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Children and Young People Consensus

A network of clinicians with an interest in the care of children and young people with Postural Tachycardia Syndrome (PoTS) has developed this quick reference guide to support healthcare professionals in the effective management of patients with this condition.

This resource provides an overview of the aetiology and pathophysiology of PoTS, alongside its clinical presentation, diagnostic criteria, recommended investigations, and associated comorbidities. It also outlines current management approaches, including both pharmacological and non-pharmacological strategies.

Download a printable copy here.

If you have any comments or queries, please email: enquiries@potsuk.org.

Quick Reference Guide – Managing PoTS

Introduction

Postural tachycardia syndrome (PoTS) or postural orthostatic tachycardia syndrome (POTS) is a chronic disorder often starting in adolescence with symptoms of orthostatic intolerance (symptoms that develop on standing and resolve when supine), the most common of which are light-headedness/presyncope and palpitations.  In addition, there are symptoms unrelated to posture such as fatigue, brain fog and gastrointestinal disturbances. It is diagnosed by demonstrating a persistent tachycardia on active standing, NASA lean test or head-up tilt test and in absence of postural hypotension. Symptoms must be present for at least 3 months.

Aetiology and pathophysiology

The aetiology is unknown and likely multifactorial. Symptoms may start insidiously without triggers but are often related to triggers including viral illness and puberty.   The underlying pathogenesis could be:

  • dysregulation of the autonomic nervous system’s response to hypovolaemia (central vascular hypovolaemia with decreased venous return and excessive compensatory tachycardia)
  • autonomic neuropathy related (sympathetic autonomic dysfunction causing reduced vasoconstriction, venous pooling, decreased cardiac output and compensatory tachycardia)
  • hyperadrenergic states (excessive sympathetic activation causing tachycardia, tremors and palpitations)
  • Some patients may have an autoimmune cause or there may be concurrent autoimmune disease

It can also arise from combination of all above elements.

Comorbid conditions are common (see below).

Symptoms

  • the predominant presenting symptoms in typical adolescent PoTS patients are dizziness/ light-headedness /presyncope.
  • GI symptoms (nausea, vomiting, abdominal pain, IBS-like symptoms)
  • fatigue, brain fog
  • chest pains, palpitations
  • heat and exercise intolerance
  • headaches
  • tremors
  • sleep difficulties
  • breathlessness
  • visual and auditory disturbances
  • bladder issues.
  • transient loss of consciousness (syncope) is reported but most are vasovagal episodes

 Some symptoms persist when the patient is supine.

Signs

  • Patients may have no physical signs apart from a tachycardia on standing
  • There may be purple discolouration of lower legs and hands (acrocyanosis) on standing.
  • It is important to seek symptoms and signs of other conditions which mimic PoTS (see below).

Diagnostic Criteria

Patients should have experienced PoTS symptoms for a minimum for 3 months

Patients with PoTS (12-18 years old) should show a persistent rise of heart rate from the immediate supine HR of 40 bpm (30 bpm in adults) or more within 10 minutes of standing (rate criteria do not apply in the context of anxiety/pain).

If typical PoTS symptoms but 40bpm threshold not quite met, individualise approach -management as for PoTS may be beneficial.

There should be no significant fall in BP (>20/10 mm Hg) during this test. A significant fall in systolic or diastolic BP indicates orthostatic hypotension or precedes vasovagal syncope which indicates stopping the test. Coexisting vasovagal syncope does not preclude a diagnosis of PoTS.

Investigations

  • Active Stand Test: Check HR (heart rate) and blood pressure (BP) after lying for 5 min, then stand the patient unsupported and still, with bare feet, monitoring HR and BP at frequent intervals for up to 10 min. Use continuous ECG monitoring for HR when available as brachial intermittent devices may miss brief changes. Note acrocyanosis when present.
  • HR and BP data collected at home may be unreliable.
  • NASA Lean Test is less well validated but sometimes used. Interpretation is similar to the Active Stand Test. 
  • Tilt table test: the criterion of 40 bpm is used as for the Active stand. PoTS research has not been precise on HR change and it is based on consensus opinion.   Tilt tests are only available in some specialist centres; they add a greater dimension of study in doubtful cases and when conducted for a longer period are valuable in diagnosis of syncope.

