POTS Syndrome: Symptoms, Causes & Treatment Guide

What Is POTS and How Does It Affect the Body?

POTS (Postural Orthostatic Tachycardia Syndrome) is a disorder of the autonomic nervous system characterized by an excessive increase in heart rate when standing up. The heart rate rises by at least 30 beats per minute (or exceeds 120 bpm) within 10 minutes of standing, causing symptoms like dizziness, fatigue, and palpitations. POTS affects approximately 1-3 million people in the United States alone.

Postural Orthostatic Tachycardia Syndrome is classified as a form of dysautonomia, which means dysfunction of the autonomic nervous system. The autonomic nervous system controls many vital body functions that happen automatically, including heart rate, blood pressure, digestion, and temperature regulation. In POTS, this system doesn't respond appropriately when you change from a lying or sitting position to standing.

When a healthy person stands up, gravity causes blood to pool in the lower body. The autonomic nervous system quickly compensates by constricting blood vessels and slightly increasing heart rate to maintain blood flow to the brain. In people with POTS, this compensation mechanism doesn't work correctly. The blood vessels don't constrict adequately, causing the heart to beat much faster to maintain adequate blood circulation. This excessive heart rate increase is what causes the symptoms of POTS.

The condition predominantly affects women of reproductive age, with approximately 80% of POTS patients being female. Most cases develop between ages 15 and 50, often appearing after puberty, pregnancy, or a viral illness. While POTS is not immediately life-threatening, it can significantly impact quality of life and ability to perform daily activities.

Types of POTS

Research has identified several subtypes of POTS, which may respond differently to treatments. Understanding your specific type can help guide treatment decisions.

• Neuropathic POTS: The most common form, caused by damage to small nerve fibers that control blood vessel constriction. Often associated with peripheral neuropathy.
• Hyperadrenergic POTS: Characterized by elevated norepinephrine levels when standing, causing symptoms like tremor, anxiety, and high blood pressure upon standing.
• Hypovolemic POTS: Associated with low blood volume, which exacerbates symptoms. May respond well to increased fluid and salt intake.
• Secondary POTS: Occurs as a result of another condition, such as Ehlers-Danlos syndrome, autoimmune disorders, or diabetes.

How Common Is POTS?

POTS is more common than many people realize. It's estimated that 1-3 million people in the United States have POTS, though many cases go undiagnosed. The condition has received increased attention since the COVID-19 pandemic, as many Long COVID patients develop POTS-like symptoms. Studies suggest that 2-14% of COVID-19 survivors may experience dysautonomia symptoms.

Despite its prevalence, POTS is often underdiagnosed or misdiagnosed. Many patients report seeing multiple doctors over several years before receiving a correct diagnosis. Increasing awareness among healthcare providers and the public is crucial for earlier diagnosis and treatment.

What Are the Symptoms of POTS?

POTS symptoms primarily occur when standing and include rapid heart rate (tachycardia), dizziness, lightheadedness, fatigue, brain fog, palpitations, exercise intolerance, nausea, headaches, and near-fainting or fainting. Symptoms typically improve when lying down and may be worse in the morning, after meals, or in hot environments.

The symptoms of POTS can vary widely between individuals and may fluctuate from day to day. Most symptoms are related to the body's difficulty maintaining adequate blood flow to the brain when upright. While standing, the heart races to compensate for blood pooling in the lower body, and the brain may not receive optimal blood supply, leading to many of the characteristic symptoms.

Primary Cardiovascular Symptoms

The hallmark cardiovascular symptoms of POTS relate directly to the abnormal heart rate response when standing. These symptoms are often what first bring patients to medical attention.

• Tachycardia: Heart rate increases by 30+ beats per minute or exceeds 120 bpm within 10 minutes of standing. Many patients describe feeling their heart "racing" or "pounding."
• Palpitations: Awareness of the heartbeat, often described as fluttering, skipping, or thumping sensations. Can occur at rest or with activity.
• Chest discomfort: Some patients experience chest pain or tightness, which should always be evaluated to rule out cardiac conditions.
• Blood pooling: Visible discoloration (purple or red) of the legs and feet when standing, caused by blood collecting in the lower extremities.

Neurological and Cognitive Symptoms

Reduced blood flow to the brain when standing leads to several neurological symptoms that can significantly impact daily functioning and quality of life.

