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Postural Orthostatic Tachycardia Syndrome

  • Postural orthostatic tachycardia syndrome is characterized by orthostatic tachycardia in the absence of orthostatic hypotension and additional multisystem manifestations

  • Common symptoms include palpitations, lightheadedness, extreme fatigue, exercise intolerance, headache, and mental clouding

Terminology

  • Outdated terms for postural orthostatic tachycardia syndrome include Da Costa syndrome,(1) soldier’s heart, mitral valve prolapse syndrome,(2) neurocirculatory asthenia, orthostatic tachycardia, and orthostatic intolerance (3)

  • Orthostatic hypotension: sustained decrease in systolic blood pressure 20 mm Hg and higher or diastolic blood pressure 10 mm Hg and higher within 3 minutes of standing or on head-up tilt test (4)

  • Initial orthostatic hypotension: transient drop in systolic blood pressure of 40 mm Hg or more, or diastolic blood pressure 20 mm Hg or more, which occurs within 15 seconds of standing, with blood pressure recovery within 45 seconds of standing (5,6)

  • Peripheral acrocyanosis (postural orthostatic tachycardia syndrome feet): dependent cyanosis characterized by dark red-blue discoloration of the feet

  • Orthostatic intolerance: a constellation of symptoms that include frequent, recurrent, or persistent lightheadedness, palpitations, tremulousness, generalized weakness, blurred vision, exercise intolerance, and fatigue on standing. These symptoms can occur with or without orthostatic tachycardia or syncope. Individuals with orthostatic intolerance have 1 or more of these symptoms associated with reduced ability to maintain upright posture

Epidemiology

  • Outdated terms for postural orthostatic tachycardia syndrome include Da Costa syndrome,(1) soldier’s heart, mitral valve prolapse syndrome,(2) neurocirculatory asthenia, orthostatic tachycardia, and orthostatic intolerance (3)

  • Orthostatic hypotension: sustained decrease in systolic blood pressure 20 mm Hg and higher or diastolic blood pressure 10 mm Hg and higher within 3 minutes of standing or on head-up tilt test (4)

  • Initial orthostatic hypotension: transient drop in systolic blood pressure of 40 mm Hg or more, or diastolic blood pressure 20 mm Hg or more, which occurs within 15 seconds of standing, with blood pressure recovery within 45 seconds of standing (5,6)

  • Peripheral acrocyanosis (postural orthostatic tachycardia syndrome feet): dependent cyanosis characterized by dark red-blue discoloration of the feet

  • Orthostatic intolerance: a constellation of symptoms that include frequent, recurrent, or persistent lightheadedness, palpitations, tremulousness, generalized weakness, blurred vision, exercise intolerance, and fatigue on standing. These symptoms can occur with or without orthostatic tachycardia or syncope. Individuals with orthostatic intolerance have 1 or more of these symptoms associated with reduced ability to maintain upright posture

Quality of Life in Postural Orthostatic Tachycardia Syndrome

  • Patients with postural orthostatic tachycardia syndrome are often young females of childbearing age whose work productivity and quality of life are negatively affected (10 )

  • Functional impairment in patients with postural orthostatic tachycardia syndrome has been reported to be similar to that in chronic obstructive pulmonary disease and congestive heart failure (11)

  • Over 25% of postural orthostatic tachycardia syndrome patients are unable to work as a result of their disability (11)

  • Postural orthostatic tachycardia syndrome patients describe poorer sleep quality, more daytime sleepiness, less sleep efficiency,(12) greater fatigue, and substandard quality of life compared to healthy subjects (10)

Postural Orthostatic Tachycardia Syndrome Triggers

  • Patient-reported survey data show that in approximately 40% of postural orthostatic tachycardia syndrome patients, symptoms began following acute stressors (eg, presumed viral illness [most common], pregnancy, major surgery)

  • Symptoms develop more insidiously in the remainder of patients (7)

  • Some patients have developed postural orthostatic tachycardia syndrome following infection with the SARS-CoV2 virus.(13) This has been termed long-COVID postural orthostatic tachycardia syndrome. The exact mechanisms are uncertain, but they are presumed to be a sequelae of the immune response to the virus

  • A small observational study found that about 80% of female patients report an exacerbation of symptoms around menstruation (14)

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