Postural Orthostatic Tachycardia Syndrome (POTS)

• Postural Orthostatic Tachycardia Syndrome, and Overlapping Syndrome (POTS) was first defined in the adult population as an increase in heart rate by more than 30 bpm or an increase to heart rate greater than 120 bpm within 10 minutes when moving from supine to the upright position (1)
  • patients with similar clinical and physiologic profiles were later identified in the teenager population
    • patients with POTS are predominantly young women ranging in age groups from menarche to menopause (2)
      • predominantly affects females (approximately 90% of cases), with peak incidence from ages 13 to 29 years
      • affects an estimated 0.1% to 1% of the US population
      • consensus criteria define POTS by chronic symptoms of orthostatic intolerance accompanied by a sustained heart rate increase of at least 30 beats/min (≥40 beats/min in adolescents aged 12-19 years) within 10 minutes of standing or head-up tilt, in the absence of orthostatic hypotension, defined as a decrease in either systolic blood pressure of at least 20 mm Hg or diastolic blood pressure of at least 10 mm Hg with positional change
  • Clinical features
    • development of tachycardia and other symptoms during upright position, and relieved by recumbence, are central features of this syndrome complex (3,4)
    • symptoms always include dizziness and light-headedness
    • frank syncope can occur at times, although not a predominant feature
    • other possible associated symptoms include:
      • headache, tunnel vision, fatigue, neurocognitive impairment, exercise intolerance, weakness, dyspnea, tremulousness, nausea, chest or abdominal pain, sweating, anxiety, and palpitations
    • intensity and frequency of symptoms is often variable and in some instances may occur and persist even when the patient is supine
  • Pathophysiology:
    • many of the symptoms in the upright position appear to be related to a reduced cerebral blood flow
      • during upright posture, some patients increase depth of respiration, causing hypopnea and cerebral vasoconstriction, resulting in symptoms of light-headedness, visual blurring, and weakness
      • underlying cause of orthostatic hyperpnoea remains elusive, but is likely multifactorial
        • patients with POTS do not have significant orthostatic hypotension or overt systemic autonomic neuropathy
        • several mechanisms of POTS have been suggested, including length-dependent autonomic neuropathy, beta-receptor supersensitivity, alpha-receptor hypersensitivity or hyposensitivity, altered sympatho-parasympathetic balance, brain stem dysregulation, idiopathic hypovolemia, and excessive venous pooling
  • Diagnosis (2,5):
    • need a complete medical history to evaluate the triggers, time of onset, severity of orthostatic intolerance, possible associated nonorthostatic symptoms, and precipitating or aggravating factors
    • initial evaluation should exclude other conditions that cause sinus tachycardia, including thyroid disease, adrenal insufficiency, pheochromocytoma, cardiomyopathy, valvular heart disease, congenital heart disease, chronic lung disease, medication effects (e.g., stimulants, norepinephrine reuptake inhibitors, and diuretics), anemia, and dehydration
    • requires comprehensive cardiac and neurologic examinations
    • head-up tilt test with noninvasive beat-to-beat hemodynamic monitoring
  • Treatment:
    • requires specialist advice
    • first-line treatment includes (2)
      • nonpharmacological
        • strategies to improve cardiac preload, including increased fluid and sodium intake, lower-body compression garments;
        • avoidance of heat exposure, dehydration, and prolonged standing;
        • structured supervised aerobic training
      • pharmacological therapies
        • should be individualized and may include:
          • β-blockers
          • ivabradine
          • midodrine
          • fludrocortisone
          • pyridostigmine
        • patients often respond to but are sometimes highly sensitive to beta-antagonists
          • a nonselective beta-blocker such as propranolol can be started at low dose, titrated upward according to heart rate response and tolerance over 2-4 weeks

Patient Support organisations and further information about POTS:

• www.stars.org.uk
  
  
• www.potsuk.org

Reference:

• 1. Schondorf R, Low P. Idiopathic postural orthostatic tachycardia syndrome: An attenuated form of acute pan dysautonomia. Neurology 1993; 43: 132-137.
• 2. Chung TH, Raj SR. Postural Orthostatic Tachycardia Syndrome (POTS): A Review. JAMA. Published online August 24, 2026.
• 3. Stewart JM, Gewitz MH, Weldon A, Munoz J. Patterns of orthostatic intolerance: The orthostatic tachycardia syndrome and adolescent chronic fatigue. J Pediatr 1999; 135: 218-225.
• 4. Tanaka H et al. Instantaneous orthostatic hypotension in children and adolescents: a new entity of orthostatic intolerance. Pediatr Res 1999; 46: 691-696
• 5. Safavi-Naeini P, Razavi M. Postural Orthostatic Tachycardia Syndrome. Tex Heart Inst J. 2020 Feb 1;47(1):57-59. doi: 10.14503/THIJ-19-7060. PMID: 32148459; PMCID: PMC7046364