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Postural tachycardia syndrome—current experience and concepts

Nature Reviews Neurology volume 8, pages 22–34 (2012)Cite this article

Subjects

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.

Key Points

  • 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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Figure 1: Blood pressure and heart rate profiles obtained while supine and during head-up tilt.
Figure 2: Changes in blood pressure and heart rate associated with various stimuli in a patient with PoTS.
Figure 3: Clinical signs of PoTS.
Figure 4: Blood pressure and heart rate profiles during 24-h ambulatory monitoring using the London Autonomic Units protocol.

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Acknowledgements

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.

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Authors and Affiliations

  1.  Autonomic and Neurovascular Medicine Unit, Imperial College London, St Mary's Hospital, 2nd Floor, Queen Elizabeth the Queen Mother Wing, Praed Street, London, W2 1NY, UK

    Christopher J. Mathias, David A. Low, Valeria Iodice & Andrew P. Owens

  2.  Autonomic Unit, University College London Institute of Neurology and National Hospital for Neurology and Neurosurgery, Queen Square, London, WC1N 3BG, UK

    Christopher J. Mathias

  3. Department of Neurology, University Medical Center Ljubljana, Zaloška cesta 2, Ljubljana, 1000, Slovenia

    Mojca Kirbis

  4. University College London Division of Medicine, Hypermobility Clinic, Center for Rheumatology, Windeyer Building, 46 Cleveland Street, London, W1T 4JF, UK

    Rodney Grahame

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  3. Valeria Iodice
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  4. Andrew P. Owens
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  5. Mojca Kirbis
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  6. Rodney Grahame
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Contributions

All six authors contributed to writing the article. C. J. Mathias, D. A. Low, V. Iodice, A. P. Owens and M. Kirbis researched data for the article. C. J. Mathias, D. A. Low, V. Iodice and R. Grahame made substantial contributions to discussions of the content, and C. J. Mathias, D. A. Low, A. P. Owens and R. Grahame undertook review and/or editing of the manuscript before submission.

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Correspondence to Christopher J. Mathias.

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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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