• Dizziness and lightheadedness: Feeling unsteady or like the room is spinning, especially when standing up or being upright for extended periods.
• Brain fog: Difficulty concentrating, memory problems, and feeling mentally "cloudy." Many patients describe this as one of their most disabling symptoms.
• Presyncope and syncope: Feeling like you might faint (presyncope) or actually fainting (syncope). About 30% of POTS patients experience fainting episodes.
• Headaches: Often described as "coat hanger" headaches affecting the back of the head and shoulders, related to reduced blood flow to the brain.
• Visual disturbances: Blurred vision, tunnel vision, or seeing spots, especially when standing.

Fatigue and Exercise Intolerance

Chronic fatigue is one of the most common and debilitating symptoms of POTS. Unlike normal tiredness, this fatigue is often disproportionate to activity level and not relieved by rest. Exercise intolerance is particularly frustrating for patients who were previously active.

The fatigue in POTS has multiple causes: the heart is working harder than normal, the autonomic nervous system is dysregulated, and chronic sleep disturbances are common. Many patients describe feeling exhausted even after minimal exertion and may need to rest after simple activities like showering or standing to prepare a meal.

Exercise intolerance manifests as an exaggerated heart rate response to physical activity, shortness of breath, and rapid exhaustion. Paradoxically, while exercise is challenging, carefully structured exercise programs are one of the most effective treatments for POTS.

Gastrointestinal Symptoms

The autonomic nervous system also controls digestion, so many POTS patients experience gastrointestinal symptoms. These can include:

• Nausea: Often worse after eating or when standing
• Bloating and early satiety: Feeling full quickly when eating
• Constipation or diarrhea: Altered bowel motility is common
• Abdominal pain: May be related to blood pooling in abdominal vessels

What Causes POTS?

POTS can be caused by viral infections (including COVID-19), autoimmune conditions, connective tissue disorders like Ehlers-Danlos syndrome, pregnancy, surgery, or trauma. In many cases, the exact cause remains unknown. The underlying mechanism involves dysfunction of the autonomic nervous system that controls blood vessel constriction and heart rate regulation.

Understanding what causes POTS is an active area of research. While the exact mechanisms vary between patients, all forms of POTS involve some dysfunction in how the autonomic nervous system regulates cardiovascular responses to position changes. Several potential triggers and associated conditions have been identified.

Viral Infections and Post-Infectious POTS

One of the most common triggers for POTS is a viral infection. Many patients report that their symptoms began after an illness such as influenza, mononucleosis, or more recently, COVID-19. The connection between viral illness and POTS may involve several mechanisms:

Viral infections can trigger autoimmune responses that affect the autonomic nervous system. Research has found that many POTS patients have autoantibodies against certain receptors in the heart and blood vessels. Additionally, some viruses may directly damage the small nerve fibers that control blood vessel function, leading to neuropathic POTS.

The COVID-19 pandemic has brought increased attention to post-infectious POTS. Studies show that a significant portion of Long COVID patients develop POTS or POTS-like symptoms, potentially due to autoimmune mechanisms, viral persistence, or direct damage to the autonomic nervous system.

Connective Tissue Disorders

Ehlers-Danlos syndrome (EDS), particularly the hypermobile type, is strongly associated with POTS. Up to 80% of hypermobile EDS patients may have POTS. The connection may be related to increased stretchiness of blood vessels, which allows more blood to pool in the lower body when standing. Additionally, the connective tissue abnormalities may affect the structure of small blood vessels and nerves.

Autoimmune Conditions

POTS is more common in people with autoimmune diseases such as Sjögren's syndrome, lupus, and autoimmune thyroid conditions. Researchers have found various autoantibodies in POTS patients that may contribute to autonomic dysfunction. This has led to interest in immunotherapy treatments for some patients.

Other Triggers and Associated Conditions

• Pregnancy: Some women develop POTS during or after pregnancy, possibly related to blood volume changes
• Surgery or trauma: Major surgery or physical trauma can trigger POTS in susceptible individuals
• Puberty: POTS commonly develops during adolescence, possibly related to rapid growth and hormonal changes
• Deconditioning: Prolonged bedrest or inactivity can lead to POTS-like symptoms
• Mast cell activation syndrome: Some POTS patients have concurrent mast cell disorders

How Is POTS Diagnosed?

