Diagnosing POTS can be challenging in general practice due to its wide range of symptoms and overlap with other conditions. Women, who represent the majority of those affected, face especially long diagnostic delays, with their symptoms frequently misattributed to anxiety or depression, further delaying appropriate care.

Primary care physicians are uniquely positioned to change this. By considering POTS early in your diagnostic process, you can help reduce delays, improve patient outcomes, and alleviate the significant burden this condition places on individuals and the healthcare system.

This guide provides practical tools and strategies to help you identify and manage POTS with confidence, ensuring your patients receive timely and effective care. Together, we can improve the lives of those living with this often-overlooked condition.

Begin the diagnostic process by obtaining a comprehensive medical history from the patient. Pay close attention to symptoms suggestive of autonomic dysfunction, including :

1. 1. Orthostatic symptoms
      • Dizziness/light-headedness
      • Postural tachycardia
      • Headache
      • Pre-syncope/syncope
      • Palpitations
      • Fatigue
      • Exercise intolerance
   2. Gastrointestinal symptoms
      • Nausea,
      • Early satiety
      • Decreased appetite/weight loss
      • Bloating
      • Constipation
      • Reflux [in the setting of young age and normal weight]
   3. Thermoregulatory/Secretory issues
      • Heat and cold intolerance
      • Excessive sweating lack of sweating
      • Dry eyes/mouth
   4. Bladder symptoms
      • Hesitancy/retention
      • Urgency/frequency
   5.  Vasomotor
      • Facial flushing
      • Dependent acrocyanosis [purple/red colouring of peripheries during orthostasis]
   6. Other systemic features
      • Brain fog
      • Pain syndromes
      • Post exertional malaise

The following recommendations are informed by international consensus statements on POTS diagnosis. Diagnostic resources  can be found by clicking on the hyperlinks below: 

1. Therapeutic update

2. Malmo POTS Symptom Score (MAPS)

3. Active Standing Test

Pre-screening

Before diagnosing POTS, it is essential to assess for other conditions that can cause orthostatic symptoms. While some of these conditions may coexist with POTS, identifying and treating them is crucial to ensure accurate diagnosis and optimal management. Examples of such conditions include:

• Acute dehydration
• Medications which affect hydration or sympathetic responses: (Diuretics, stimulants, SRNIs)
• Prolonged and sustained deconditioning from bed rest
• Recreational drug effects
• Structural cardiac disorders: (eg: valvular disorders).
• Endocrinopathies: (Adrenal disorders, carcinoid tumours, hyperthyroidism, pheochromocytomas)
• Malnutrition: (Significant nutritional deficiencies and anaemia).

Tilt Table Testing Not Required: It’s important to note that a formal tilt table test is not mandatory for the diagnosis of POTS. While tilt table testing can be useful in some cases, especially for research purposes or when the diagnosis is unclear, it is not essential for the clinical diagnosis of POTS.

Step-by-Step Approach to Active Standing Test

Optimal Testing Time:

• At least 20 minutes is required to undertake an active standing test in a clinical setting.  This takes into account the time for preparation, supine resting and active standing. To maximise the effective use of physician time, consider engagement of practice nurses to undertake standing test assessments.
• Conduct testing in the morning when symptoms are typically more pronounced and patients are well-rested.

Preparation:

• Ask the patient to wear loose fitting, comfortable clothing.
• Verify that the patient has not taken medications that restrict heart rate. (eg: beta blockers)
• Where possible undertake the test in a fasted state.
• Remove shoes and socks to ensure that dependant acrocyanosis (red/purple changes in skin colour) can be monitored throughout the test.

Equipment:

• An electronic BP monitor (a manual BP monitor may be required to confirm low blood pressure readings). 
• A pulse oximeter for constant monitoring of heart rate.
• A patient bed (ideally electric)

Active Standing Test Instructions:

• Ensure the patient has been lying at rest for at least 5 minutes prior to testing.
• Inform the patient on what the test will entail.
• Have the patient stand up from the lying position in one smooth motion.
• The patient should not walk after standing (as this promotes blood return and will skew the test result).
• For safety reasons have the patient stand with a bed behind them in case of syncope. 
• Encourage the patient not to fidget, talk or move their feet throughout the test.
• Monitor and document the patient’s blood pressure and heart rate every minute for 10 minutes.
• Ask the patient if they are experiencing any of the following throughout the testing period: nausea,  vision changes, dizziness, headache, light headedness.
• Objectively monitor for signs of perfuse sweating, tremulousness and dependent acrocyanosis. 

Diagnosis of POTS requires:

• A sustained rise in heart rate of ≥30 bpm in adults (or ≥40 bpm in adolescents) within the first 10 minutes of standing, OR
• An absolute heart rate > 120 beats per minute.
• An absence of orthostatic hypotension, defined as a decrease in systolic blood pressure of ≥20 mmHg or diastolic blood pressure of ≥10 mmHg within the first 3 minutes of standing.
• Presence of unexplained symptoms for at least 3 months

Interpretation and Considerations:

• The Canadian Position Statement suggests that it is not appropriate to apply the delta heart rate rise to individuals with a resting heart rate <60 bpm.
• Note that transient ‘initial orthostatic hypotension’ and ‘late orthostatic hypotension’ does not exclude POTS. However sustained orthostatic hypotension which initiates within the first three minutes of standing or tilt, is consistant with a diagnosis of ‘orthostatic hypotension’ NOT POTS. 
• Note that POTS symptoms fluctuate and one negative test does not exclude a POTS diagnosis.  Consider repeat home standing tests to confirm. 
• Postural hypertension (a blood pressure increase in response to standing or tilt) is a hallmark feature of hyperadrenergic POTS.
• We recommend the use of the Malmo POTS Symptom survey as an adjunctive diagnostic tool. A score ≥42 should illicit high suspicion for POTS even in the absence of a positive stand test.
• Syncope may be present in POTS, however it is more common for people with POTS NOT to faint during standing test.
• When syncope does occur in POTS it may be accompanied by seizure activity that results from cerebral hypoperfusion and hypoxia. In such instances, recumbance and elevation of the legs should be sufficient to restore blood supply to the brain. Once the patient is sufficiently recovered, offer an oral bolus of 500 mls of water or electrolyte drink to enhance recovery. 

