Postural orthostatic tachycardia syndrome: The Mayo Clinic experience

Mark J. Thieben, Paola Sandroni, David M. Sletten, Lisa M. Benrud-Larson, Robert D. Fealey, Steven Vernino, Vanda A. Lennon, Win Kuang Shen, Phillip A. Low

Research output: Contribution to journalArticle

277 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)308-313
Number of pages6
JournalMayo Clinic proceedings
Volume82
Issue number3
DOIs
StatePublished - Mar 2007

ASJC Scopus subject areas

  • Medicine(all)

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    Thieben, M. J., Sandroni, P., Sletten, D. M., Benrud-Larson, L. M., Fealey, R. D., Vernino, S., Lennon, V. A., Shen, W. K., & Low, P. A. (2007). Postural orthostatic tachycardia syndrome: The Mayo Clinic experience. Mayo Clinic proceedings, 82(3), 308-313. https://doi.org/10.4065/82.3.308