The following is an overview of
POTS written in understandable terms for
patients, their families, and anyone else who
may need to learn about POTS. Find links to
more detailed explanations, in-depth
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POTS is short for Postural Tachycardia Syndrome. It is referred to by some physicians, patients, and researchers as Postural Orthostatic Tachycardia Syndrome, or just Postural Tachycardia. At Dysautonomia SOS, we refer to POTS as Postural Tachycardia Syndrome because that is how it is used in most medical publications, as Postural and Orthostatic have very similar meanings.
It is a type of Orthostatic Intolerance and dysautonomia. Dysautonomia is defined as any malfunction or condition of the Autonomic Nervous System (ANS), and includes many other conditions detailed on our website.
Let’s break down what the name POTS means:
Postural means relating to posture, or a change in position.
Tachycardia refers to a rapid heart rate.
Syndrome means a collection of symptoms, and not a disease. Technically, a syndrome is defined as a group of signs and symptoms that occur together and characterize a particular abnormality.
So, POTS is a collection of symptoms that result from a heart rate increase when standing up. When a person with POTS stands up, the heart pumps faster to help keep the blood pressure up, and try to help the blood in their body reach their brain and other vital organs. The body of a person with POTS does not respond to gravity the same way a non-POTS person does. This is exhausting for a POTS patient, as their body has to work much harder to maintain a “normal” state than a healthy person. Many equate it to feeling like they are running a marathon from doing the simplest tasks, all the while feeling dizzy and fatigued. Some people with POTS pass out, and some have to lay down frequently to keep from passing out.
The majority of POTS patients are female, of child bearing age, and under 50. The current ratio according to medical literature is 5:1 of women to men. Most look perfectly healthy on the outside. There are thought to be anywhere from 500,000 to millions of people in the US with POTS and other forms of Orthostatic Intolerance. The Mayo Clinic estimates that 1 in 100 teens will develop POTS.
In order to meet the most recent POTS criteria for diagnosis, a person’s heart rate must increase at least 30 beats per minute (BPM), or go above 120 BPM within 10 minutes of standing, and for kids/teens the requirement in 40 BPM. This may happen with or without a change in blood pressure, and symptoms are relieved by lying down. There must also be other symptoms of Orthostatic Intolerance, such as excessive fatigue, headaches, and dizziness (a list of common symptoms can be found below).
The recent definitions by some doctors say that if your blood pressure drops significantly, you do not have POTS, but instead have Orthostatic Hypotension, but this varies by publication and physician. Most researchers and physicians also include that you must have symptoms for 3-6 months to rule out acute causes, such as those resulting from dehydration or bed rest.
Many patients are diagnosed using a Head Up Tilt Table Test (HUTT), or by taking sets of heart rate and blood pressure measurements in the lying, sitting, and standing positions (called a set of "orthostatics"), and by taking an extensive medical history. More details of testing are listed below.
POTS symptoms can come and go, and change in intensity. The following summarizes some of the more common symptoms, but by no means is a complete list. POTS is complicated because many patients have underlying and/or coexisting conditions causing symptoms, and it is hard to figure out which is causing each issue. They can change from day to day – even hour to hour, making POTS a potentially debilitating syndrome that can impact one’s quality of life dramatically.
Symptoms can include:
• Fainting or near fainting
• Visual blurring or tunnel vision
• Tremulousness (shakiness) and weakness, especially of the legs
• Exercise intolerance
• Neck pain, also called “coat hangar pain”
• Shortness of breath
• Anxiety and panic feelings
• Chest pain
• Nausea and other stomach and digestive symptoms
• Acral coldness or pain (meaning in the extremities)
• Concentration difficulties (brain fog)
• Headaches and migraines
• Sweating problems, either too much or too little
• Increased heart rate while laying down
• Foot swelling and obvious blood pooling in the legs
• An increase in adrenalin levels in the blood (called norepinephrine and catecholamines)
UNDERLYING CAUSES OF POTS:
POTS is thought to be caused by many things, which is also known as being heterogeneous. Some of the things thought to cause POTS include:
• Low blood volume, also called Hypovolemia.
• Blood pooling in the extremities, also known as Acrocyanosis, sometimes caused by valve or muscle pump defects in the legs, neuropathy (destruction of nerves), and low blood volume. Other diseases and conditions can cause blood pooling in the arms and legs as well.
• Autonomic Neuropathy, which is when there is damage to the autonomic nerves. Autonomic nerves are the nerves that control things that happen unconsciously, like sweating. This damage can cause problems with the signals between the brain and the body, including the heart and blood vessels. It is estimated by the Mayo Clinic that up to 50% of all POTS patients have some form of Autonomic Neuropathy.
