Postural orthostatic tachycardia syndrome
| Postural orthostatic tachycardia syndrome | |
|---|---|
| Other names | POTS |
| Tachycardia after a postural change in a patient with POTS | |
| Specialty | Cardiology, neurology |
| Symptoms | More often with standing: lightheadedness, syncope, trouble thinking, tachycardia, weakness, palpitations, heat intolerance, acrocyanosis |
| Usual onset | Most common (modal) age of onset is 14 years |
| Types | Neuropathic POTS, Hyperadrenergic POTS, Secondary POTS. |
| Causes | Antibodies against the Alpha 1 adrenergic receptor and muscarinic acetylcholine M4 receptor |
| Risk factors | Family history, Ehlers Danlos Syndrome, Mast Cell Activation Syndrome, or autoimmune conditions. Certain medications may also trigger POTS in certain individuals. In some people chemotherapy may trigger POTS. |
| Diagnostic method | An increase in heart rate by 30 beats/min with standing for those 18 and over and 40 BPM in those under 18. Many times autonomic testing (including but not limited to a tilt table test and QSART) are used to make an accurate diagnosis. In some cases, a simple measurement of heart rate before and after standing up from a supine position may be used to make a diagnosis when a complete tilt table test is unattainable. |
| Differential diagnosis | Dehydration, heart problems, adrenal insufficiency, epilepsy, Parkinson's disease, anemia |
| Treatment | Avoiding factors that bring on symptoms, increasing dietary salt and water, compression stockings, exercise, medications exercise programs designed for POTS patients, such as the CHOP protocol, can be especially helpful. Physical therapy with a knowledgeable therapist is also often recommended. |
| Medication | Off label Medications: Beta blockers, Ivabradine, midodrine, fludrocortisone, and Pyridostigmine. |
| Prognosis | c. 90% improve with treatment, 25% of patients unable to work |
| Frequency | ~ 1,000,000 ~ 3,000,000 (US) |
Postural orthostatic tachycardia syndrome (POTS) is a condition characterized by an abnormally large increase in heart rate upon sitting up or standing. POTS is a disorder of the autonomic nervous system that can lead to a variety of symptoms, including lightheadedness, brain fog, blurred vision, weakness, fatigue, headaches, heart palpitations, exercise intolerance, nausea, difficulty concentrating, tremulousness (shaking), syncope (fainting), coldness, pain, or numbness in the extremities, chest pain, and shortness of breath. Many symptoms are exacerbated with postural changes, especially standing up. Other conditions associated with POTS include myalgic encephalomyelitis/chronic fatigue syndrome, migraine headaches, Ehlers–Danlos syndrome, asthma, autoimmune disease, vasovagal syncope, chiari malformation, and mast cell activation syndrome. POTS symptoms may be treated with lifestyle changes such as increasing fluid, electrolyte, and salt intake, wearing compression stockings, gentle postural changes, exercise, medication, and physical therapy.
The causes of POTS are varied. In some cases, it develops after a viral infection, surgery, trauma, autoimmune disease, or pregnancy. It has also been shown to emerge in previously healthy patients after contracting COVID-19, in people with Long COVID (post-COVID-19 condition), about 30 % present with POTS-like orthostatic tachycardia, or possibly in rare cases after COVID-19 vaccination, though causative evidence is limited and further study is needed. POTS is more common among people who got infected with SARS-CoV-2 than among those who got vaccinated against COVID-19. Risk factors include a family history of the condition. 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. This increased heart rate should occur in the absence of orthostatic hypotension (>20 mm Hg drop in systolic blood pressure) to be considered POTS. A spinal fluid leak (called spontaneous intracranial hypotension) may have the same signs and symptoms as POTS and should be excluded. Prolonged bedrest may lead to multiple symptoms, including blood volume loss and postural tachycardia. 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.
Treatment may include:
- avoiding factors that bring on symptoms,
- increasing dietary salt and water,
- small and frequent meals,
- avoidance of immobilization,
- wearing compression stockings, and
- medication. Medications used may include:
More than 50% of patients whose condition was triggered by a viral infection get better within five years. About 80% of patients have symptomatic improvement with treatment, while 25% are so disabled they are unable to work. A retrospective study on patients with adolescent-onset has shown that five years after diagnosis, 19% of patients had full resolution of symptoms.
It is estimated that 1–3 million people in the United States have POTS. The average age for POTS onset is 20, and it occurs about five times more frequently in females than in males.