Postural orthostatic tachycardia syndrome

Douglas J Lanska MD FAAN MS MSPH (Dr. Lanska of the Great Lakes VA Healthcare System and the University of Wisconsin School of Medicine and Public Health has no relevant financial relationships to disclose.)
Originally released February 20, 2008; last updated February 7, 2017; expires February 7, 2020

This article includes discussion of POT syndrome, central hyperadrenergic POTS, mast cell activation disorders, neuropathic POTS, and POTS. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

The author explains the clinical presentation, pathophysiology, diagnostic workup, and management of postural orthostatic tachycardia syndrome (POTS), an often disabling syndrome of symptomatic orthostatic tachycardia in the absence of orthostatic hypotension. POTS is a restricted sympathetic dysautonomia with neuropathic and central forms resulting from a constellation of different disorders. Although there is no universally effective therapy for postural orthostatic tachycardia syndrome, pharmacological treatment can include beta-blocking agents to blunt orthostatic increases in heart rate, alpha-adrenergic agents to increase peripheral vascular resistance, mineralocorticoid agents to increase blood volume, and serotonin reuptake inhibitors.

Key points

 

• Postural orthostatic tachycardia syndrome (POTS) is an often-disabling syndrome of symptomatic orthostatic tachycardia in the absence of orthostatic hypotension.

 

• POTS is a restricted sympathetic dysautonomia with neuropathic and central forms resulting from a constellation of different disorders.

 

• The symptoms of postural orthostatic tachycardia syndrome suggest a hyperadrenergic state and impaired cerebral perfusion.

 

• In addition to the orthostatic tachycardia, physical findings in postural orthostatic tachycardia syndrome can include a murmur or click of mitral valve prolapse (although significant mitral regurgitation is unusual) and prominent dependent acrocyanosis.

 

• There is no universally effective therapy for postural orthostatic tachycardia syndrome.

 

• Nonpharmacological treatment can include increasing fluid/salt intake, increasing aerobic exercise, lower-extremity strength training, and compression stockings, as well as counseling and training for management of pain or anxiety and family education.

 

• Pharmacological treatment can include beta-blocking agents to blunt orthostatic increases in heart rate, alpha-adrenergic agents to increase peripheral vascular resistance, mineralocorticoid agents to increase blood volume, and serotonin reuptake inhibitors.

Historical note and terminology

Postural orthostatic tachycardia syndrome (POTS) is an often disabling syndrome of symptomatic orthostatic tachycardia in the absence of orthostatic hypotension. POTS is a restricted sympathetic dysautonomia with neuropathic and central forms resulting from a constellation of different disorders. The syndrome has also been called orthostatic tachycardia, orthostatic intolerance, idiopathic orthostatic intolerance and postural tachycardia, neurocirculatory asthenia, mitral valve prolapse syndrome, irritable heart, and soldier's heart, among others. There is also clinical overlap with chronic fatigue syndrome, especially in adolescent cases (Stewart and Weldon 2000; Ocon et al 2012; Okamoto et al 2012; Reynolds et al 2014), although patients with both conditions apparently represent a specific subset of those with chronic fatigue syndrome (Reynolds et al 2014).

The content you are trying to view is available only to logged in, current MedLink Neurology subscribers.

If you are a subscriber, please log in.

If you are a former subscriber or have registered before, please log in first and then click select a Service Plan or contact Subscriber Services. Site license users, click the Site License Acces link on the Homepage at an authorized computer.

If you have never registered before, click Learn More about MedLink Neurology  or view available Service Plans.