POTS is diagnosed using a tilt table test or active standing test that measures heart rate and blood pressure changes over 10 minutes of standing. The diagnostic criteria require: heart rate increase of 30+ bpm (40+ for ages 12-19) within 10 minutes of standing, symptoms lasting over 6 months, and absence of significant blood pressure drop. Blood tests rule out other conditions.

Diagnosing POTS requires documenting the characteristic heart rate response to standing while excluding other conditions that could cause similar symptoms. The diagnostic process typically involves several steps, including clinical evaluation, specific tests for autonomic function, and laboratory tests to rule out other conditions.

Diagnostic Criteria for POTS

According to the Heart Rhythm Society Expert Consensus Statement, the diagnosis of POTS requires all of the following:

1. Heart rate increase: An increase of ≥30 beats per minute (or heart rate exceeding 120 bpm) within 10 minutes of standing or upright tilt. For adolescents ages 12-19, the threshold is ≥40 bpm.
2. Chronic symptoms: Symptoms of orthostatic intolerance (dizziness, palpitations, lightheadedness, etc.) must be present for at least 6 months.
3. Absence of orthostatic hypotension: There should not be a sustained blood pressure drop of >20/10 mmHg within 3 minutes of standing.
4. No other explanation: Other medical conditions that could explain the symptoms must be excluded.

Tilt Table Test

The tilt table test is considered the gold standard for diagnosing POTS. During this test, you lie strapped to a special table that can tilt to an almost standing position (usually 70-80 degrees). Heart rate and blood pressure are continuously monitored while you're tilted upright for 10-45 minutes.

In a positive test for POTS, heart rate will increase by at least 30 bpm or exceed 120 bpm within 10 minutes of tilting, without a significant drop in blood pressure. The test also monitors for symptoms and can help identify the specific type of POTS.

Active Standing Test

An alternative to the tilt table test is the active standing test, which can be performed in a regular clinic setting. After resting in a supine position for at least 5 minutes, you stand still for 10 minutes while heart rate and blood pressure are measured at regular intervals.

While simpler than the tilt table test, the active standing test may be slightly less sensitive because patients can use their leg muscles to help blood return to the heart. However, it's a practical option when tilt table testing is not available.

Blood Tests and Other Evaluations

Several conditions can cause symptoms similar to POTS and must be excluded through testing:

• Complete blood count: To check for anemia, which can cause fatigue and dizziness
• Thyroid function tests: Both hyperthyroidism and hypothyroidism can affect heart rate
• Blood glucose and diabetes screening: Diabetic neuropathy can cause autonomic dysfunction
• Cortisol levels: Adrenal insufficiency can cause similar symptoms
• Vitamin B12 and iron studies: Deficiencies can contribute to fatigue and neuropathy
• Catecholamine levels: May be measured in hyperadrenergic POTS
• Electrocardiogram (ECG): To evaluate heart rhythm and structure
• Echocardiogram: May be performed to rule out structural heart problems

How Is POTS Treated?

POTS treatment focuses on lifestyle modifications as first-line therapy: increased fluid intake (2-3 liters daily), increased salt (3-10 grams daily), compression garments, and graded exercise therapy. Medications like fludrocortisone, midodrine, and beta-blockers may be added if lifestyle changes are insufficient. Treatment is individualized based on POTS subtype and specific symptoms.

There is no single cure for POTS, but most patients can achieve significant symptom improvement with proper treatment. The approach is individualized based on the specific type of POTS, severity of symptoms, and which treatments are most effective for each patient. Treatment typically begins with lifestyle modifications, with medications added as needed.

Lifestyle Modifications – First-Line Treatment

Lifestyle changes form the foundation of POTS treatment and are effective for many patients. These modifications aim to increase blood volume, improve blood vessel tone, and enhance the body's ability to compensate for position changes.

Fluid and Salt Intake

Increased fluid intake is crucial for POTS management. Most patients are advised to drink 2-3 liters of fluids daily, spread throughout the day. Water is good, but beverages containing electrolytes may be more effective at maintaining blood volume. Some patients benefit from oral rehydration solutions or electrolyte supplements.

Increased salt intake helps the body retain fluids and expand blood volume. Patients without hypertension or kidney problems are often advised to consume 3-10 grams of salt daily. This can be achieved through dietary changes, salt tablets, or adding salt to water. Your doctor will advise on the appropriate amount based on your specific situation.

