The following are key points to remember from this review article about postural orthostatic tachycardia syndrome (POTS):

1. POTS is a syndrome of orthostatic intolerance characterized by a heart rate increment of ≥30 bpm, often with standing heart rates >120 bpm, within 10 minutes of standing or head-up tilt, and in the absence of orthostatic hypotension (a decrease in systolic blood pressure [BP] of ≥20 mm Hg and/or decrease in diastolic BP of ≥10 mm Hg).
2. It is the most common form of orthostatic intolerance in young people (predominantly premenopausal women). Presyncope is much more common than syncope in POTS, but it is not infrequent that POTS coexists with episodes of neurally mediated (reflex) syncope.
3. Cardiovascular deconditioning is a universal feature in all POTS. Overlapping pathophysiological variants that may contribute to an individual’s susceptibility to develop POTS include peripheral autonomic neuropathy, excessive venous pooling, hypovolemia in the setting of volume dysregulation, hyperadrenergic states, mast cell activation disorders, and autoimmunity.
4. Chronic symptoms and comorbidities that cannot physiologically be explained by orthostatic intolerance or tachycardia, but are common in patients with POTS include chronic fatigue, dizziness, syncope, migraines, functional gastrointestinal disorders, chronic nausea, fibromyalgia, and joint hypermobility.
5. POTS should be differentiated from neurogenic orthostatic hypotension (NOH), which can occur in disorders such as multiple system atrophy, Parkinson disease, Lewy body dementia, pure autonomic failure, autoimmune autonomic ganglionopathy, and other autonomic neuropathies. With orthostatic hypotension, there should be at least a 20-point drop in systolic BP by 3 minutes of tilt and typically the heart rate increment is minimal.
6. Inappropriate sinus tachycardia (IST) is sometimes confused with POTS, especially because both occur in young women, but IST occurs independent of body position. The key to making a diagnosis of IST is ambulatory monitoring; IST patients, unlike patients with POTS, demonstrate relative night-time supine tachycardia.
7. Formal autonomic function testing is helpful to evaluate for other types of autonomic impairment and differentiate among POTS subtypes.
8. Nonpharmacological treatment is the mainstay therapy for POTS and includes:
   
   
   • Exercise conditioning with a recumbent bike, rowing machine, or swimming. This approach allows patients to exercise while avoiding the upright position and improves tolerance of the program. (Medications can be considered in patients with severe symptoms as a bridge to help minimize some symptoms and allow patients to initiate the exercise program. Such medications include propranolol, midodrine, pyridostigmine, fluodrocortisone.)
   • Increasing blood volume can be accomplished by drinking 3 liters of water per day and liberalizing salt intake by ingesting 5-10 g of sodium per day.
   • Avoidance of large and heavy meals, alcohol, and heat exposure.
   • Wearing compression stockings up to the top of the thighs or higher and abdominal binders and they must extend at least to the top of the thighs and preferably to the abdomen.
   • Sleeping with head of bed elevated and performance of physical counter maneuvers such as leg crossing and squatting.
   • Behavioral and cognitive therapy may be used to obtain long-term control of symptoms, particularly when anxiety, hypervigilance, or catastrophizing behaviors are present.

Clinical Topics: Arrhythmias and Clinical EP, Cardiovascular Care Team, Diabetes and Cardiometabolic Disease, Prevention, Sports and Exercise Cardiology, Vascular Medicine, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Exercise, Sleep Apnea

Keywords: Arrhythmias, Cardiac, Autoimmunity, Blood Pressure, Cardiovascular Deconditioning, Cognitive Behavioral Therapy, Dizziness, Exercise Therapy, Fibromyalgia, Gastrointestinal Diseases, Hypotension, Orthostatic, Hypovolemia, Lewy Body Disease, Midodrine, Migraine Disorders, Monitoring, Ambulatory, Multiple System Atrophy, Orthostatic Intolerance, Parkinson Disease, Postural Orthostatic Tachycardia Syndrome, Propranolol, Secondary Prevention, Swimming, Syncope, Tachycardia, Sinus

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