Original contributionPostural orthostatic tachycardia syndrome and general anesthesia: a series of 13 cases☆
Introduction
Orthostatic intolerance defines a group of symptoms characterized by cerebral hypoperfusion and/or sympathetic activation that appear on standing upright and remit in the supine position. Patients may complain of headache, nausea, abdominal pain, lightheadedness, diminished concentration, syncope, anxiety, weakness, fatigue, exercise intolerance, palpitations, dyspnea, and chest pain. Some patients also may have generalized complaints, including fatigue, sleep disturbance, and migraine headaches. Orthostatic intolerance may be diagnosed on the basis of these symptoms only and does not require any hemodynamic abnormalities. The postural orthostatic tachycardia syndrome (POTS) is characterized by symptoms of orthostatic intolerance associated with excessive tachycardia while in the upright position, without orthostatic hypotension. Diagnostic criteria include a) a sustained increase in heart rate (HR) of 30 beats per minute (bpm) or greater during 10 minutes of assuming an upright position, b) no associated hypotension, and c) symptoms of orthostatic intolerance, which must be present for at least three months [1]. In severe forms of the disease, HR may increase to more than 120 bpm on standing.
Little is known about the anesthetic implications of POTS. Anesthetic techniques have been described for other forms of autonomic dysfunction [2], [3], [4], [5], [6], but the optimal anesthetic management of a patient with POTS is uncertain. Few investigators have described the use of regional techniques for labor analgesia or cesarean delivery in obstetrical patients [7], [8], [9]. In one case of cesarean delivery [8], epidural anesthesia was converted to general anesthesia due to patient discomfort and tachycardia. The authors stated that “the patient was more cardiovascularly stable”. A single case report described the management of general anesthesia in a patient with orthostatic intolerance syndrome associated with postural tachycardia and blood pressure (BP) lability. This case showed a possible intraoperative complication in patients with orthostatic intolerance, namely, wide swings in mean arterial blood pressure (MAP) and HR despite adequate preoperative intravenous (IV) hydration [10].
The aims of this study were to investigate the perioperative management of patients with POTS and to identify perioperative complications, including hemodynamic instability and unplanned admission to the intensive care unit (ICU). We hypothesized that patients with POTS would have an increased occurrence of hypotension and increased need for pressors (POTS pts have sympathetic denervation of the legs with loss of vasomotor tone), cardiovascular collapse (POTS pts have an unstable adrenergic system and baroreflex dysfunction), and arrhythmias (POTS pts have symptoms of sympathetic overactivity and elevated plasma norepinephrine levels).
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Materials and methods
Following Mayo Clinic Institutional Review Board approval, the medical records of patients diagnosed with POTS at the Mayo Clinic between January 1, 1993 and December 31, 2003, who subsequently underwent general anesthesia for an elective procedure, were reviewed. Cases of POTS were previously defined and identified by Thieben et al. through a manual chart review of patients seen by two Mayo Clinic POTS specialists [1]. Briefly, POTS was diagnosed if patients met the following inclusion
Results
A total of 13 patients (12 women, one man) underwent surgical procedures following the POTS diagnosis. Demographic data are summarized in Table 1. Mean age at the time of POTS diagnosis was 28 ± 13 years (range 15-55 yrs). The median time from POTS diagnosis to surgery was one year (range 0-10 yrs). Procedures and anesthetic details are summarized in Table 2, Table 3. The postoperative course is summarized in Table 4.
The composite autonomic severity score was equal to or less than 3 for all
Discussion of results
We report a series of 13 surgical procedures during general anesthesia in patients with POTS. The major findings of this case series include the observation that three patients had prolonged intraoperative hypotension requiring vasopressor medications and additional fluid boluses during a variety of procedures that differed in stress level. Of note, all patients recovered without additional complications or long-standing effects from hypotension. PACU LOS was not excessive and there were no
Acknowledgments
The authors sincerely thank Nisha Charkoudian, PhD, and Michael Joyner, MD, for their advice and expertise in the production of this manuscript.
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Supported by NIH grant NS32352, National Institutes of Health, Bethesda, MD, USA; and the Department of Anesthesiology, Mayo Clinic and the Mayo Foundation, Rochester, MN, USA.