Canadian Cardiovascular Society Position Statement on Postural Orthostatic Tachycardia Syndrome (POTS) and Related Disorders of Chronic Orthostatic I

Dolphin

Senior Member (Voting Rights)
https://www.sciencedirect.com/science/article/abs/pii/S0828282X19315508
Edited to add: Full article https://www.onlinecjc.ca/article/S0828-282X(19)31550-8/fulltext

Canadian Journal of Cardiology
Volume 36, Issue 3, March 2020, Pages 357-372

Society Position Statement
Canadian Cardiovascular Society Position Statement on Postural Orthostatic Tachycardia Syndrome (POTS) and Related Disorders of Chronic Orthostatic Intolerance

Author links open overlay panel
Satish R.Raj MD, MSCI, FRCPC(Chair)a
Juan C.GuzmanMD, MSc, FRCPC(Co-chair)bPaulaHarveyBMBS, PhDcLawrenceRicherMD, MSc, FRCPCdRonaldSchondorfMD, PhD, FRCPCeColetteSeiferMD, FRCPCfNicolasThibodeau-JarryMD, MMSc(MedEd)gRobert S.SheldonMD, PhD, FRCPCa


https://doi.org/10.1016/j.cjca.2019.12.024Get rights and content

Abstract
The current definition of postural orthostatic tachycardia syndrome (POTS) dates back to a small case series of patients with a subacute illness who presented with excessive orthostatic tachycardia and orthostatic intolerance, in the absence of another recognized disease. Conventional POTS criteria require an excessive orthostatic tachycardia in the absence of substantial orthostatic hypotension, and predominant symptoms of orthostatic intolerance, worse with upright posture and better with recumbence. POTS is a heterogeneous syndrome with likely several underlying pathophysiological processes, and not a specific disease. The primary panel for this Canadian Cardiovascular Society position statement sought to provide a contemporary update of the best evidence for the evaluation and treatment of POTS. We performed a systemic review of evidence for the evaluation of treatment of POTS using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) methodology, and developed recommendations on the basis of the Canadian Cardiovascular Society approach to position statements. One identified problem was that numerous patients who did not meet criteria for POTS would still be given that diagnoses by providers to validate the illness even though this diagnosis is incorrect. This includes patients with postural symptoms without tachycardia, orthostatic tachycardia without symptoms, and those with orthostatic tachycardia but another overt cause for excessive tachycardia. We developed a novel nomenclature ecosystem for orthostatic intolerance syndromes to increase clarity. We also provide more clarity on how to interpret the orthostatic vital signs. These concepts will need to be prospectively assessed.

Résumé
La définition actuelle du syndrome de tachycardie orthostatique posturale (STOP) remonte à une petite série de cas de patients atteints d’une maladie subaiguë qui présentaient une tachycardie orthostatique excessive et une intolérance orthostatique, en l’absence d’une autre maladie reconnue. Selon les critères classiques, le STOP implique une tachycardie orthostatique excessive en l’absence d’hypotension orthostatique marquée, ainsi que des symptômes prédominants d’intolérance orthostatique, s’aggravant en position verticale et s’atténuant en position allongée. Le STOP est une entité hétérogène où interviennent probablement plusieurs processus physiopathologiques sous-jacents; il ne s’agit pas d’une maladie en tant que telle. Le principal groupe d’experts à l’origine de cet énoncé de position de la Société canadienne de cardiologie a cherché à présenter les données probantes les plus récentes et les meilleures en matière d’évaluation et de traitement du STOP. Nous avons effectué un examen systématique des données probantes aux fins d’évaluation du traitement du STOP conformément à la méthodologie GRADE (Grading of Recommendations, Assessment, Development, and Evaluation) et formulé des recommandations fondées sur le cadre de référence relatif aux énoncés de position de la Société canadienne de cardiologie. Notre démarche a notamment fait ressortir une problématique, où un diagnostic de STOP avait été posé chez de nombreux patients malgré l’incongruence de leur état et des critères définissant le syndrome. Un tel diagnostic erroné avait notamment été prononcé chez des patients présentant des symptômes posturaux en l’absence de tachycardie, une tachycardie orthostatique asymptomatique ou encore une tachycardie orthostatique en présence d’une autre cause manifeste de tachycardie excessive. Par souci de clarté, nous avons élaboré un nouvel écosystème de classification des syndromes d’intolérance orthostatique. Dans la même optique, nous avons également précisé les critères d’interprétation des signes vitaux orthostatiques. Ces concepts devront être l’objet d’une évaluation prospective.
 
