Postural Orthostatic Tachycardia Syndrome (POTS) is a condition whereby normal regulation of autonomic functions like blood pressure and heart rate are affected. This form of dysautonomia is characterised by an abnormal increase in heart rate upon standing or transitioning to an upright position. POTS can affect people of all ages but is more commonly diagnosed in females, particularly those aged 15–50.
Signs & Symptoms
The symptoms of POTS can vary widely between individuals but often include:
- Increased heart rate: A rise of more than 30 beats per minute (bpm) in adults or 40 bpm in adolescents within 10 minutes of standing.
- Light-headedness or dizziness: Especially when standing up from a sitting or lying position.
- Fatigue: Often severe and disabling, unrelated to exertion.
- Palpitations: A sensation of the heart pounding or fluttering.
- Brain fog: Difficulty concentrating or thinking clearly.
- Nausea: Sometimes accompanied by abdominal discomfort or bloating.
- Exercise intolerance: Reduced ability to perform physical activities without worsening symptoms.
- Headaches: Which may worsen upon standing or physical exertion.
- Fainting (syncope): In severe cases, a loss of consciousness can occur.
Other symptoms may include shaking, excessive sweating, difficulty sleeping, and cold extremities due to poor blood circulation.
Causes and Contributing Factors
Postural Orthostatic Tachycardia Syndrome (POTS) has a complex and multifactorial aetiology. While the exact mechanisms remain unclear, several theories and underlying conditions have been linked to its development. These factors often overlap, making it essential to consider each patient’s unique circumstances when identifying the cause.
1. Autonomic Nervous System Dysfunction
POTS is fundamentally a condition of autonomic dysregulation. The autonomic nervous system (ANS) controls involuntary bodily functions such as heart rate, blood pressure, and digestion. In POTS:
- Sympathetic Overactivity: The “fight or flight” response may be overactive, leading to a rapid increase in heart rate upon standing.
- Impaired Parasympathetic Response: The “rest and digest” mechanisms may not adequately counteract the effects of standing, further contributing to symptoms.
This dysfunction can be primary (no identifiable cause) or secondary (linked to other medical conditions).
2. Blood Volume Abnormalities
Many individuals with POTS exhibit hypovolaemia (reduced blood volume). This occurs due to:
- Reduced plasma volume: The liquid component of blood is decreased, impairing the circulatory system’s ability to maintain adequate blood flow.
- Inadequate sodium retention: Sodium is crucial for maintaining fluid balance. Abnormal sodium regulation can worsen hypovolaemia.
When blood volume is low, the body compensates by increasing heart rate to maintain blood pressure and blood flow to vital organs, contributing to POTS symptoms.
3. Cardiovascular Abnormalities
In some cases, the blood vessels in POTS patients fail to constrict effectively upon standing (a process called venoconstriction). This results in:
- Blood pooling in the lower extremities: Reduced blood return to the heart, causing symptoms like dizziness and fainting.
- Inefficient blood distribution: The heart has to pump harder to compensate for the reduced blood flow to the brain and other vital organs.
4. Neurological and Autoimmune Conditions
POTS is often associated with neurological and autoimmune conditions. Examples include:
- Small Fiber Neuropathy: Damage to the small nerves that control blood vessel function can impair normal autonomic responses.
- Autoimmune Dysregulation: Autoantibodies targeting adrenergic (involved in sympathetic activity) or muscarinic (involved in parasympathetic activity) receptors may interfere with autonomic function. Studies have shown increased prevalence of autoimmune diseases such as lupus or Sjögren’s syndrome in POTS patients.
5. Viral Infections
POTS frequently develops following viral illnesses, suggesting a possible post-viral syndrome. Viruses may:
- Trigger an autoimmune response that attacks the autonomic nervous system.
- Lead to prolonged inflammatory responses that disrupt normal autonomic regulation.
- Contribute to deconditioning, where prolonged illness and reduced activity weaken the body, exacerbating symptoms.
Examples of implicated viruses include Epstein-Barr virus (associated with glandular fever), influenza, and SARS-CoV-2 (the virus responsible for COVID-19).
6. Connective Tissue Disorders
Conditions like Ehlers-Danlos Syndrome (EDS), particularly the hypermobile subtype, are frequently associated with POTS. In EDS:
- Weak connective tissue leads to joint hypermobility and vascular laxity.
- Blood vessels may struggle to constrict effectively, causing pooling in the legs and triggering POTS symptoms.
This overlap is significant, as EDS is often underdiagnosed, especially in younger populations.
7. Hormonal Influences
Hormonal changes can influence the onset or severity of POTS:
- Adolescence: POTS often begins during the teenage years, coinciding with significant hormonal shifts.
