Postural orthostatic tachycardia syndrome

Información en español Title

Not a rare disease

Other Names:

POTS; Postural tachycardia syndrome; Chronic othostatic intolerance

Categories:

Heart Diseases; RDCRN

Summary Summary

Postural orthostatic tachycardia syndrome (POTS) is a condition characterized by too little blood returning to the heart when moving from a lying down to a standing up position (orthostatic intolerance). Orthostatic Intolerance causes lightheadedness or fainting that can be eased by lying back down. In people with POTS, these symptoms are also accompanied by a rapid increase in heart rate.^[1] Although POTS can affect men and women of all ages, most cases are diagnosed in women between the ages of 15 and 50.^[1] The cause of POTS is unknown. However, episodes often begin after major surgery, trauma, or a viral illness. In women, episodes may also begin after pregnancy and the symptoms may worsen or the number of episodes may increase right before menstruation.^[1]^[2] The goal of treatment is to increase blood flow and improve circulatory problems that may be causing POTS.^[1]^[3]^[4]

Last updated: 1/23/2017

Symptoms Symptoms

Postural orthostatic tachycardia syndrome (POTS) is characterized by orthostatic intolerance and a rapid increase in heart rate.^[1] People with POTS often have hypovolemia (low blood volume) and high levels of plasma norepinephrine while standing, reflecting increased sympathetic nervous system activation. Approximately half of affected people have a small fiber neuropathy that impacts their sudomotor nerves (those that activate the sweat glands). Some individuals develop a reddish-purple color in the legs when they stand, thought to be due to blood pooling or poor circulation. The color change subsides upon returning to a reclined position.^[5]

Other symptoms that have been reported in people with POTS include:^[2]^[4]^[3]^[5]

Blurred vision
Lightheadedness, dizziness or fainting
Heart palpitations
Headache
Poor concentration
Tiredness
Gastrointestinal symptoms (for example, nausea, cramps, bloating, constipation, diarrhea)
Shortness of breath
Head, neck or chest discomfort
Weakness
Sleep disorders
Difficulty exercising
Anxiety
Coldness or pain in the extremities

While the underlying causes of POTS are still being studied, a number of underlying conditions have been associated with POTS or with symptoms seen in POTS.^[5] More information about these conditions can be viewed on Dysautonomia International's website.

Last updated: 2/1/2017

Cause Cause

The cause of postural orthostatic tachycardia syndrome (POTS) is poorly understood. However, episodes often begin after a pregnancy, major surgery, trauma, or a viral illness and may increase right before a menstrual period.^[1]

Many researchers suspect that the condition may have more than one cause. The following abnormalities can be associated with POTS and may play a role in it's development:^[1]^[4]

Impaired function of nerves in certain muscles, especially those in the legs and feet.
An abnormal decrease in the amount of blood circulating in the body (caused, for example, by blood pooling in the abdomen and legs).
Too little blood returning to the heart when moving from a lying down to a standing up position.
Changes in heart and blood vessel function.
Abnormal regulation of blood pressure.
Increased fight-or-flight response.

Although most cases of POTS occur in people with no history of the condition in their family, some affected people report a family history of orthostatic intolerance. This suggests that in some cases, genetic factors may play a role in the development of POTS. Some studies also suggest that normal variations (polymorphisms) in certain genes (NOS3, ADRB2) may be associated with an increased risk of developing the condition. A mutation in the norepinephrine transporter gene (SLC6A2) has been found in one family with POTS.^[4]^[3]

Last updated: 1/20/2017

Inheritance Inheritance

Most cases of postural orthostatic tachycardia syndrome (POTS) do not appear to be inherited. However, some people with POTS do report a family history of orthostatic intolerance. This suggests that inherited factors may play a role in the development of POTS in some families.^[3]^[4]

Last updated: 1/21/2017

Diagnosis Diagnosis

A diagnosis of postural orthostatic tachycardia syndrome (POTS) is often suspected based on characteristic signs and symptoms. Additional testing can then be ordered to confirm the diagnosis. Many physicians will conduct a physical examination that includes measurement of blood pressure and heart rate while lying, sitting, and standing. A tilt table test, in which blood pressure and heart rate are checked while a person is safely moved from a lying down position to standing upright, may also be recommended.^[2]^[4]

Last updated: 1/21/2017

Treatment Treatment

Because research suggests that postural orthostatic tachycardia syndrome (POTS) has a variety of causes, no single treatment is effective for all affected people. Management of POTS aims to improve low blood pressure and problems with the heart and blood vessels that may be causing the condition. In some people with POTS, simple lifestyle changes such as adding extra salt to the diet, drinking more fluids, exercising, and avoiding factors that make the condition worse, may lessen symptoms. Certain medications may also be helpful in treating POTS.^[1]^[2]^[4] Some researches suggest volume repletion and fludrocortisone (0.05 to 0.2 mg per day) as the first line of therapy. Some patients may benefit from midodrine or beta blocking agents. Other treatment options remain under investigation, and further confirmation of benefit is needed before they can be recommended.^[4]