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Thanks for that link, @Denise :)

I found this table interesting:

https://www.onlinecjc.ca/action/showFullTableHTML?isHtml=true&tableId=tbl1&pii=S0828-282X(19)31550-8

It summarizes five terms they've defined for different criteria: POTS, POTS plus, PSWT, PSWT plus, and PTOC.

POTS = postural orthostatic tachycardia syndrome
PSWT = postural symptoms without tachycardia
PTOC = postural tachycardia of other cause

Edited to add: "These newly described disorders were created on the basis of expert opinion of the primary panel and will need to be evaluated to determine if further modifications are required."
 
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This position statement seems to differ quite a bit from that of the recent Van Campen article discussed here
https://www.s4me.info/threads/cereb...an-campen-et-al-2020.13583/page-5#post-238383

https://www.sciencedirect.com/science/article/pii/S2467981X20300044
I think it's the difference between POTS and generalised OI. Many patients with ME have symptoms of OI, even if they don't meet POTS criteria. It's just that POTS is a catchier name, so people use it to refer to ME-OI.
 
Thanks for that link, @Denise :)

I found this table interesting:

https://www.onlinecjc.ca/action/showFullTableHTML?isHtml=true&tableId=tbl1&pii=S0828-282X(19)31550-8

It summarizes five terms they've defined for different criteria: POTS, POTS plus, PSWT, PSWT plus, and PTOC.

POTS = postural orthostatic tachycardia syndrome
PSWT = postural symptoms without tachycardia
PTOC = postural tachycardia of other cause

Edited to add: "These newly described disorders were created on the basis of expert opinion of the primary panel and will need to be evaluated to determine if further modifications are required."

So according to this, people with ME who meets POTS criteria should be diagnosed as POTS plus, and those who don't meet POTS criteria but who have orthostatic intolerance, should be diagnosed as PSWT plus?
 
When I was first learning about ME, about 1984 after becoming ill in 1968, one of the symptoms they spoke about was feeling bad when standing still. This had always been a problem for me and I often walked rather than stand waiting for a bus. Both had payback but the latter gave less.

In recent years this has become known as POTS but that syndrome may not encompass everything that is going on in ME and we have to be careful that patients who do not get the tachycardia of POTS have it recorded as another negative test showing that we imagine our symptoms.

It is good if the phenomenon is being more widely examined and not restricted to a narrow definition.
 
When I was first learning about ME, about 1984 after becoming ill in 1968, one of the symptoms they spoke about was feeling bad when standing still. This had always been a problem for me and I often walked rather than stand waiting for a bus. Both had payback but the latter gave less.

In recent years this has become known as POTS but that syndrome may not encompass everything that is going on in ME and we have to be careful that patients who do not get the tachycardia of POTS have it recorded as another negative test showing that we imagine our symptoms.

It is good if the phenomenon is being more widely examined and not restricted to a narrow definition.

It is important to distinguish between POTS and orthostatic intolerance. POTS is a specific condition with a specific definition, albeit still poorly understood. Orthostatic intolerance is a symptom that is associated with POTS but not sufficient of itself for a diagnosis of POTS, rather like the relationship between the symptom of fatigue and the condition ME. Also orthostatic intolerance as a symptom like fatigue may potentially have very diverse causes. Further we need to have a clearer definition of orthostatic intolerance to be certain different people are meaning the same thing by this term.

Both orthostatic intolerance and POTS are increasingly found to be associated with ME, but can not with certainty say they are causally related to the ME or coincidental. How does orthostatic intolerance in ME relate to orthostatic intolerance in POTS occurring in isolation? Is POTS a unitary condition? Does POTS occurring in conjunction with ME mirror or diverge from POTS occurring in isolation? Etc.

Like so much in this area there is a need for clearer definitions and a better understanding of the phenomenology of orthostatic intolerance as well as experimental investigation.

So many of us with ME have issues relating to being upright, but not all of us fit the definition of POTS. However can we yet say what are the significant descriptors of our orthostatic issues and are they the same from patient to patient?
 
The hypothalamus was said to be dysfunctional in ME in the old days. It is part of the brain that controls the autonomic nervous system and helps keep the body in homeostasis.

Temperature is often difficult to control in ME and I have felt that hypothalamus problems explain a lot of my symptoms including the dysautonomia and POTS.

Mt personal view is that ME is causing damage and that POTS and so on is a consequence of that damage. looking at people with POTS who do not have ME may not help us.
 
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