Additional investigations when diagnosis is in doubt or comorbidities require assessment:

Blood tests:

  • FBC
  • ferritin
  • thyroid function tests
  • vitamin D
  • kidney function tests
  • blood sugar
  • coeliac screen
  • early morning cortisol
  • B12 and folate

Cardiac investigations where history signs and symptoms indicate: 

  • 12-lead ECG if there are cardiac symptoms such as palpitations or syncope.
  • Echocardiogram –mandatory if abnormal cardiac examination or ECG, or other indications of structural heart disease (eg family history of early suspected cardiac death).
  • 24-hour ECG – useful adjunct to assess the basal HR and how frequent is tachycardia (very valuable in differential diagnosis with inappropriate sinus tachycardia).
  • Ambulatory BP monitoring – can help in medication selection.

Differential diagnosis

Consider “PoTS mimics” such as:

  • orthostatic hypotension
  • inappropriate sinus tachycardia
  • vasovagal syncope and other causes of orthostatic intolerance
  • anaemia
  • Addison’s disease
  • arrhythmias
  • structural heart disease

If BMI < 17 kg/m2 it is important to ensure that any disordered eating is stabilised with the patient’s weight restored and that usual cardiovascular function has returned before reassessment for PoTS.

Co-morbidities

  • post‑acute infective syndromes including Long COVID
  • hypermobile Ehlers-Danlos syndromes
  • myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS)
  • neurodiversity
  • mast cell activation syndrome
  • migraine
  • depression/anxiety
  • complex pain syndromes
  • Sjogren’s syndrome
  • gastroparesis

A PoTS-like picture may present in:

  • anorexia/eating disorders
  • systemic lupus
  • diabetes
  • sarcoidosis

Comorbidities may be contributing to symptoms which combined with those of PoTS may affect various elements of wellbeing and further contribute to poor school attendance.

Management

When PoTS is diagnosed, start with non-pharmacological management strategies before considering medication.

  • Non- Pharmacological/Conservative:
    • fluid intake (2-3litres/day)
    • increase salt intake (5-10 g/day) in older children, and 0.12g/kg/day in 2 divided doses in smaller children
    • waist-high compression garments
    • carefully managed age and patient appropriate activity programme incorporating aerobic exercise, (initially when supine) – caution in those with post exertional malaise (eg ME/CFS)
    • balanced meals low in refined carbohydrates
    • avoid prolonged standing and immobilisation
    • ensure sleep hygiene
    • temperature regulation avoiding overheating e.g. long/hot showers/baths
  • Pharmacological: When significant ongoing symptoms persist despite conservative measures:
    • Where possible reduce or stop drugs that may worsen symptoms (raise HR, reduce BP or cause fatigue) e.g. amitriptyline

Doses in Table are for older children. See BNF guidance for more details and for younger/smaller children.

  • Other management considerations:
    • Attend to comorbidities
    • Support with reassurance, education and empathy
    • Pain management
    • Physiotherapy
    • Occupational therapy to achieve agreed goals
    • Psychologist/psychiatrist input, if necessary

Follow-up

Review at intervals (e.g. every 3–6 months) assessing:

  • medication side-effects
  • interactions and compliance with other necessary medication
  • symptom trajectory
  • orthostatic vitals
  • exercise tolerance and functional status
  • school attendance
  • comorbidity management

PoTS UK Subscription for Healthcare Professionals

This consensus statement on Diagnosing and Managing Children and Young People with PoTS was developed by PoTS UK in collaboration with a network of clinicians with a specialist interest in the condition.

Dr Anbarasu Theodore Anbu, Consultant Paediatrician, Alder Hey Children’s NHS Foundation Trust

Dr Kate Dharmarajah, Lead for Paediatric Long Covid, Evelina London Children’s hospital

Prof Philip R Fischer, MD, Professor of Pediatrics, Mayo Clinic, USA

Dr John E. Fortunato, M.D., MBA; Professor of Paediatrics, Northwestern University Feinberg School of Medicine

Dr. Alessandro Giardini, MD, PhD, Consultant Paediatric Cardiologist, Great Ormond Street Hospital

Dr Oliver Jefferis, Consultant Paediatrician, Oxford University Hospitals NHS Foundation Trust

Dr Lesley Kavi, Chair of PoTS UK

Dr Karen McLeod, Consultant Paediatric Cardiologist, Royal Hospital for Children, Glasgow

Dr Pramod Nair, Paediatrician with expertise in cardiology and POTS, Bedford Hospital, Bedfordshire Hospital NHS Trust

Prof Richard Sutton DSc MB BS FRCP Emeritus Professor of Clinical Cardiology Department of Cardiology Hammersmith Hospital Campus

Dr Richard Tozer, Consultant Paediatrician, Torbay and South Devon NHS Foundation Trust

Dr Terry Segal, Consultant in General Paediatrics and Adolescent Medicine, University College Hospital London