Advertisement
Mayo Clinic Proceedings Home

Postural Orthostatic Tachycardia Syndrome: The Mayo Clinic Experience

      OBJECTIVE

      To evaluate the prevalence and pathogenetic mechanisms of postural orthostatic tachycardia syndrome (POTS).

      PATIENTS AND METHODS

      We reviewed the medical records of patients with POTS seen at the Mayo Clinic in Rochester, Minn, from January 1, 1993, through December 31, 2003. All patients were required to have had a full autonomic reflex screen. The results of the following additional tests were evaluated: thermoregulatory sweat test, plasma catecholamine measurement, serum ganglionic (α3) acetylcholine receptor antibody detection, and 24-hour urinary sodium measurement.

      RESULTS

      We identified 152 patients (86.8% female; mean ± SD age, 30.2±10.3 years) with a mean duration of symptoms of 4.1 years. The mean orthostatic heart rate increment was 44 beats/min. Half the patients had sudomotor abnormalities (apparent on both the quantitative sudomotor axon reflex test and thermoregulatory sweat test), and 34.9% had significant adrenergic impairment, indicating that at least half of the patients had a neuropathic pattern of POTS. In 13.8% of patients, onset was subacute, and ganglionic acetylcholine receptor antibody was detected in 14.6%, suggesting an autoimmune origin in at least 1 in 7 patients. Hyperadrenergic status was documented in 29.0% of patients (standing plasma norepinephrine level ≥600 pg/mL), and at least 28.9% were presumably hypovolemic (24-hour urinary sodium level <100 mEq/24h). The lack of correlation between urinary sodium and standing norepinephrine levels suggests that mechanisms other than hypovolemia accounted for the hyperadrenergic state.

      CONCLUSION

      Our findings suggest a neuropathic basis for at least half the cases of POTS and that a substantial percentage of cases may be autoimmune. Hyperadrenergic and hypovolemic correlates are likely compensatory or exacerbating.
      AChR (acetylcholine receptor), CASS (composite autonomic severity score), IQR (interquartile range), POTS (postural orthostatic tachycardia syndrome), QSART (quantitative sudomotor axon reflex test)
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic and PersonalCorporate R&D Professionals
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Mayo Clinic Proceedings
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      REFERENCES

        • Schondorf R
        • Low PA
        Idiopathic postural orthostatic tachycardia syndrome: an attenuated form of acute pandysautonomia?.
        Neurology. 1993; 43: 132-137
        • 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
        • Jacob G
        • Robertson D
        • Mosqueda-Garcia R
        • Ertl AC
        • Robertson RM
        • Biaggioni I
        Hypovolemia in syncope and orthostatic intolerance role of the renin-angiotensin system.
        Am J Med. 1997; 103: 128-133
        • Jacob G
        • Costa F
        • Shannon JR
        • et al.
        The neuropathic postural tachycardia syndrome.
        N Engl J Med. 2000; 343: 1008-1014
        • Streeten DH
        • Scullard TF
        Excessive gravitational blood pooling caused by impaired venous tone is the predominant non-cardiac mechanism of orthostatic intolerance.
        Clin Sci (Lond). 1996; 90: 277-285
        • Tani H
        • Singer W
        • McPhee BR
        • et al.
        Splanchnic-mesenteric capacitance bed in the postural tachycardia syndrome (POTS).
        Auton Neurosci. 2000; 86: 107-113
        • Novak V
        • Spies JM
        • Novak P
        • McPhee BR
        • Rummans TA
        • Low PA
        Hypocapnia and cerebral hypoperfusion in orthostatic intolerance.
        Stroke. 1998; 29: 1876-1881
        • Singer W
        • Spies JM
        • McArthur J
        • et al.
        Prospective evaluation of somatic and autonomic small fibers in selected autonomic neuropathies.
        Neurology. 2004; 62: 612-618
        • Vernino S
        • Low PA
        • Fealey RD
        • Stewart JD
        • Farrugia G
        • Lennon VA
        Autoantibodies to ganglionic acetylcholine receptors in autoimmune autonomic neuropathies.
        N Engl J Med. 2000; 343: 847-855
        • Low PA
        Autonomic nervous system function.
        J Clin Neurophysiol. 1993; 10: 14-27
        • Low PA
        • Caskey PE
        • Tuck RR
        • Fealey RD
        • Dyck PJ
        Quantitative sudomotor axon reflex test in normal and neuropathic subjects.
        Ann Neurol. 1983; 14: 573-580
        • Low PA
        • Denq JC
        • Opfer-Gehrking TL
        • Dyck PJ
        • O'Brien PC
        • Slezak JM
        Effect of age and gender on sudomotor and cardiovagal function and blood pressure response to tilt in normal subjects.
        Muscle Nerve. 1997; 20: 1561-1568
        • Low PA
        Composite autonomic scoring scale for laboratory quantification of generalized autonomic failure.
        Mayo Clin Proc. 1993; 68: 748-752
        • Fealey RD
        • Low PA
        • Thomas JE
        Thermoregulatory sweating abnormalities in diabetes mellitus.
        Mayo Clin Proc. 1989; 64: 617-628
        • El-Sayed H
        • Hainsworth R
        Salt supplementation increases plasma volume and orthostatic tolerance in patients with unexplained syncope.
        Heart. 1996; 75: 134-140
        • Stewart JM
        Pooling in chronic orthostatic intolerance: arterial vasoconstrictive but not venous compliance defects.
        Circulation. 2002; 105: 2274-2281
        • Freeman R
        • Lirofonis V
        • Farquhar WB
        • Risk M
        Limb venous compliance in patients with idiopathic orthostatic intolerance and postural tachycardia.
        J Appl Physiol. 2002; 93: 636-644
        • Stewart JM
        Microvascular filtration is increased in postural tachycardia syndrome.
        Circulation. 2003 Jun 10; 107 (Epub 2003 May 19.): 2816-2822
        • Gordon VM
        • Opfer-Gehrking TL
        • Novak V
        • Low PA
        Hemodynamic and symptomatic effects of acute interventions on tilt in patients with postural tachycardia syndrome.
        Clin Auton Res. 2000; 10: 29-33
        • Karas B
        • Grubb BP
        • Boehm K
        • Kip K
        The postural orthostatic tachycardia syndrome: a potentially treatable cause of chronic fatigue, exercise intolerance, and cognitive impairment in adolescents.
        Pacing Clin Electrophysiol. 2000; 23: 344-351
        • Stewart JM
        Autonomic nervous system dysfunction in adolescents with postural orthostatic tachycardia syndrome and chronic fatigue syndrome is characterized by attenuated vagal baroreflex and potentiated sympathetic vasomotion.
        Pediatr Res. 2000; 48: 218-226
        • Jordan J
        • Shannon JR
        • Diedrich A
        • Black BK
        • Robertson D
        Increased sympathetic activation in idiopathic orthostatic intolerance: role of systemic adrenoreceptor sensitivity.
        Hypertension. 2002; 39: 173-178
        • Razumovsky AY
        • DeBusk K
        • Calkins H
        • et al.
        Cerebral and systemic hemodynamics changes during upright tilt in chronic fatigue syndrome.
        J Neuroimaging. 2003; 13: 57-67