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    Information On POTS Syndrome

    Postural Orthostatic Tachycardia Syndrome

    What is POTS

    POTS is a common form of dysautonomia which largely affects women of child-bearing age.  The prevalence of POTS worldwide and in Australia is not currently understood, however some experts suggest that as many as 1 in 100 young women may experience POTS in their lifetime.

    Triggers

    POTS may be triggered by physical stressors such as viral infection, surgery, concussion, and pregnancy. In more recent times there has been increased reporting of POTS post SARS-CoV-2 infection, with some evidence that people with ‘Post Acute Covid Syndrome’ also have dysautonomia. POTS may also appear gradually and may be associated with familial genetic factors such as collagen disorders.

    Symptoms

    POTS often presents with the hallmark cardiac symptoms of orthostatic dizziness, light-headedness, and postural induced tachycardia.  However, as the autonomic nervous system is broadly affected, POTS is also accompanied by a constellation of symptoms that span multiple organ systems. A detailed health history may reveal:

    • Sleep disturbance
    • Lethargy
    • Systemic pain
    • Cognitive disruption (‘brain fog’)
    • Bladder symptoms (over-active bladder, urinary retention)
    • Gastrointestinal disturbance (bloating, early satiety, anorexia, constipation and/or diarrhoea)
    • Headache and neck and shoulder pain (‘coathanger’ pain)
    • Heightened sensitivity to light, noise, smell and taste.

    Comorbidities

    There appears to be multiple comorbidities that have a higher prevalence in POTS than the general population.  These include (but are not limited to):

    • Chiari Malformation
    • Coeliac Disease
    • Chronic Fatigue Syndrome/Myalgic Encephalomyelitis
    • Irritable Bowel Syndrome
    • Hypermobility Spectrum Disorder
    • Hypermobile Ehlers Danlos Syndrome
    • Migraine
    • Fibromyalgia
    • Mast Cell Activation Syndrome
    • Median Arcuate Ligament Syndrome
    • Sjogren’s Syndrome

     

    Prognosis

    POTS is not a condition that is normally ‘outgrown’. Depending on the underlying cause and severity, some individuals may continue to experience symptoms throughout their lifetime. It is important to know that 25% of patients diagnosed with POTS are debilitated to the extent that they cannot attend school or work.  Diagnostic delays are common, and many patients are mis-diagnosed or have their symptoms attributed to psychogenic causes.  Not only does this compound the impact of their illness but it likely delays access to appropriate care and treatment.  As POTS has a peak onset in the adolescent/young adult years, it has the capacity to disrupt education, social, economic, and vocational engagement, and development.  Early recognition and intervention are paramount to maximising positive outcomes for patients. Compassionate, multidisciplinary care is essential to improving quality of life.

    Diagnosis

    If you suspect your patient may have POTS, the following assessments may be helpful in confirming a diagnosis.

    • Thorough health history to identify common POTS symptoms and co-morbidities
    • Assessment of symptoms to establish onset triggers and chronicity
    • Standing Test/Poor Man’s Tilt Table. Repeat blood pressure and heart rate readings:
      • After 5 minutes of laying supine
      • After standing (without leaning or moving) at intervals of 2 minutes for a total of 10 minutes

    POTS should be suspected if there is:

    • A sustained heart rate rise on standing ≥ 30 bpm (or ≥40 bpm in adolescents)
    • An absence of orthostatic hypotension (≥20/10 mmHg blood pressure drop on standing)
    • Chronicity of symptoms ≥3 months from trigger
    • Associated orthostatic symptoms such as light-headedness, visual disturbance, pre-syncope
    • An absence of a known secondary cause of symptoms (dehydration, medication, anaemia, sepsis)

     

    Investigations

    To establish the absence of secondary causes of symptoms the following investigations are recommended before confirming a POTS diagnosis.

    • 12 lead ECG
    • Routine pathology including a full blood count and thyroid function
    • Holter monitor (to rule out arrhythmias)
    • Echocardiogram (to rule out structural abnormalities)

    POTS Treatment

    Treatment

    Hypovolaemia of unknown aetiology is common in POTS. (However, routine pathology rarely identifies this as both red cell and plasma volume remain relative to each other).  For this reason, POTS treatments predominately target plasma expansion and venous return. A multidisciplinary approach to lifestyle adjustments is also encouraged as the first step to treatment.  Some treatment options include:

    Lifestyle management

    • Where blood pressure allows, increase consumption of NaCl 10-12g and water 2-3 litres daily
    • Encourage wearing medical grade compression-wear (from ankle to waist) during waking hours to reduce blood pooling and increase venous return
    • Encourage avoidance of hot environments
    • Graded exercise can help to increase blood volume and improve venous return. De-conditioning may precipitate functional decline however, in some patients, post-exertional malaise may complicate participation in low grade exercise. Early involvement of an Exercise Physiologists familiar with the treatment of POTS is highly recommended to manage this aspect of recovery
    • Consider dietician/nutritional involvement where there is reduced oral intake due to gastrointestinal disturbance

     

    Pharmacotherapy Management

    Pharmacotherapy in POTS may require expert input and response is dependent on the severity of symptoms as well as the underlying pathogenesis of the condition.  Pharmacotherapy is usually targeted at increasing plasma volume, slowing heart rate and increasing venous return.

    Some examples of treatment options are:

    • Fludrocortisone for plasma expansion
    • Beta Blockers/heart rate control propranolol and ivabradine
    • Alpha receptor agonists Phenylephrine/Midodrine
    • IV fluids for acute decompensation or for refractory symptomology
    • Stimulants for fatigue/concentration

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