PoTS — Postural tachycardia syndrome

Postural Tachycardia Syndrome

The autonomic dysfunction that manifests as PoTS is poorly understood yet can cause alarming and distressing symptoms that hugely impact upon an individual’s quality of life. The experience of PoTS includes dizziness, palpitations and syncope, triggered by a range of stimuli such as heat, exertion, food ingestion and others. The variable nature and the plethora of symptoms that can be subtle on occasion, extending to the more overt collapse, can mean that day to day living becomes extremely challenging. The shift in the sense of self with PoTS as with other conditions, defines the suffering that the individual bears.

PoTS is often associated with hypermobility, joint hypermobility syndrome (JHS) and Ehlers-Danlos Syndrome Hypermobility Type (EDS-HT). Many patients who visit the clinic with chronic pain and functional pain syndromes will demonstrate hypermobility on moving — lumbar spine, knees, elbows, thumbs, little fingers. It makes sense then, to be vigilant for symptoms of PoTS and dysautonomia.

For further information about PoTS, visit the PoTS UK website

There are several very good reviews of PoTS:

Postural tachycardia syndrome–current experience and concepts –Mathias CJ, Low DA, Iodice V, Owens AP, Kirbis M, Grahame R.

Abstract
Postural tachycardia syndrome (PoTS) is a poorly understood but important cause of orthostatic intolerance resulting from cardiovascular autonomic dysfunction. PoTS is distinct from the syndromes of autonomic failure usually associated with orthostatic hypotension, such as pure autonomic failure and multiple system atrophy. Individuals affected by PoTS are mainly young (aged between 15 years and 40 years) and predominantly female. The symptoms–palpitations, dizziness and occasionally syncope–mainly occur when the patient is standing upright, and are often relieved by sitting or lying flat. Common stimuli in daily life, such as modest exertion, food ingestion and heat, are now recognized to be capable of exacerbating the symptoms. Onset of the syndrome can be linked to infection, trauma, surgery or stress. PoTS can be associated with various other disorders; in particular, joint hypermobility syndrome (also known as Ehlers-Danlos syndrome hypermobility type, formerly termed Ehlers-Danlos syndrome type III). This Review describes the characteristics and neuroepidemiology of PoTS, and outlines possible pathophysiological mechanisms of this syndrome, as well as current and investigational treatments.

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Postural tachycardia syndrome: a heterogeneous and multifactorial disorder — full article free here
Benarroch EE.

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.

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