Malady Wise

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POTS2

Maladywise

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

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

Reference