Postural Orthostatic Tachycardia Syndrome (PoTS) - under review

Overview

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

Local specialists advise that postural orthostatic tachycardia syndrome (POTS) is a clinical syndrome characterised by an increase in heart rate within 10 minutes of standing (≥30 beats per minute) in the absence of other causes, such as anaemia, hyperthyroidism, adrenal insufficiency and low body weight (1). It is often associated with symptoms including chest pain, headaches, chronic fatigue, irritable bowel syndrome and cognitive difficulty (often referred to as ‘brain fog’).

POTS should not be considered a `heart condition` but rather a multi-system disorder caused by an autoimmune process.

Most patients can be diagnosed and treated primary care with secondary care involvement reserved in cases of diagnostic uncertainty such as cardiac arrhythmia or consideration of second line medication.

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

The most common features include unexplained tachycardia and other symptoms during upright posture which is relieved by recumbence. Dizziness and pre-syncope are common although full syncope is uncommon.

Other features include: Headache, tunnel vision, fatigue, neurocognitive impairment (brain fog), exercise intolerance, weakness, dyspnea, tremulousness, nausea, chest or abdominal pain, sweating, heat intolerance, anxiety, irritable bowel-type symptoms and palpitations.

Secondary causes

Further information including possible secondary causes can be found in the link below:

Postural Orthostatic Tachycardia Syndrome (PoTS Syndrome)(3).

Prognosis

POTS is not associated with increased mortality. Fifty percent of patients improve with time alone and two thirds of patients receiving pharmacological therapy report an overall improvement (3).

Who to Refer

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

Referral to a specialist (cardiologist, gastroenterologist, urologist, rheumatologist, neurologist) should be considered only in patients with diagnositic uncertainty or significant ongoing symptoms despite appropriate lifestyle measures / management in primary care. Specialist referral is based upon predominant bodily system affected (eg palpitations/chest pain to cardiology IBS to gastroenterology, fatigue to CFS/Long COVID clinic etc).

There is no specialist POTS clinic at UHBW or NBT.

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 required for diagnosis.

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 ) :

• Encourage oral fluid (at least 2-3L per day) until urine is pale yellow
• Encourage adequate salt intake (10g per day). 1 teaspoon of salt is approx 2.3g.This is not advisable in patients with high blood pressure or kidney/ heart disease.
• Avoid recumbancy/bedrest.
• Encourage standing and exercise such as swimming, rowing, lower limb resistance training, walking, jogging and pilates.
• Elevate the head end of your bed (2 novels under bed legs) to avoid horizontal sleep
• Consider support tights or other forms of compression clothing, to improve return blood flow in lower limbs.
• Rise slowly after lying down – sit for a while before standing
• Avoid excess caffeine and alcohol

Medication

Currently there are no licensed medications for the treatment of POTS and no approved medications on the BNSSG formulary. Pharmacological treatment should be guided towards the patient’s specific symptom.

In patients with postural dizziness/lightheadedness, fludrocortisone (0.1 to 0.2mg orally once daily) can be given.

In patients with symptomatic tachycardia a low dose betablocker can be given. Non-selective betablockers such as propranolol or metoprolol may be more effective than more selective betablockers in patients with POTS (6). Start at lowest dose (eg propranolol 10-20mg tds) and uptitrate to effect.

Ivabradine (5mg bd) can be used in patients unable to take betablockers. However this is not 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 referring to a specialist. For POTS patients with cardiac symptoms, consider cardiology advice and guidance  or refer appropriate patients via eRS to Cardiology 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 (7). 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.

Resources

References

(1) Canadian Cardiovascular Society Position Statement on Postural Orthostatic Tachycardia Syndrome (POTS) and Related Disorders of Chronic Orthostatic Intolerance

(2) GP Guide: PoTS on a Page - PoTS UK

(3) PoTS - patient.info

(4) PoTS - Clincal presentation, aetiology and management

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

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

(7) 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.