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Pathophysiology and Classification of PoTS

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Postural Tachycardia Syndrome

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Abstract

Postural tachycardia syndrome (PoTS) is a heterogeneous clinical syndrome of orthostatic intolerance. Distinct subtypes (neuropathic, hypovolemic, and hyperadrenergic PoTS) have been developed in an effort to better understand the physiological underpinnings of the disorder. These subtypes are problematic, due to lack of standard definitions, implied exclusivity, and poor explanatory power regarding the underlying pathophysiology. Our continued investigation of PoTS has recently revealed a number of novel mechanisms, such as the involvement of the immune system (including mast cell activation syndrome), physical deconditioning, norepinephrine transporter deficiency, and impaired cerebral autoregulation. A more comprehensive paradigm is needed, including a precise description of how these diverse processes might overlap, and how each contributes to the clinical presentation of a given PoTS patient. This understanding is critical to the diagnosis and treatment of patients with PoTS.

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Abbreviations

PoTS:

Postural Tachycardia Syndrome

NET:

Norepinephrine transporter

QSART:

Quantitative sudomotor axon reflex testing

BP:

Blood pressure

QDIRT:

Quantitative direct and indirect reflex testing

IENFD:

Intra-epidermal nerve fiber density

QST:

Quantitative sensory testing

RAAS:

Renin–Angiotensin–Aldosterone system

MCAS:

Mast Cell Activation Syndrome

CBF:

Cerebral Blood Flow

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Acknowledgments

The authors would like to acknowledge the many patients that have volunteered their time to contribute to this body of work. Their participation is critical to the ongoing investigation of POTS.

Funding

Dr. Raj has a research grant related to Postural Tachycardia Syndrome from the Canadian Institutes of Health Research, Ottawa, Canada (grant number MOP142426). Dr. Lloyd has a post-doctoral scholarship through the Cummings School of Medicine.

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Authors and Affiliations

  1. Libin Cardiovascular Institute of Alberta, Cumming School of Medicine, University of Calgary, Calgary, Canada

    Matthew G. Lloyd & Satish R. Raj

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  1. Matthew G. Lloyd
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Correspondence to Satish R. Raj .

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Editors and Affiliations

  1. King's College Hospital, London, UK

    Nicholas Gall

  2. Faculty of Health, Education and Life Sciences, Birmingham City University, Birmingham, UK

    Lesley Kavi

  3. Barts NIHR Biomedical Research Centre, William Harvey Research Institute, Queen Mary University of London, London, UK

    Melvin D. Lobo

Ethics declarations

Dr. Raj is a network investigator of the Cardiac Arrhythmia Network of Canada (CANet; London, Ontario, Canada). Dr. Raj serves on the Medical Advisory Board of Dysautonomia International and PoTS UK, both without financial compensation. Dr. Lloyd has reported that he has no relationships relevant to the contents of this chapter to disclose.

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Lloyd, M.G., Raj, S.R. (2021). Pathophysiology and Classification of PoTS. In: Gall, N., Kavi, L., Lobo, M.D. (eds) Postural Tachycardia Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-54165-1_5

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