orthostatic Tachycardia Syndrome was first defined in the adult population as an increase in heart rate by more than 30 bpm or an increase to heart rate greater than 120 bpm within 10 minutes when moving from supine to the upright position (1).

Epidemiology

• Female sex - prevalance in females predominate over males by 5:1
• Mean age of onset in adults is about 30 years and most patients are between the ages of 20-40 years.

Clinical features

• development of tachycardia and other symptoms during upright position, and relieved by recumbence, are central features of this syndrome complex 
• symptoms always include dizziness and light-headedness
• frank syncope can occur at times, although not a predominant feature
• other possible associated symptoms include:
  • headache, tunnel vision, fatigue, neurocognitive impairment, exercise intolerance, weakness, dyspnea, tremulousness, nausea, chest or abdominal pain, sweating, anxiety, and palpitations
• intensity and frequency of symptoms is often variable and in some instances may occur and persist even when the patient is supine

Secondary causes

Further information including possible secondary causes can be found here.(2)

Prognosis

Although long-term prognosis of PoTS is poorly explored, around 50% of patients spontaneously recover within 1-3 years (3).

Who to Refer

Many patients can be investigated, diagnosed and managed in primary care and do not need referral.

Referral to a cardiologist should be considered in patients with diagnositic uncertainty or significant ongoing symptoms despite appropriate lifestyle measures / management in primary care.  

What to do before referral

Examination

• Pulse: the diagnostic feature is a sustained increase in pulse rate on standing. (See Active Stand test below)
• Blood pressure: usually normal or increased.
• Peripheral circulation: there is often a dark red-blue discolouration of the lower extremities on standing and the feet are cold. This is called dependent acrocyanosis.

Active Stand test

This test can be done by the patient in their own home or in primary care using a pulse oximeter or blood pressure machine. It takes about 10- 15 minutes to complete. Warn the patient that they may feel light headed and should be accompanied when they do the test. They should sit or lie down if they think they are going to fall or lose consciousness.

• Lay on your back for 5 minutes and be as still as possible.
• While still laying down, take the pulse (and blood pressure if possible) and write it down.
• Stand up and remain still without leaning for 2 minutes and then take the pulse (and BP) while still standing. 
• If there is no significant change in pulse, continue standing and take the pulse (and BP) at 5 and 10 minutes. 
• If the heart rate is >120 at any point while standing or if there is an increase in pulse of 30 beats per minute (adults) or 40 beats per minute (chidren and young people aged 18 and under) then this is a positive test.

Investigations

• Blood tests - FBC, UE, Calcium, TFT, fasting glucose or HbA1c
• 12 lead ECG - consider echo if abnormal
• Ambulatory ECG is not routinely required but can be considered if paroxysmal arrhythmias are suspected.
• Head up tilt test is not routinely required for diagnosis and would only be used in a specialist setting.

Review medication

Certain medications may cause or contribute to symptoms and should be reviewed including:

• Alpha-blockers.
• Beta-blockers.
• Calcium-channel blockers
• Angiotensin-converting enzyme (ACE) inhibitors.
• Diuretics.
• Nitrates.
• Phenothiazines.
• Tricyclic antidepressants.
• Monoamine-oxidase inhibitors.
• Opiates.
• Phosphodiesterase-5 inhibitors.

Management in Primary Care

If the Active Stand Test is positive and investigations are otherwise reassuring then a diagnosis can be confidently made in primary care.

Initial management should be aimed at reassurance that the condition is often self limiting and can be treated with conservative lifestyle measures.

Lifestyle measures that help reduce symptoms include ( from NHS guidance on PoTS ) :

• drink plenty of fluids until your urine is pale yellow
• keep active, but pace yourself and choose your exercise carefully – swimming, rowing, lower limb resistance training, walking, jogging and pilates can help you keep fit and build muscle (strong calf muscles should help pump blood back to your heart)
• elevate the head end of your bed, so you're not sleeping fully horizontal
• try wearing support tights or other forms of compression clothing, to improve blood flow in your legs
• avoid long periods of standing
• rise slowly after lying down – sit for a while before standing
• avoid drinking lots of caffeine or alcohol
• include more salt in your diet, however this is not advisable if you have high blood pressure or kidney or heart disease.

Medication

Currently there are no licensed medications for the treatment of PoTS and no approved medications on the BNSSG formulary.

If lifestyle measures do not help to control symptoms then local cardiologists suggest a low dose betablocker can be trialled in primary care (non-formulary and off licence). Their advice and current evidence suggests that non-selective betablockers such as propranolol (or metoprolol) may be more effective than more selective betablockers in patients with PoTS (5).

Other medications to treat PoTS should not be used in primary care and patients keen to try these should be referred.

Ivabradine is a drug that is being used by some cardiologists but is not currently licensed or on the BNSSG formulary and should not be initiated in primary care.

If medications are prescribed in primary care or on advice of a specialist then please see the principals of prescribing non-formulary drugs below:

Non-formulary prescribing in BNSSG

Referral

If the diagnosis is not clear or if symptoms are not controlled with management in primary care, then consider obtaining cardiology advice and guidance or refer appropriate patients via eRS to Cardiology arrhythmia clinic (NBT or UHBW).

Some patients may also benefit from support for underlying anxiety or other mental health problems, so consider directing them to NHS Talking Therapies.

Managing patient expectation

Please manage patient expectation when referring. PoTS can be difficult to manage and there are currently no licensed medications to treat PoTS.

COVID-19 and inappropriate tachycardia

A proportion of post-COVID-19 patients present with autonomic symptoms including chest pain, palpitations, breathlessness and pre-syncope/syncope (6). See the Long COVID (post COVID-19 syndrome) page for further information.

Patients with tachycardia may or may not have orthostatic symptoms and will not therefore necessarily meet criteria for a diagnosis of PoTS.

A NTproBNP test should be considered in addition to other bloods to help rule out heart failure secondary to COVID-19.

Patients can otherwise often be managed in primary care and should be investigated and managed in the same way as PoTS as detailed in the sections above. 

Referral to cardiology via eRS should be considered for patients who have more significant and persisting symptoms that are not responding to conservative management.

Resources

References

(1) PoTS - GP notebook

(2) PoTS - patient.info

(3) PoTS - Clincal presentation, aetiology and management

(4) Observational case series of postural tachycardia syndrome (PoTS) in post COVID-19 patients - British Journal of Cardiology - Jan 2022

(5) Evidence Review (May 2022) - Use of beta-blockers in PoTS

(6) Evidence Review (May 2022) - PoTS in Long COVID

Patient advice

NHS guidance on PoTS

British Heart Foundation on PoTS

Efforts are made to ensure the accuracy and agreement of these guidelines, including any content uploaded, referred to or linked to from the system. However, BNSSG ICB cannot guarantee this. This guidance does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer, in accordance with the mental capacity act, and informed by the summary of product characteristics of any drugs they are considering. Practitioners are required to perform their duties in accordance with the law and their regulators and nothing in this guidance should be interpreted in a way that would be inconsistent with compliance with those duties.

Information provided through Remedy is continually updated so please be aware any printed copies may quickly become out of date.