• Denervation of the legs, or a loss of nerve supply. Causes of denervation include disease, chemical toxicity, physical injury, or intentional surgical interruption of a nerve.
• Increased Angiotensin II and decreased Nitric Oxide (NO), which causes veins to not constrict properly.
• Autoimmune Diseases such as Multiple Sclerosis, Sjogren’s, Lupus, Myasthenia Gravis, Diabetes, Guillain-Barré Syndrome, Fibromyalgia, Amyloidosis, Ankylosing Spondylitis, Raynaud's, Celiac , Chronic Fatigue and Immune Dysfunction Syndrome (CFIDS), Chronic Fatigue Syndrome - also known as Myalgic Encephalomyelitis (ME), Autoimmune Autonomic Ganglionopathy (AAG), Sarcoidosis, and general connective tissue diseases are thought to either cause or be associated with POTS.
• Post-Viral or Sudden Onset means that something sudden triggered POTS symptoms. A virus, pregnancy, a car accident, a traumatic event (such as surgery), a growth spurt, etc… brought out the underlying condition that is causing POTS.
• There are a variety of other diseases and syndromes thought to cause (or are associated with) POTS, including Ehler’s-Danlos Syndrome, Adrenal Diseases such as Cushing’s and Addison’s, Mast Cell Activation Syndrome (MCAS), Lyme Disease (and associated co-infections), Mitochondrial Diseases, and Paraneoplastic neurological syndromes.
• Other conditions that can cause or have been associated with POTS include: Traumatic Brain Injuries, Chiari Malformations, Gulf War Syndrome, Heavy Metal Poisoning, Parasites (such as Chagas), Liver Cirrhosis, Mycoplasma pneumonia, the Epstein - Barr virus, the Nutcracker Syndrome, as well as reactions to drugs and medications.
This is a LONG growing and changing list. We strongly urge you to look for the underlying cause of POTS, as stated above – it is not a disease, it is a syndrome, and once you find the cause your doctor may be able to treat the symptoms of POTS more effectively. Sometimes the cause cannot be found, which is termed "idiopathic" POTS.
A full cardiac work up should also be performed if you are told you have POTS to rule out any structural or electrical issues with your heart. Conditions to rule out may be serious or life threatening, and symptoms may mimic or cause POTS. They include structural abnormalities such as congenital defects or "holes" in the heart, valve defects, cardiomyopathy, and cardiac atrophy (de-conditioning causing the heart to become smaller), to name a few.
Testing depends on the symptoms of each individual patient. Additional cardiac testing or blood work may be performed as well. A thorough medical exam and history will be taken, and the most common test to diagnose POTS is a Tilt Table Test.
Some of the more common Autonomic Tests used to diagnose POTS and other forms of autonomic dysfunction include:
-Head Up Tilt Table Test (HUTT): The patient lies on a stretcher-like support. Straps that are similar to seat belts are attached around the abdomen and legs and the patient is tilted upright. The exact angle of the tilt varies and is usually between 60 and 80 degrees. The tilting goes on for up to 45 minutes, and blood pressure and heart rate measurements are taken.
-Basic Orthostatics: Heart rate and blood pressure measurements are taken at intervals while laying, sitting, and standing. Also called, "A Poor Man's Tilt."
-ECG monitoring: Recording the electrical activity of the heart.
-A cold pressor test: The cold pressor test evaluates efferent sympathetic function. It is carried out by placing the hand in a basin filled with 50% ice and 50% water for approximately 1 minute.
-Isometric hand-grip test: During this test, a patient squeezes a hand-grip until their arm is fatigued, and blood pressure and heart rate are monitored.
-Deep breathing tests: Deep breathing evaluates the vagal function on heart rate modulation during slow inhalation for 5 seconds and exhalation for 5 seconds over 90 seconds.
-The Valsalva Maneuver: The Valsalva maneuver is performed by blowing through a closed mouthpiece with a tiny leakage (16-gauge hole) to maintain 40 mmHg for approximately 15 s. It tests several components of the baroreflex arc.
-Quantitative Sudomotor Axon Test (QSART): The test requires a mild electrical stimulation on the skin which allows acetylcholine, a naturally occurring chemical, to stimulate sweat glands. The QSART measures the volume of sweat produced by this stimulation.
-Thermoregulatory Sweat Test (TST): The thermoregulatory sweat test and the quantitative sudomotor axon reflex test both determine the sweat gland function and the integrity of efferent cholinergic sympathetic nervous system function.