Last updated: 1/20/2017

Prognosis Prognosis

The long-term outlook (prognosis) for people with postural orthostatic tachycardia syndrome (POTS) is generally good, but may be poor in some cases.^[5]^[1]^[4] While many people have fairly mild symptoms and can continue with regular daily activities, others may be severely affected with limited abilities.^[5] Some people with POTS report significantly improved symptoms within a year,^[6] while others don't improve with treatment and may worsen over time.^[5]

With proper lifestyle changes (for example, exercise and diet) and medical treatments, many affected people see an improvement in symptoms and quality of life. In some cases, people with POTS may even become symptom-free over time.^[1]^[4]

Last updated: 2/1/2017

Statistics Statistics

According to Dysautonomia International, POTS is estimated to affect 1 out of 100 teenagers and, including adult patients, a total of 1 million to 3 million Americans.^[7] It is most frequently seen in young women, often less than 35 years of age.^[8] The female to male ratio is 5:1. It is common after stress such as sepsis, pregnancy, fever, surgery or trauma.^[9]

Last updated: 2/1/2017

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Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Postural orthostatic tachycardia syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

The Autonomic Rare Diseases Clinical Research Consortium is a team of doctors, nurses, research coordinators, and research labs throughout the U.S., working together to improve the lives of people with many types of autonomic disorders through research. The Autonomic Rare Diseases Clinical Research Consortium has a registry for patients who wish to be contacted about clinical research opportunities.

For more information on the registry see: RDCRN Autonomic Disorders Consortium Contact Registry

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Dysautonomia Information Network
PO Box 10057
Swanzey, NH 03446, NH 03446
E-mail: webmaster@dinet.org
Website: https://www.dinet.org/
Dysautonomia International
P.O. Box 596
East Moriches, NY 11940
E-mail: info@DysautonomiaInternational.org
Website: http://www.dysautonomiainternational.org/
Standing Up To POTS
1994 Ballentine Pike
Springfield, OH 45502
E-mail: info@standinguptopots.org
Website: http://standinguptopots.org/

Do you know of an organization? We want to hear from you.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

Dysautonomia International offers an information page on Postural orthostatic tachycardia syndrome. Please click on the link to access this resource.
The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
PubMed is a searchable database of medical literature and lists journal articles that discuss Postural orthostatic tachycardia syndrome. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

My daughter was diagnosed basilar migraine several months ago. I feel she could also have POTS. Can the two conditions co-exist? See answer

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References References

NINDS Postural Tachycardia Syndrome Information Page. National Institute of Neurological Disorders and Stroke. December 2015; https://www.ninds.nih.gov/Disorders/All-Disorders/Postural-Tachycardia-Syndrome-Information-Page.
Postural Tachycardia Syndrome (POTS). Autonomic Disorders Consortium. http://www.rarediseasesnetwork.org/ARDCRC/patients/learnmore/POTS/index.htm.
Mohini Gurme, MD. Idiopathic Orthostatic Hypotension and other Autonomic Failure Syndromes. Medscape Reference. November 2015; http://emedicine.medscape.com/article/1154266-overview.
Kaufmann H & Freeman R. Postural tachycardia syndrome. UpToDate. March 2015; http://www.uptodate.com/contents/postural-tachycardia-syndrome.
Postural Orthostatic Tachycardia Syndrome. Dysautonomia International. http://www.dysautonomiainternational.org/page.php?ID=30. Accessed 3/6/2016.
Kimpinski K, Figueroa JJ, Singer W, Sletten DM, Iodice V, Sandroni P, Fischer PR, Opfer-Gehrking TL, Gehrking JA, Low PA. A prospective, 1-year follow-up study of postural tachycardia syndrome. Mayo Clin Proc. August 2012; 87(8):746-752.
What is dysautonomia?. Dysautonomia International. 2012; http://www.dysautonomiainternational.org/page.php?ID=34.
Autonomic Disorders Information for Patients and Families. Autonomic Disorders Consortium. https://www.rarediseasesnetwork.org/cms/autonomic/Learn-More/Disorder-Definitions.
A K Agarwal, R Garg, A Ritch, and P Sarkar. Postural orthostatic tachycardia syndrome. Postgrad Med J. July, 2007; 83(981):478-480. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2600095/#__sec1title.