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The Different Types of POTS Syndrome

Have you ever stood up too fast and gotten lightheaded or dizzy from it? If this happens to you frequently, you may be wondering if it is a cause for concern. 

Typically, lightheadedness caused by standing up too fast is related to something called orthostatic hypotension, a condition that causes your blood pressure to drop when you move from sitting down to standing up. Similar yet slightly different symptoms can occur from a different condition called POTS also referred to as Postural Orthostatic Tachycardia Syndrome or Postural Tachycardia Syndrome.

POTS is an abnormal increase in heart rate that happens when you move from sitting or lying down to standing up. This can cause a variety of symptoms such as dizziness, fatigue and heart palpitations. POTS is a complex and surprisingly common condition, affecting an estimated 500,000 to 1,000,000 people in the United States alone. This article will break down the different types of POTS as well as symptoms, diagnosis and treatment.

Understanding POTS

The autonomic nervous system is the part of your body that is responsible for helping to regulate things like blood pressure and heart rate. When conditions like POTS occur, it is typically due to an issue or dysfunction of the autonomic nervous system, also referred to as “autonomic dysfunction.” POTS can also be related to other conditions such as autoimmune disorders and viral infections.

When you’ve been lying down or sitting for a while, gravity causes blood to settle in the lower half of your body, in areas like your abdomen, legs and arms. Upon standing up quickly, your body works to move that blood back up to essential organs such as the heart and brain. When your body detects a lack of blood flow and oxygen in the upper half, it responds by releasing certain hormones to facilitate circulation, leading to an increased heart rate.

POTS occurs when the heart rate rises by at least 30 beats per minute during the first 10 minutes of transitioning from a horizontal to a standing position. An elevated heart rate can result in various symptoms, including lightheadedness, brain fog, shortness of breath, dizziness, fatigue and chest discomfort or palpitations.

POTS vs. Orthostatic Hypotension: What’s the Difference?

POTS and orthostatic hypotension could easily be mistaken for one another. Both conditions may be referred to as types of “orthostatic intolerances.” The main difference between these two similar conditions is: 

  • Orthostatic hypotension is a drop in blood pressure due to a change in position
  • POTS is an increase in heart rate due to a change in position 

These conditions can happen together and often involve similar symptoms but treatment approaches will vary. It is important to work with your doctor to determine an accurate diagnosis that ensures an effective treatment approach.  

Subtypes of POTS

There are several subtypes of POTS including hyperadrenergic POTS, neuropathic POTS, secondary POTS, pediatric POTS and hypovolemic POTS. Here’s a closer look at each of them:

Hyperadrenergic POTS

Hyperadrenergic POTS occurs when the sympathetic nervous system (your “fight-or-flight” system) is overactive, causing an excess of norepinephrine in the bloodstream. This subtype of POTS may be associated with symptoms like anxiety and increased blood pressure, in addition to the typical symptoms like palpitations. Treating this type of POTS may require medications that help to regulate norepinephrine levels such as beta blockers. 

Neuropathic POTS

Neuropathic POTS is usually related to neuropathy or damage to the nerves. Nerve fibers that line the blood vessels help keep the blood pressure stable. When the small fiber nerves that line the blood vessels break down, it is harder for these vessels to tighten when they need to, which can lead to neuropathic POTS. 

Diagnosing neuropathic POTS can require specialized testing, such as skin biopsies. Treatment usually involves treating the underlying neuropathic dysfunction and managing symptoms. 

Secondary POTS

Secondary POTS occurs when a POTS diagnosis is knowingly associated with another disease such as Lyme disease, diabetes, lupus or Sjogren’s syndrome. Diseases that lead to POTS are usually those that cause issues with the autonomic nervous system or autoimmune diseases. 

Pediatric POTS

POTS doesn’t just affect adults, it can also affect children and adolescents. Diagnosing POTS in children looks slightly different than in adults. Rather than looking for a heart rate increase of 30 beats per minute or more, doctors will look for an increase of 40 beats per minute or more.

Symptoms of POTS may vary slightly in children as well, including things like excessive sweating and difficulty thinking or concentrating. POTS can have a major impact on the pediatric population, causing higher rates of mental health challenges like anxiety and depression. 

