Elsevier

Mayo Clinic Proceedings

Volume 87, Issue 12, December 2012, Pages 1214-1225
Mayo Clinic Proceedings

Review
Postural Tachycardia Syndrome: A Heterogeneous and Multifactorial Disorder

https://doi.org/10.1016/j.mayocp.2012.08.013Get rights and content

Abstract

Postural tachycardia syndrome (POTS) is defined by a heart rate increment of 30 beats/min or more within 10 minutes of standing or head-up tilt in the absence of orthostatic hypotension; the standing heart rate is often 120 beats/min or higher. POTS manifests with symptoms of cerebral hypoperfusion and excessive sympathoexcitation. The pathophysiology of POTS is heterogeneous and includes impaired sympathetically mediated vasoconstriction, excessive sympathetic drive, volume dysregulation, and deconditioning. POTS is frequently included in the differential diagnosis of chronic unexplained symptoms, such as inappropriate sinus tachycardia, chronic fatigue, chronic dizziness, or unexplained spells in otherwise healthy young individuals. Many patients with POTS also report symptoms not attributable to orthostatic intolerance, including those of functional gastrointestinal or bladder disorders, chronic headache, fibromyalgia, and sleep disturbances. In many of these cases, cognitive and behavioral factors, somatic hypervigilance associated with anxiety, depression, and behavioral amplification contribute to symptom chronicity. The aims of evaluation in patients with POTS are to exclude cardiac causes of inappropriate tachycardia; elucidate, if possible, the most likely pathophysiologic basis of postural intolerance; assess for the presence of treatable autonomic neuropathies; exclude endocrine causes of a hyperadrenergic state; evaluate for cardiovascular deconditioning; and determine the contribution of emotional and behavioral factors to the patient's symptoms. Management of POTS includes avoidance of precipitating factors, volume expansion, physical countermaneuvers, exercise training, pharmacotherapy (fludrocortisone, midodrine, β-blockers, and/or pyridostigmine), and behavioral-cognitive therapy. A literature search of PubMed for articles published from January 1, 1990, to June 15, 2012, was performed using the following terms (or combination of terms): POTS; postural tachycardia syndrome, orthostatic; orthostatic; syncope; sympathetic; baroreceptors; vestibulosympathetic; hypovolemia; visceral pain; chronic fatigue; deconditioning; headache; Chiari malformation; Ehlers-Danlos; emotion; amygdala; insula; anterior cingulate; periaqueductal gray; fludrocortisone; midodrine; propranolol; β-adrenergic; and pyridostigmine. Studies were limited to those published in English. Other articles were identified from bibliographies of the retrieved articles.

Section snippets

Symptoms Reflecting Orthostatic Intolerance

POTS is one of the most common syndromes of orthostatic intolerance; others include reflex (neurally mediated, vasovagal) syncope and orthostatic hypotension in its several forms.3, 10 The manifestations of POTS that reflect orthostatic intolerance (POTS in the strict sense) include those of cerebral hypoperfusion and reflex sympathetic activation. Up to one-third of patients may develop secondary orthostatically triggered vasovagal (reflex, neurally mediated) syncope.11 As in all types of

Comorbidities in POTS

Many patients with POTS experience chronic symptoms that cannot be mechanistically explained by postural intolerance or excessive tachycardia.2, 37 Many of these symptoms are also prevalent in patients without orthostatic intolerance; in these cases, excessive postural tachycardia is secondary to hypovolemia, prolonged bed rest, physical deconditioning, and anxiety, in various combinations.

Potential Mechanisms of Persistence of Symptoms

The persistence of orthostatic symptoms despite adequate control of the heart rate and the coexistence of many nonorthostatic symptoms commonly reported by patients with POTS suggest that impaired processing of viscerosensory (including cardiovascular) information, conditioning, and behavioral amplification also play a contributory role in this disorder. For example, many triggers, such as viral illness (particularly if associated with gastrointestinal fluid loss), prolonged bed rest, or both,

Evaluation and Management of Patients With POTS

Patients with POTS require a multidisciplinary evaluation and multimodality treatment.2, 12

Conclusions and Perspective

POTS is a prototypical chronic, potentially disabling condition with no clear pathologic substrate and multiple interacting pathophysiologic mechanisms. Thus, it resembles functional visceral pain/dysmotility disorders, fibromyalgia, chronic headache, and chronic fatigue syndrome. In POTS, as in all these comorbid disorders, symptoms frequently develop after a triggering factor such as a viral illness or surgical procedure and persist despite resolution of the underlying condition. This

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