Postural tachycardia syndrome (POTS) is a multifactorial clinical syndrome defined by an increase in heart rate of ≥30 bpm on standing from supine position (or ≥40 bpm in children). It is associated with symptoms of cerebral hypoperfusion that are worse when upright and improve when in supine position. Patients often have additional symptoms including severe fatigue and difficulty concentrating. There are several possible pathophysiologic mechanisms including hypovolaemia, small-fibre peripheral neuropathy and hyperadrenergic states. POTS can also be associated with several disorders including mastocytosis, Ehlers-Danlos syndrome (hypermobility type) and autoimmune disorders. The treatment is focused on symptom relief and not solely on reducing tachycardia. Given its varying presentations, it is important to employ a practical, mechanism-focused approach to the diagnosis and management of POTS.
- postural tachycardia syndrome
- orthostatic intolerance
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Contributors PKJ and BHS: drafting of manuscript and tables; revision of manuscript. SRR: drafting and critical revision of manuscript and tables for intellectual content.
Competing interests SRR: He serves on scientific advisory board—Lundbeck Pharmaceuticals, GE Healthcare; serves on the Medical Advisory Board for multiple non-profit POTS Patient Groups (no financial compensation); Funding for travel—POTS UK International Speakers' Fund; serves on editorial boards for Autonomic Neuroscience: Basic and Clinical (Elsevier) Clinical Autonomic Research (Springer), Frontiers in Autonomic Neurosciences; none are compensated financially; honoraria—Lundbeck Pharmaceutical; government research support: NIH R01 102387 and Canadian Institutes of Health Research MOP 142426; Foundation Research Support—Dysautonomia International; Commercial Research Support—Medtronic Canada; witness or consultant for a legal proceeding dealing with POTS causality.
Provenance and peer review Commissioned; externally peer reviewed. This paper was reviewed by Gordon Ingle, London, UK, Wojtek Rakowicz, Hampshire, UK, and Paul Cooper, Manchester, UK.