Hypovolemic POTS 

Hypovolemic POTS occurs when symptoms are related to the blood volume being low. This usually happens as a result of an injury or illness that causes a loss of body fluid, such as a major wound, burn injury or sickness that causes vomiting or diarrhea. The main treatment for hypovolemic POTS would involve replacing the fluid that has been lost.

Common Symptoms of POTS

Symptoms of POTS vary significantly among patients. Factors such as age, overall health and underlying conditions cause every POTS patient to have a different experience. For this reason, it is important to seek out healthcare from a variety of specialists, including cardiologists, neurologists and any other specialists you may need. Some of the most common symptoms of POTS include:

  • Dizziness and lightheadedness when you stand up
  • Palpitations or an irregular heartbeat. This may feel like a discomfort or fluttering in the chest. 
  • Chronic fatigue
  • Shortness of breath
  • Underlying conditions

About 16-20% of people with POTS also have an autoimmune condition and many people with POTS have a family history of an autoimmune condition. Some studies have noted links between POTS and autoimmune conditions like Ehlers-Danlos Syndrome and Sjogren’s Syndrome. While immune system abnormalities and dysfunction may be related to POTS, the exact connection is not yet fully understood. 

Mast Cell Activation and Viral Illness: How the Immune Response can Trigger POTS

The immune system includes special cells called mast cells. These cells release chemicals such as histamine and cytokines to aid in the body’s immune response. There is ongoing research on mast cell activation and POTS. Some studies suggest that mast cell activation is a potential contributor to the symptoms of POTS. It is important to note that there is debate surrounding this subject and research is ongoing.  

Other research suggests that viral infections can lead to the development of POTS. In some cases, patients develop post-viral neuropathy, which affects the autonomic nervous system leading to the development of POTS. Viral infections can also sometimes trigger an autoimmune response which may contribute to POTS.

Diagnosing POTS

POTS can be a difficult condition to diagnose due to the vagueness of the symptoms. Many people who have POTS experience symptoms for months or years before getting diagnosed. In diagnosing POTS, your provider will ask a variety of questions about your health history, current medications and existing health conditions. They will also perform a physical exam and may recommend a tilt table test. 

A tilt table test involves lying on a special, movable table that you are strapped into. Healthcare workers then tilt the table to move your body from lying down to a standing position while you are safely strapped in. They will monitor your vital signs closely during the test to look for any changes in heart rate and blood pressure. Your doctor may also recommend additional diagnostic tests such as blood and urine tests to help rule out other possible conditions. 

Treatment Approaches

There is no cure for POTS. However, there are a variety of approaches that can be used to manage symptoms, including medications and lifestyle adjustments. 

Medication Overview

There are no medications specifically approved by the FDA for treating POTS. Doctors sometimes prescribe certain medications off-label to help with managing symptoms of POTS. Medications used to manage POTS are listed below.

  • Norepinephrine is sometimes prescribed to help tighten the blood vessels and bring more blood up to the heart and brain. 
  • Fludrocortisone helps to increase salt retention and blood volume.
  • Midrodine causes the blood vessels to tighten to help keep the blood where it needs to be and prevent blood pooling.
  • Beta-blockers help to reduce the heart rate when it is elevated.
  • Pyridostigmine stimulates the parasympathetic nervous system activity which helps to decrease the elevated heart rate. 

Lifestyle Modifications

Exercise can help with managing POTS since it improves heart health and strengthens muscles. Reclined aerobic exercises such as swimming, rowing and the recumbent bike are thought to be the best in managing POTS.

Diet modifications can also help with managing POTS. The main diet recommendations to help with POTS include limiting salt intake and consuming enough fluids. Staying hydrated helps to prevent hypovolemia and support a healthy blood volume.

Final Thoughts

POTS may be a complicated disease but it doesn’t have to complicate your life. By working with a knowledgeable, multidisciplinary healthcare team, you can gain a full understanding of your condition and get on board with an effective plan to help keep your symptoms at bay. The healthcare providers at UCF Health are here to help you. Schedule your visit today.