-Serum catecholemines: Measurements of catecholemines in the blood (both lying down and standing) involve blood levels being measured after resting lying flat for a period of time, and then again after standing for a period of time to detect abnormal levels.
-A blood plasma volume test: There are two types of blood volume tests, one is a nuclear lab procedure used to measure the volume (amount) of blood in the body, the other (called a Daxor) uses a dye to measure levels. The test also measures the volume of plasma and of red cells in the blood, useful for measuring hypovolemia in dysautonomic patients.
Treatment can include a variety of drug related options, as well as lifestyle changes that help improve symptoms in some. Again, this will vary greatly from patient to patient, as the underlying cause of POTS impacts how a patient will respond. Always check with your physician before starting any treatment. Find suggestions and more detailed descriptions for non-drug related treatments in our Tips and Tools section. Some of the more common lifestyle adjustments include:
-Increasing fluid and sodium intake: this helps to retain fluids and expand blood volume. Check with your doctor for a suggested amount, but most recommend 3-10 grams of sodium a day.
-Wearing compression stockings: this helps to prevent blood pooling and increases circulation. Abdominal binders are sometimes worn to help with abdominal pooling. Obtain full compression by wearing full length compression pantyhose that have a high waist.
-Exercising regularly: de-conditioning greatly affects POTS in most cases, and keeping as active as possible (under physician supervision) is extremely important. Many people start in the recumbent position or by doing floor exercises, and work their way to a more upright routine.
-Dietary Changes: many people with POTS react to a variety of foods and have issues with digestion. Commons triggers and allergies include gluten, dairy, and processed carbohydrates. Eating smaller, more frequent meals may help as well, by causing less blood to flow into your abdomen and pool during digestion.
-Possibly avoid alcohol and caffeine: alcohol dilates the blood vessels (can increase pooling) and dehydrates – both of which are a bad idea for POTS patients. Some can tolerate alcohol in moderate amounts, but use extreme caution and make sure to hydrate well when consuming alcohol. Caffeine can cause tachycardia, so take care when consuming it. Some actually people benefit from the rise in blood pressure from caffeine, as everyone is different.
-Elevate the head of the bed: Some patients benefit from elevating their bed to help recondition the body at night by not lying flat. This also helps with fluid retention and prevents acid reflux. Propping up the bed with bricks or books is the most common way to do this. Take care to check with your physician, as some patients have extreme blood pooling or other medical conditions that may be contraindicated for this option.
Quality of Life & Outlook:
POTS patients’ quality of life can vary greatly, due to the many things that cause POTS. It is not contagious, is not life threatening (though some rare underlying causes may be), and generally improves with time and treatment adjustments. It can be extremely disabling though, and frustrating for patients and caregivers to cope with, especially when first diagnosed. Symptoms can change from day to day, and hour to hour, and this can make it difficult for physicians to treat. Treatment usually focuses on managing the symptoms and underlying causes, and there is no cure for POTS per say, since it is a syndrome (and not a disease). POTS can also mimic anxiety, and since many people with POTS are young and “healthy looking,” they may be misdiagnosed as having a panic or anxiety disorder for extended periods of time. POTS is also not widely known (which we are changing!), and this can make finding treatment a challenge.
Many teens outgrow POTS or learn to live a highly functional life while managing symptoms. For some, POTS may go into remission and then flare up again, often with new and confusing symptoms. Unfortunately, for an estimated 25% of the POTS population, quality of life is compared to those with Congestive Heart Failure or Chronic Obstructive Pulmonary Disease. This severely limits day to day activities such as work, school, and other lifestyle issues such as hygiene and childcare. There are people with POTS that are forced to use a wheelchair to function.
The most recent and longest study (8-10 year follow up) of outcomes of POTS patients (Sousa A. et al, 2011) stated that out of 16 patients, 10 were completely without symptoms, and only 2 had what was considered "severe" and debilitating POTS with daily episodes. This gives us hope that more studies will be done, and we will continue to confirm that most POTS patients do get better with time and appropriate treatment. There IS exciting research taking place, and many more resources for patients and families as they adjust to life with POTS. Find links to many of them on our website, including listings of support groups, how to connect with others, tips and tools for patients, and various resources for coping with POTS.
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13. Postural tachycardia syndrome and anxiety disorders. Editors Note by Svetlana Blitshteyn, Clinical Assistant Professor of Neurology State University of New York at Buffalo School of Medicine and Biomedical Sciences.
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Written by Claire Martin, President of Dysautonomia SOS.
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