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

• Coeliac Disease
• Endometriosis
• Fibromyalgia
• Irritable Bowel Syndrome
• Hypermobility Spectrum Disorder
• Hypermobile Ehlers Danlos Syndrome
• Migraine
• Myalgic Encephalomyelitis/Chronic Fatigue Syndrome
• Mast Cell Activation Syndrome

Anxiety and POTS

The risk of misdiagnosis of anxiety and depression in POTS is very high, particularly amongst women. Many anxiety surveys primarily rely on the reporting of somatic symptoms, which are likely to be common in individuals with Postural Orthostatic Tachycardia Syndrome (POTS). This reliance on somatic symptoms may result in an inaccurate representation of the true prevalence of anxiety in POTS patients. For more detailed information, please refer to an excellent article on the relationship between POTS and anxiety disorders: HERE

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.

Lifestyle Modifications

Lifestyle modifications should be the first line therapy for POTS and require attention to education to fully effect the benefits

Salt and Fluid Intake:

• The Canadian consensus guidelines recommends 10,000 mg of salt daily to increase plasma blood volume. Integrating salt supplementation by incorporating low carbohydrate electrolyte drinks  is encouraged. 
• Ensure adequate fluid intake (2-3 liters/day) to expand plasma volume and alleviate symptoms. Advise reduction of high caffeine drinks that may increase diuresis and vasodilation.

Compression Garments

• Recommend the use of compression garments, particularly those offering ankle-to-waist and abdominal compression, to improve venous return and attenuate orthostatic intolerance.

Dietary Adjustments

• Evidence from the Vanderbilt Autonomic Center highlights that dysregulation of glucose/insulin response in POTS contributes to heightened symptoms postprandially. Therefore, advocating for lower carbohydrate, smaller and more frequent meals is generally recommended.
• Consider involving a dietitian for patients with weight loss or restrictive eating patterns due to functional gastric symptoms.

Additional Therapies

Exercise:

• Advise patients on the benefits of exercise in improving vagal tone, expanding blood volume, and reducing inflammation.
• Caution is needed in highly symptomatic patients, and recumbent step-wise movement is advised.
• It is important that patients with marked post exertional malaise are not ‘pushed’ through traditional rehabilitation programs which  may worsen fatigue.
• Engagement with a POTS-aware exercise physiologist is recommended for individualised exercise planning.

Supportive Therapies

• There is no evidence that Cognitive Behavioral Therapy (CBT) is an effective ‘treatment’ in POTS.
• However, supportive psychotherapy may aid patients adapt to the marked changes in their lifestyle due to the chronic nature of their condition.
• Although not curative, it is likely that vagal stimulation breathing techniques may be beneficial for symptom control.

Medication Avoidance

• Avoid medications known to exacerbate POTS symptoms, such as SNRIs, Yasmin oral contraceptive pill, and diuretics. Carefully consider potential blood pressure-lowering effects of stimulants like Guanfacine.

Management of Triggers

• Avoid orthostatic activities particularly those associated with heat, such as hot showers
• Addressing triggers such as environmental allergies may provide additional symptom relief and should be integrated into treatment plans.
• Lifestyle modifications, though beneficial, often necessitate adjunctive pharmacotherapy for significant symptom relief. It’s important to note that all medications used in POTS management are off-label and typically require a trial-and-error approach. For more information on international consensus statements on POTS management please: CLICK HERE

Pharmacological Interventions

Heart Rate Control:

• Ivabradine: Acts by selectively inhibiting the If current in the sinoatrial node, reducing heart rate without affecting blood pressure. It’s often preferred for heart rate control in POTS due to its minimal effect on blood pressure.
• Propranolol: A non-selective beta-blocker that reduces sympathetic effects on heart rate and blood pressure. Lower doses are preferred in POTS to avoid vasodilatory effects which may exacerbate fatigue and orthostatic symptoms.

Plasma Expansion:

• Fludrocortisone: A synthetic corticosteroid that acts on the kidneys to increase sodium and water retention, expanding plasma volume and improving orthostatic intolerance. Caution should be taken to consider the side effect profile of Florinef.
• Desmopressin: A synthetic analogue of vasopressin that enhances water reabsorption in the kidneys and constricts blood vessels, leading to increased plasma volume and improved orthostatic symptoms. This medication has restricted access and is not routinely available for treatment of POTS in Australia unless there is evidence of diabetes insipidus.

Vasopressor Enhancement:

• Midodrine: A selective alpha-1 adrenergic agonist that constricts blood vessels, leading to increased blood pressure, thereby improving orthostatic symptoms.
• Phenylephrine: Classified as an alpha-1 adrenergic agonist, phenylephrine functions by constricting blood vessels, thereby raising blood pressure.s