Postural tachycardia syndrome—current experience and concepts

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.

Postural tachycardia syndrome: multiple symptoms, but easily missed

“…8 PoTS may be secondary to other underlying conditions such as deconditioning after prolonged bed rest or space flight. It is increasingly recognised in joint hypermobility syndrome (indistinguishable from Ehlers-Danlos Syndrome -hypermobility type) 3 and has been associated with other conditions including diabetes, amyloidosis, sarcoidosis, systemic lupus erythematosus, cancers, and toxins (alcohol, heavy metals, and chemotherapy). In some patients, PoTS may be a presenting feature of pure autonomic failure or multiple system atrophy.…”

“…Experienced physicians 3 and patient support groups 11,12 are aware that patients often acquire inappropriate psychiatric diagnoses and consequently, distrust of doctors is common. Interestingly, using an anxiety assessment tool that relies on cognitive symptoms as opposed to somatic ones, PoTS patients have a tendency to lower anxiety levels than controls.…”

“…1 In patients with PoTS this mechanism does not respond appropriately; the exact pathophysiology remains unclear. 3 A likely mechanism is inadequate vasoconstriction on standing, resulting in pooling of blood in splanchnic and peripheral vasculature. Heart rate and catecholamine levels increase further to compensate.…”

“…8 Management initially involves physiological methods including graduated exercise programmes, high fluid and salt intake (not in hyperadrenergic PoTS), and support tights. 3,8 Triggers such as prolonged standing or sitting, alcohol, heat, and large meals should be avoided. Postural countermanoeuvres can abort a syncopal attack.…”

The association between muscle strength and activity limitations in patients with the hypermobility type of Ehlers–Danlos syndrome: the impact of proprioception

“…Patients diagnosed with Ehlers-Danlos syndrome, are characterized by an altered structural integrity of connective tissue [1,2] resulting in frailty [3] and multi-systemic manifestations like orthostatic intolerance, [4] hyper-elastic skin, [5] organ dysfunction [6] and joint instability. [7] The phenotype of EDS-HT is heterogeneous, in which the severity of complaints varies from mild to severe.…”