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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Chronic orthostatic symptoms with a dramatic rise in heart rate (> 30 bpm) or > 120 bpm on standing (within 10 minutes) with no orthostatic hypotension (frank syncope is rare)

  • Evidence of dysautonomia on functional testing

GENERAL CONSIDERATIONS

  • May be primary or secondary to another disorder

  • Many patients have a dramatic increase in heart rates up to 120–170 bpm within 2–5 minutes of upright tilt

  • Many patients may have been previously labeled as suffering from panic attacks or chronic anxiety

  • May be familial

  • Norepinephrine reuptake transporter protein gene mutation leading to excessive serum epinephrine is identified in familial form

  • Autonomic neuropathies and acetylcholine receptor antibodies in the peripheral autonomic ganglia have been seen in postfebrile illness (postviral) postural orthostatic tachycardia syndrome (POTS)

  • Possible link between Ehlers-Danlos syndrome III and POTS

  • May occur in diabetes mellitus, amyloidosis, sarcoidosis, alcoholism, chemotherapy (particularly with the vinca alkaloids), heavy metal poisoning, Sjögren’s syndrome, and lupus erythematosus

  • May occur after pregnancy

  • May be a paraneoplastic manifestation

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Exercise intolerance

  • Extreme fatigue

  • Lightheadedness

  • Diminished concentration

  • Tremulousness

  • Nausea, headache

  • Near syncope

  • Occasionally syncope

PHYSICAL EXAM FINDINGS

  • Generally normal other than orthostatic tachycardia

DIFFERENTIAL DIAGNOSIS

  • Prolonged immobilization

  • Significant rapid weight loss

  • Orthostatic hypotension

  • Neurocardiogenic syndrome

  • Chronic fatigue syndrome

  • Other neuropathies (eg, syringomyelia)

  • Supraventricular tachyarrhythmias

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • CBC, basic metabolic panel

  • Fasting glucose

  • In appropriate situations: serum protein electrophoresis, paraneoplastic panel

  • Antinuclear antibody test

ELECTROCARDIOGRAPHY

  • Usually normal in supine resting position

IMAGING STUDIES

  • Echocardiogram to exclude structural heart disease

DIAGNOSTIC PROCEDURES

  • Tilt-table testing shows an excessive increase in heart rate without any or minimal drop in blood pressure

TREATMENT

CARDIOLOGY REFERRAL

  • Patients not responding to conservative measures may benefit from cardiac electrophysiology consultation

HOSPITALIZATION CRITERIA

  • If there is syncope, brief hospitalization may be required

  • POTS generally can be managed as outpatient

MEDICATIONS

  • Avoid dehydration, extreme heat, and alcohol consumption

  • Elevate head end of the bed to help condition orthostatic stress

  • Aerobic exercise and strength training (if deconditioned, consider water exercise)

  • Patients’ responses to individual drugs vary substantially

  • Combination therapy may be better

  • Salt and fluid replacement

  • Fludrocortisone 0.2 mg/day PO

  • Autonomic agents: alpha1-adrenergic agonists (midodrine 10 mg 3 times per day), cardioselective beta blockers (metoprolol 50 mg/day)

  • Selective serotonin reuptake inhibitor (SSRI) such as paroxetine 20 mg/day

  • Epoetin alpha 50–100 U SC or IV, 3 times per week until desired hematocrit is attained

  • Patient and family education

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