Compression Garments

Compression stockings and abdominal binders help prevent blood from pooling in the lower body when standing. Waist-high compression stockings (30-40 mmHg) are most effective, though some patients find knee-high stockings helpful as well. Abdominal compression garments can also help by preventing blood pooling in the splanchnic (abdominal) circulation.

Position and Activity Modifications

• Elevate the head of your bed: Sleeping with the head elevated 4-6 inches can help the body adapt to upright posture
• Stand up slowly: Move from lying to sitting to standing gradually
• Avoid prolonged standing: Sit when possible, shift weight, or cross legs while standing
• Physical counter-maneuvers: Crossing legs, squatting, or tensing leg muscles can help when feeling dizzy
• Avoid triggers: Heat, alcohol, large meals, and dehydration can worsen symptoms

Exercise Therapy

Graded exercise therapy is one of the most effective treatments for POTS, though it can be challenging to implement. The goal is to recondition the cardiovascular system while avoiding symptom flares. Most programs start with recumbent exercises (swimming, rowing, recumbent cycling) that are done lying down or semi-reclined to minimize symptoms.

A typical exercise program begins with 15-20 minutes of recumbent exercise 3-4 times weekly, gradually increasing duration and intensity over 2-3 months. Upright exercises are added slowly as tolerance improves. It can take 3-6 months of consistent exercise to see significant improvement, but studies show that exercise therapy can substantially reduce symptoms and heart rate responses.

Medications for POTS

When lifestyle modifications are insufficient, several medications may be helpful. The choice of medication depends on the specific type of POTS and individual symptoms. All medications should be prescribed and monitored by a healthcare provider familiar with POTS.

Volume Expansion

• Fludrocortisone: A synthetic mineralocorticoid that helps the body retain salt and water, increasing blood volume. It's often a first-line medication for POTS.
• Desmopressin (DDAVP): Helps the kidneys retain water, useful for some patients who don't respond to fludrocortisone.

Vasoconstricting Agents

• Midodrine: Constricts blood vessels, helping to prevent blood pooling. Taken before activities that worsen symptoms.
• Pyridostigmine: Enhances nerve signals to blood vessels and can help moderate heart rate increases.

Heart Rate Control

• Beta-blockers: Low-dose beta-blockers like propranolol or metoprolol can help control the excessive heart rate increase, though they must be used carefully to avoid worsening fatigue.
• Ivabradine: A newer medication that specifically slows heart rate without affecting blood pressure, showing promise in POTS treatment.

Other Medications

• Clonidine or methyldopa: May help in hyperadrenergic POTS by reducing excessive catecholamine release
• Stimulants: Medications like modafinil may help with fatigue and brain fog in some patients
• SSRIs: Some selective serotonin reuptake inhibitors have been shown to help POTS symptoms, independent of their antidepressant effects

How Can You Manage Daily Life with POTS?

Living with POTS requires learning your personal triggers and developing coping strategies. Key management strategies include pacing activities, staying hydrated, wearing compression garments, avoiding triggers (heat, large meals, alcohol), maintaining a consistent sleep schedule, and building a support network. Many patients can continue work and activities with appropriate accommodations.

Managing daily life with POTS requires adapting activities and routines to work around symptoms. While this can be challenging, many patients develop effective strategies that allow them to maintain good quality of life, continue working, and participate in activities they enjoy.

Understanding Your Triggers

Everyone with POTS has different triggers that worsen their symptoms. Common triggers include:

• Heat exposure: Hot weather, hot showers, and saunas can dilate blood vessels and worsen symptoms
• Dehydration: Even mild dehydration significantly impacts symptoms
• Large meals: Blood diverts to the digestive system after eating, worsening orthostatic symptoms
• Alcohol: Causes blood vessel dilation and dehydration
• Morning hours: Blood volume is lowest after sleeping, making mornings typically harder
• Menstrual periods: Hormonal changes can worsen POTS symptoms
• Prolonged standing: Standing in lines or at events is particularly challenging

Practical Daily Management Tips

Developing a consistent daily routine helps manage POTS symptoms. Here are practical strategies that many patients find helpful:

• Start the day slowly: Drink water and eat something salty before getting out of bed. Sit on the bed edge before standing.
• Stay consistently hydrated: Drink fluids throughout the day, not just when thirsty. Set reminders if needed.
• Eat smaller, frequent meals: This reduces the blood flow diversion that occurs with large meals.
• Plan activities for your best times: Many POTS patients feel better in the afternoon or evening.
• Take rest breaks: Pace activities and rest before becoming exhausted.
• Keep cool: Use fans, cool compresses, and air conditioning. Take lukewarm rather than hot showers.
• Use assistive strategies: Sit when possible, use a shower chair, and have a stool for standing tasks.