- Menstrual Cycle: Symptoms can worsen during certain phases due to changes in fluid retention and hormonal levels.
- Pregnancy: While some women experience improvement during pregnancy due to increased blood volume, others report worsening symptoms postpartum.
Hormonal dysregulation, such as reduced renin-angiotensin-aldosterone activity (which helps regulate blood pressure and fluid balance), may also play a role.
8. Physical Deconditioning
Prolonged inactivity, such as after surgery, bed rest, or illness, can lead to:
- Loss of muscle tone: Particularly in the legs, which are essential for maintaining blood flow when standing.
- Reduced cardiovascular fitness: Making it harder for the heart and blood vessels to respond effectively to positional changes.
- Impaired autonomic reflexes: Leading to delayed or exaggerated heart rate responses upon standing.
9. Genetic Factors
While no single genetic mutation has been definitively linked to POTS, familial cases suggest a possible genetic predisposition. Genes affecting connective tissue integrity, immune responses, or autonomic regulation may contribute.
10. Stress and Psychosocial Factors
Although not a direct cause, stress can worsen POTS symptoms by:
- Triggering the sympathetic nervous system.
- Reducing adherence to lifestyle modifications like proper hydration and exercise.
- Contributing to physical and mental fatigue.
It is essential to distinguish between stress as a contributing factor and the misconception that POTS is purely psychological.
11. Other Secondary Causes
POTS may also arise secondary to other medical conditions, such as:
- Mast Cell Activation Syndrome (MCAS): Characterised by abnormal mast cell activity, which can lead to excessive histamine release, affecting blood pressure and heart rate.
- Chronic Fatigue Syndrome (CFS/ME): Overlapping symptoms, such as fatigue and exercise intolerance, are common.
- Diabetes: Autonomic neuropathy from poorly controlled diabetes may result in POTS-like symptoms.
Stress and other lifestyle factors, such as poor hydration, can exacerbate symptoms but are not direct causes.
Grading
POTS is not typically graded in severity in the same way some conditions are. However, symptoms can range from mild, where daily activities are possible with minor adjustments, to severe, where individuals may be bedridden or require significant assistance with daily living tasks.
Prognosis
The outlook for POTS varies widely. Some people experience improvement or remission over time, especially with appropriate management. Others may have long-term symptoms that fluctuate in intensity. Recovery may depend on the underlying cause, with those having POTS secondary to a specific condition often improving if that condition is treated. However, many individuals learn to manage symptoms effectively and maintain a good quality of life with the right treatment plan.
Treatment
Management of POTS focuses on reducing symptoms, improving quality of life, and addressing underlying causes where possible.
Lifestyle Modifications
- Hydration and salt intake: Increasing water and salt consumption helps boost blood volume and reduce symptoms. This should always be done under medical guidance.
- Compression garments: Wearing compression stockings or abdominal binders can help improve circulation.
- Postural strategies: Techniques like crossing the legs while standing or gradually transitioning from lying to standing can prevent sudden heart rate increases.
- Dietary adjustments: Eating smaller, frequent meals and avoiding excessive sugar or caffeine can minimise symptom triggers.
Physiotherapy and Rehabilitation
Physiotherapy is an essential part of POTS management. A graded exercise program, developed by a physiotherapist, can help improve cardiovascular fitness, muscle strength, and autonomic regulation. Key aspects include:
- Recumbent exercises: Starting with activities like cycling or rowing in a seated or lying position to avoid symptom exacerbation.
- Gradual progression: Slowly increasing exercise intensity and duration over weeks or months.
- Strength training: Focusing on large muscle groups, particularly in the legs, to aid blood return to the heart.
- Stretching and balance work: To maintain mobility and reduce the risk of dizziness or falls.
Physiotherapists also educate patients on recognising and managing their triggers.
Medications
Medications may be prescribed to manage symptoms or address underlying issues. This should be discussed with your primary care provider and can include:
- Beta-blockers: To regulate heart rate.
- Fludrocortisone: To increase blood volume.
- Midodrine: To constrict blood vessels and improve blood pressure.
- Ivabradine: To specifically target heart rate reduction.
Psychological Support
Living with a chronic condition like POTS can be emotionally challenging. Psychological support, such as cognitive behavioural therapy (CBT) or mindfulness techniques, can help manage stress and improve coping strategies.
Alternative Therapies
Some individuals find symptom relief through complementary approaches, including:
- Acupuncture.
- Massage therapy.
- Relaxation techniques like yoga or meditation.
It is essential to consult health professionals to ensure these therapies are safe and suitable.
Information is provided for education purposes only. Always consult your physiotherapist or other health professional