Postural orthostatic tachycardia syndrome

Not to be confused with Pott disease.

Postural orthostatic tachycardia syndrome
Other namesPOTS
Acrocyanosis in a male Norwegian POTS patient. The patient's legs appear red and purple due to the condition.
Tachycardia after a postural change in a patient with POTS
SpecialtyCardiology, neurology
SymptomsMore often with standing: lightheadedness, syncope, trouble thinking, tachycardia, weakness,[1] palpitations, heat intolerance, acrocyanosis
Usual onsetMost common (modal) age of onset is 14 years[2]
TypesNeuropathic POTS, Hyperadrenergic POTS, Secondary POTS.
CausesAntibodies against the Alpha 1 adrenergic receptor and muscarinic acetylcholine M4 receptor[3][4][5]
Risk factorsFamily history,[1] Ehlers Danlos Syndrome or Mast Cell Activation Syndrome
Diagnostic methodAn increase in heart rate by 30 beats/min with standing[1]
Differential diagnosisDehydration, heart problems, adrenal insufficiency, epilepsy, Parkinson's disease,[6] anemia
TreatmentAvoiding factors that bring on symptoms, increasing dietary salt and water, compression stockings, exercise, medications [1]
MedicationOff label Medications: Beta blockers, Ivabradine, midodrine, and fludrocortisone.[1]
Prognosisc. 90% improve with treatment,[7] 25% of patients unable to work[8]
Frequency~ 1,000,000 ~ 3,000,000 (US)[9]

Postural orthostatic tachycardia syndrome (POTS) is a condition characterized by an abnormally large increase in heart rate upon sitting up or standing.[1] POTS is a disorder of the autonomic nervous system that can lead to a variety of symptoms,[10] including lightheadedness, brain fog, blurred vision, weakness, fatigue, headaches, heart palpitations, exercise intolerance, nausea, diminished concentration, tremulousness (shaking), syncope (fainting), coldness or pain in the extremities, numbness or tingling in the extremities, chest pain, and shortness of breath.[1][11][12] Other conditions associated with POTS include myalgic encephalomyelitis/chronic fatigue syndrome, migraine headaches, Ehlers–Danlos syndrome, asthma, autoimmune disease, vasovagal syncope, and mast cell activation syndrome.[10][13] POTS symptoms may be treated with lifestyle changes such as increasing fluid, electrolyte, and salt intake, wearing compression stockings, gentler and slow postural changes, avoiding prolonged bedrest, medication, and physical therapy.

The causes of POTS are varied.[14] It may develop after a viral infection, surgery, trauma, autoimmune disease, or pregnancy.[7] It has been shown to emerge in previously healthy patients after COVID-19,[15][16] or possibly in rare cases after COVID-19 vaccination, though causative evidence is limited and further study is needed.[17] POTS is more common among people who got infected with SARS-CoV-2 than among those who got vaccinated against COVID-19.[18] Risk factors include a family history of the condition.[1] POTS in adults is characterized by a heart rate increase of 30 beats per minute within ten minutes of standing up, accompanied by other symptoms.[1] This increased heart rate should occur in the absence of orthostatic hypotension (>20 mm Hg drop in systolic blood pressure)[19] to be considered POTS, though some patients with POTS do not show any changes in blood pressure upon standing. A spinal fluid leak (called spontaneous intracranial hypotension) may have the same signs and symptoms as POTS and should be excluded.[20] Prolonged bedrest may lead to multiple symptoms, including blood volume loss and postural tachycardia.[21] Other conditions that can cause similar symptoms, such as dehydration, orthostatic hypotension, heart problems, adrenal insufficiency, epilepsy, and Parkinson's disease, must not be present.[6]

Treatment may include avoiding factors that bring on symptoms, increasing dietary salt and water, small and frequent meals,[22] avoidance of immobilization,[22] wearing compression stockings, and medication.[23][24][1][25] Medications used may include beta blockers,[26] pyridostigmine,[27] midodrine[28] and fludrocortisone.[1] More than 50% of patients whose condition was triggered by a viral infection get better within five years.[7] About 80% of patients have symptomatic improvement with treatment, while 25% are so disabled they are unable to work.[8][7] A retrospective study on patients with adolescent-onset has shown that five years after diagnosis, 19% of patients had full resolution of symptoms.[29]

It is estimated that 1–3 million people in the United States have POTS.[30] The average age for POTS onset is 20, and it occurs about five times more frequently in females than in males.[1]

  1. ^ a b c d e f g h i j k l Benarroch EE (December 2012). "Postural tachycardia syndrome: a heterogeneous and multifactorial disorder". Mayo Clinic Proceedings. 87 (12): 1214–1225. doi:10.1016/j.mayocp.2012.08.013. PMC 3547546. PMID 23122672.
  2. ^ Cite error: The named reference pmid30861229 was invoked but never defined (see the help page).
  3. ^ Miller AJ, Doherty TA (October 2019). "Hop to It: The First Animal Model of Autoimmune Postural Orthostatic Tachycardia Syndrome". Journal of the American Heart Association. 8 (19): e014084. doi:10.1161/JAHA.119.014084. PMC 6806054. PMID 31547756.
  4. ^ Gunning WT, Kvale H, Kramer PM, Karabin BL, Grubb BP (September 2019). "Postural Orthostatic Tachycardia Syndrome Is Associated With Elevated G-Protein Coupled Receptor Autoantibodies". Journal of the American Heart Association. 8 (18): e013602. doi:10.1161/JAHA.119.013602. PMC 6818019. PMID 31495251.
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  17. ^ Blitshteyn S, Fedorowski A (12 December 2022). "The risks of POTS after COVID-19 vaccination and SARS-CoV-2 infection: it's worth a shot". Nature Cardiovascular Research. 1 (12): 1119–1120. doi:10.1038/s44161-022-00180-z. ISSN 2731-0590. S2CID 254617706.
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