Work and School Accommodations

Many POTS patients can continue working or attending school with appropriate accommodations. Common accommodations include:

• Ability to sit rather than stand
• Access to water and salt at all times
• Flexible scheduling or remote work options
• More frequent breaks
• Climate control (avoiding hot environments)
• Reduced physical demands when symptomatic

Mental Health and Support

Living with a chronic illness like POTS can be emotionally challenging. Anxiety and depression are common among POTS patients, both as a result of living with chronic illness and potentially as part of the dysautonomia itself. It's important to address mental health as part of comprehensive POTS management.

Connecting with others who have POTS through support groups (online or in-person) can be incredibly helpful. Organizations like Dysautonomia International provide resources, support, and community connections. Having a supportive healthcare team, family, and friends also makes a significant difference in managing this condition.

What Is the Long-Term Outlook for POTS?

The prognosis for POTS is generally favorable. Studies show that 50-80% of patients experience significant improvement over time, and some recover completely. Adolescent-onset POTS often improves or resolves with maturity. Even when POTS is chronic, most patients can achieve good quality of life with proper management. POTS is not life-threatening and does not shorten life expectancy.

Many people with POTS worry about what their future holds. The good news is that POTS is not a degenerative condition, and the outlook for most patients is positive. While complete recovery isn't guaranteed, significant improvement is common with appropriate treatment.

Recovery and Improvement Rates

Research on POTS outcomes shows encouraging results. Studies following POTS patients over time have found:

• Approximately 50-80% of patients experience significant improvement in symptoms over time
• About 19% of patients in one study achieved complete recovery
• Adolescent-onset POTS often improves as patients reach their mid-20s
• Post-viral POTS may have a better prognosis, with many patients improving within 2-5 years

Factors that may predict better outcomes include younger age at onset, identifiable trigger (like viral illness), shorter duration before treatment, good response to exercise therapy, and commitment to lifestyle modifications.

Living Well with Chronic POTS

Even when POTS is chronic, most patients can achieve a good quality of life. With proper management, many people with POTS are able to work, attend school, exercise, travel, and participate in activities they enjoy. The key is finding the right combination of treatments and lifestyle adaptations that work for you.

It's important to maintain realistic expectations while remaining hopeful. Progress may be gradual, with good days and bad days. Celebrate improvements, no matter how small, and be patient with setbacks. Many patients find that their symptoms become more predictable and manageable over time as they learn their triggers and develop effective coping strategies.

Is POTS Related to Long COVID?

Yes, POTS has emerged as a significant component of Long COVID (post-acute sequelae of SARS-CoV-2). Research estimates that 2-14% of COVID-19 survivors develop dysautonomia symptoms. Many patients diagnosed with Long COVID meet the diagnostic criteria for POTS. The mechanism may involve autoimmune responses, viral effects on the nervous system, or deconditioning from illness.

The COVID-19 pandemic has dramatically increased awareness of POTS and autonomic dysfunction. Many patients who develop prolonged symptoms after COVID-19 infection are found to have POTS, and this connection has led to increased research and clinical attention to dysautonomia.

How COVID-19 Triggers POTS

Several mechanisms may explain the connection between COVID-19 and POTS:

• Autoimmune response: COVID-19 can trigger autoantibody production that affects the autonomic nervous system
• Direct viral effects: SARS-CoV-2 may affect nerve tissues directly or through inflammation
• Deconditioning: Prolonged illness and bedrest can lead to cardiovascular deconditioning
• Mast cell activation: COVID-19 may trigger mast cell activation syndrome, which is associated with POTS

Treatment Considerations for Post-COVID POTS

Treatment for post-COVID POTS follows the same principles as other forms of POTS – lifestyle modifications, exercise therapy, and medications as needed. Some patients with post-COVID POTS may benefit from additional treatments targeting inflammation or autoimmune processes, though research is ongoing in this area.

The good news is that many patients with post-COVID POTS do improve over time. However, recovery can take months to years, and patience with the treatment process is important.