POTS2

Overview

• POTS presents with orthostatic intolerance + multi-system symptoms

• Diagnosis = active standing test with sustained HR of > 30 BPM (or 40 BPM in 12 to 19yo) W/O Hypotension

• First line therapy is lifestyle modification, fluid loading, high salt diet, compression wear and graded exercise program

• Can manifest post virally

Clinical

• Complex disorder

• Female = 85%

• Onset in adolescence

• Possible 1 % of young women experience POTS in their lifetime

Triggers

• Infection

  • 80% of patients with long COVID have POTS

• Trauma

• Concussion

• Hormone changes (menarche, post-partum, menopause)

• Surgery

• Can be insidious onset without trigger

Co-morbidities

• Hypermobility

• Migraine

• Autoimmune conditions

• ME/CFS

• Fibromyalgia

• Coeliac disease

• IBS

• hEDS

• MCAS

• MALS

• Sjogren’s Syndrome

• Chairi malformation

Features

• Orthostatic tachycardia without postural blood pressure reduction

• Dizzines

• Light-headedness

• Brain fog

• Presyncope / syncope

• Chest pain or discomfort

• Headache

• Nausea

• Visual disturbance

• Fatigue

• Tremulousness

• Symptoms improve when lying flat

Other non-orthostatic features

• Sleep disturbance

• Cognitive disturbance (Brain fog)

• Gut symptoms (Bloating, early satiety, anorexia, constipation and / or diarrhoea)

• Headache and neck and shoulder pain (Coat hanger pain)

• Systemic pain

• Thermoregulatory dysfunction

• Urinary symptoms

• Exercise intolerance

• Heightened sensitivity to light, noise, smell and taste

Trajectory

• Fluctuating condition

• Flares interspersed with periods of relatively well-controlled symptoms

Diagnosis

• 3 months of symptoms

• Tachycardia with standing

• Exclude other possible causes

  • Medications, anaemia, dehydration, fever, pain , AN, hyperventilation, thyroid

Testing

• NASA Lean Test

• 10 minute active standing test after being supine for 5 minutes

• Positive if

  • > 30 BPM rise

  • OR > 40 BPM rise if 12 t o19 yo

  • No posutral blood pressure drop

  • Occurs within 3 minutes of standing

  • Absolute HR of 120 is also diagnostic

• HR criteria dispute when clinical suspicion is high especially in adolescents

• Diurnal variations in HR response can occur and test if better performed in morning

• Negative test does not exclude due to fluctuations in condition

• Can diagnose Postural Symptoms Without Tachycardia (PSWT)

• Depending acrocyanosis with mottling of feet or hands with prolonged posture during active standing test is common

POTS Scoring Tools

• Malmo POTS Score

  • >42 = 97% sensitivity and 98% specificity

Blood tests

• FBC, UEC, Iron studies, TFT

• ECG

• Holter Monitor to rule out arrhythmias

• Echo to rule out structural causes

Treatment

• Fluid load with 2 to 3 litres per day

• High salt diet 5 to 10g per day through high salt foods

• Can add salt tablets 600mg x 2 tablets three times daily but may worsening GIT symptoms and/or be poorly tolerated

• Compression garments - medical grade - ankle to waist as tolerated

• Exercise helps but often requires recumbent activity before more upright

  • Swimming, Rowing, Cycling

• Exercise physiologist familiar with POTS can help

• Avoid common triggers including

  • Hot and humid environments

  • Hot showers

  • Prolonged standing

  • Excess alcohol, tea, coffee or energy drinks

• Monitor heart rate and blood pressure - wear activity tracker and perform standing tests weekly at home.

• Online support groups are helpful

• Multidisciplinary management is often warranted

Medication

• Orthostatic tachycardia

  • Ivabrandine - Funny current inhibitor

  • Beta Blockers - Propranolol or Metoprolol

• Fluid and volume retention

  • Fludrocortisone - Plasma expansion

  • Midrodrine - Alpha-adrenergic agonist causing vasoconstriction

• Stimulants for fatigue / concentration

Midodrine trial.

• Commence on 2.5mg three times a day +/- a fourth dose ( 3.4 hours apart.)

• Do not take unless up and active. Increase dosages as required to a maximum of 30mg total a day.

Ivabradine

• Ivabradine 2.5mg-5mg BD if required.

• Start on 2.5mg bd and titrate up to 5mg if necessary.

• Night time resting HR can be tolerated down to 45 bpm if asymptomatic.

• Aiming for a daytime resting HR 60 and standing HR under 100bpm.

Long-term

• 19% reported symptoms resolution over 5 years

• 25% of patients cannot attend school or work

• Diagnostic delays are common and many have symptoms attributed to psychological causes

Tips

• Tilt table testing is probably not necessary and an active standing test is enough

• Most patients need medications

• Medication may improve non-orthostatic symptoms

Resources

• The Australian POTS Foundation - potsfoundation.org.au

• Dysautonomia International - dysautonomiainternational.org

• Standing Up To POTS - standinguptopots.org

• POTS UK

• POTS Booklet for patients - POTS UK = 16pages

• Midodrine Fact Sheet - Aus POTS

Reference

• POTS therapy update - Aus Doc