Postural orthostatic tachycardia syndrome (POTS): Priorities for POTS care and research from a 2019 National Institutes of Health Expert Consensus Meeting - Part 2

Satish R Raj; Kate M Bourne; Lauren E Stiles; Mitchell G Miglis; Melissa M Cortez; Amanda J Miller; Roy Freeman; Italo Biaggioni; Peter C Rowe; Robert S Sheldon; Cyndya A Shibao; Andre Diedrich; David M Systrom; Glen A Cook; Taylor A Doherty; Hasan I Abdallah; Blair P Grubb; Artur Fedorowski; Julian M Stewart; Amy C Arnold; Laura A Pace; Jonas Axelsson; Jeffrey R Boris; Jeffrey P Moak; Brent P Goodman; Kamal R Chémali; Tae H Chung; David S Goldstein; Anil Darbari; Steven Vernino

doi:10.1016/j.autneu.2021.102836

Postural orthostatic tachycardia syndrome (POTS): Priorities for POTS care and research from a 2019 National Institutes of Health Expert Consensus Meeting - Part 2

Auton Neurosci. 2021 Nov:235:102836. doi: 10.1016/j.autneu.2021.102836. Epub 2021 Jun 30.

Authors

Satish R Raj¹, Kate M Bourne², Lauren E Stiles³, Mitchell G Miglis⁴, Melissa M Cortez⁵, Amanda J Miller⁶, Roy Freeman⁷, Italo Biaggioni⁸, Peter C Rowe⁹, Robert S Sheldon², Cyndya A Shibao¹⁰, Andre Diedrich¹¹, David M Systrom¹², Glen A Cook¹³, Taylor A Doherty¹⁴, Hasan I Abdallah¹⁵, Blair P Grubb¹⁶, Artur Fedorowski¹⁷, Julian M Stewart¹⁸, Amy C Arnold¹⁹, Laura A Pace²⁰, Jonas Axelsson²¹, Jeffrey R Boris²², Jeffrey P Moak²³, Brent P Goodman²⁴, Kamal R Chémali²⁵, Tae H Chung²⁶, David S Goldstein²⁷, Anil Darbari²³, Steven Vernino²⁸

Affiliations

¹ Department of Cardiac Sciences, Libin Cardiovascular Institute, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada; Autonomic Dysfunction Center, Division of Clinical Pharmacology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA. Electronic address: satish.raj@ucalgary.ca.
² Department of Cardiac Sciences, Libin Cardiovascular Institute, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada.
³ Department of Neurology, Stony Brook University Renaissance School of Medicine, Stony Brook, NY, USA; Dysautonomia International, East Moriches, NY, USA.
⁴ Department of Neurology and Neurological Sciences, Stanford University School of Medicine, Palo Alto, CA, USA.
⁵ Department of Neurology, University of Utah, Salt Lake City, UT, USA.
⁶ Department of Neural and Behavioral Sciences, Pennsylvania State University College of Medicine, Hershey, PA, USA.
⁷ Department of Neurology, Harvard Medical School, Boston, MA, USA; Center for Autonomic and Peripheral Nerve Disorders, Beth Israel Deaconess Medical Center, Boston, MA, USA.
⁸ Autonomic Dysfunction Center, Division of Clinical Pharmacology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA; Autonomic Dysfunction Center, Division of Clinical Pharmacology, Department of Pharmacology, Vanderbilt University Medical Center, Nashville, TN, USA.
⁹ Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
¹⁰ Autonomic Dysfunction Center, Division of Clinical Pharmacology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.
¹¹ Autonomic Dysfunction Center, Division of Clinical Pharmacology, Department of Medicine and Biomedical Engineering, Vanderbilt University Medical Center, Nashville, TN, USA.
¹² Pulmonary and Critical Care Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
¹³ Department of Neurology, Uniformed Services University, Bethesda, MD, USA.
¹⁴ Division of Rheumatology, Allergy, and Immunology, Department of Medicine, University of California at San Diego, La Jolla, CA, USA.
¹⁵ Children's Heart Institute, Fredericksburg, VA, USA.
¹⁶ Division of Cardiology, Department of Medicine, The University of Toledo Medical Center, USA.
¹⁷ Department of Clinical Sciences, Lund University, Malmö, Sweden; Department of Cardiology, Skåne University Hospital, Malmö, Sweden.
¹⁸ Center for Hypotension, Departments of Pediatrics and Physiology, New York Medical College, Valhalla, NY USA.
¹⁹ Autonomic Dysfunction Center, Division of Clinical Pharmacology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA; Department of Neural and Behavioral Sciences, Pennsylvania State University College of Medicine, Hershey, PA, USA.
²⁰ Center for Genomic Medicine and Department of Pediatrics, Division of Medical Genetics and Genomics, University of Utah, Salt Lake City, UT, USA.
²¹ Department of Clinical Immunology, Karolinska University Hospital, Stockholm, Sweden.
²² Jeffrey R. Boris, MD LLC, Moylan, PA, USA.
²³ Department of Pediatrics, George Washington University School of Medicine and Health Sciences, Washington, DC, USA.
²⁴ Neuromuscular Division, Department of Neurology, Mayo Clinic, Scottsdale, AZ, USA.
²⁵ Department of Neurology, Eastern Virginia Medical School, Division of Neurology, Neuromuscular and Autonomic Center, Sentara Healthcare, Norfolk, VA, USA.
²⁶ Department of Physical Medicine and Rehabilitation, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
²⁷ Autonomic Medicine Section, Clinical Neurosciences Program, Division of Intramural Research, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA.
²⁸ Department of Neurology, UT Southwestern Medical Center, Dallas, TX, USA.

Abstract

The National Institutes of Health hosted a workshop in 2019 to build consensus around the current state of understanding of the pathophysiology of postural orthostatic tachycardia syndrome (POTS) and to identify knowledge gaps that must be addressed to enhance clinical care of POTS patients through research. This second (of two) articles summarizes current knowledge gaps, and outlines the clinical and research priorities for POTS. POTS is a complex, multi-system, chronic disorder of the autonomic nervous system characterized by orthostatic intolerance and orthostatic tachycardia without hypotension. Patients often experience a host of other related disabling symptoms. The functional and economic impacts of this disorder are significant. The pathophysiology remains incompletely understood. Beyond the significant gaps in understanding the disorder itself, there is a paucity of evidence to guide treatment which can contribute to suboptimal care for this patient population. The vast majority of physicians have minimal to no familiarity or training in the assessment and management of POTS. Funding for POTS research remains very low relative to the size of the patient population and impact of the syndrome. In addition to efforts to improve awareness and physician education, an investment in research infrastructure including the development of standardized disease-specific evaluation tools and outcome measures is needed to facilitate effective collaborative research. A national POTS research consortium could facilitate well-controlled multidisciplinary clinical research studies and therapeutic trials. These priorities will require a substantial increase in the number of research investigators and the amount of research funding in this area.

Keywords: Expert Consensus; Pathophysiology; Postural orthostatic tachycardia syndrome; Treatment; Workshop.

Publication types

Consensus Development Conference, NIH
Research Support, N.I.H., Extramural
Research Support, N.I.H., Intramural

MeSH terms

Autonomic Nervous System
Consensus
Humans
National Institutes of Health (U.S.)
Orthostatic Intolerance*
Postural Orthostatic Tachycardia Syndrome* / diagnosis
Postural Orthostatic Tachycardia Syndrome* / therapy
United States

Abstract

Publication types

MeSH terms

Grants and funding