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 (PoTS) is a poorly understood but important cause of orthostatic intolerance resulting from cardiovascular autonomic dysfunction
PoTS mainly affects young individuals (aged 15–40 years) who are predominantly female; onset might be linked to infection, trauma, surgery or stress
Symptoms (palpitations, dizziness and, in some patients, syncope) usually occur when standing, and can be exacerbated by common stimuli in daily life, including modest exertion, food ingestion and heat
Proposed pathophysiological mechanisms include alterations in neural control, humoral factors, vascular properties and intravascular volume, as well as physical deconditioning
PoTS can be associated with various disorders; in particular, joint hypermobility syndrome, also known as Ehlers–Danlos syndrome (EDS) type III or EDS hypermobility type
A multifactorial treatment strategy that includes pharmacological agents as well as nonpharmacological measures and interventions is often required
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We are grateful to our patients and to the many colleagues who have referred patients to us, enabling us to expand on our experience in the diagnosis and management of postural tachycardia syndrome (PoTS) over the past decade. We are particularly grateful to K. Bleasedale-Barr, M. Tippetts and L. Mason who, along with other clinical autonomic scientists in each of our centers at St Mary's Hospital, and the National Hospital for Neurology and Neurosurgery at Queen Square, London, UK, have played a pivotal role in clinical autonomic investigation; to Dr G. Ingle (a Consultant colleague of C. J. Mathias) and Sister C. Best (Autonomic Clinical Nurse Specialist) for their help in patient management; and to the support staff who are essential for multidisciplinary work. We are also indebted to Professor Q. Aziz, Professor P. Goadsby, Dr A. Hakim, Dr H. Kaz Kaz, Mr V. Khullar, Dr M. Matharu, and Professor D. Silk, among others, for sharing their expertise and for their collaboration on the non-autonomic aspects of investigation and care of patients with PoTS and Ehlers–Danlos syndrome type III.
The authors declare no competing financial interests.
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Mathias, C., Low, D., Iodice, V. et al. Postural tachycardia syndrome—current experience and concepts. Nat Rev Neurol 8, 22–34 (2012). https://doi.org/10.1038/nrneurol.2011.187
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