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For further information, see CMDT Part 24-05: Postural Orthostatic Tachycardia Syndrome

Key Features

  • Postural orthostatic tachycardia syndrome is more common in women than men and in patients between 20 and 50 years of age

  • Its pathophysiology is uncertain but may involve cardiac deconditioning

  • May be associated with joint hypermobility syndrome and mitral valve prolapse

  • May follow pregnancy, surgery, trauma, chemotherapy, vaccinations, or viral infections

  • Possible mechanisms

    • Neuropathic postural orthostatic tachycardia syndrome: Impaired peripheral vasoconstriction due to peripheral sympathetic denervation, leading to venous pooling in the legs upon standing and a compensatory tachycardia

    • Hyperadrenergic postural orthostatic tachycardia syndrome: An exaggerated sympathetic response to standing, with markedly elevated levels of plasma norepinephrine causing the tachycardia

    • Volume dysregulation postural orthostatic tachycardia syndrome: Hypovolemia, possibly from impaired function of the renin-angiotensin system and excessive mast cell activation leading to inappropriate release of histamine during physical activity

  • The long-term prognosis is unclear but approximately 50% of patients recover within 3 years

Clinical Findings

  • Orthostatic symptoms develop with a significant tachycardia within 10 minutes of standing

  • The orthostatic symptoms include

    • Tremulousness

    • Lightheadedness

    • Palpitations

    • Visual disturbances

    • Weakness

    • Fatigue

    • Anxiety

    • Hyperventilation

    • Nausea

  • Tachycardia is considered significant if there is

    • An increase of ≥ 30 beats/min or

    • A heart rate of ≥120 beats/min within 10 minutes of standing

Diagnosis

  • Clinical

  • Diagnosis requires absence of

    • Postural hypotension

    • An autonomic neuropathy

    • Other medical problems causing a tachycardia

Treatment

  • For postural orthostatic tachycardia syndrome due to volume dysregulation or neuropathic mechanisms, management may involve

    • Volume repletion, including a high salt diet and copious fluids

    • Postural and psychophysiologic training

    • A graduated exercise program

  • For patients with hyperadrenergic postural orthostatic tachycardia syndrome, pharmacotherapy may include

    • Propranolol, 10–40 mg three times daily

    • Phenobarbital, 15 mg in the morning, 60 mg at night

    • Clonidine, 0.2 mg twice daily

  • For patients with hypotension, the clinician may consider therapy with

    • Midodrine (2.5–10 mg three times daily) or

    • Fludrocortisone (0.1– 0.2 mg daily)

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