Pipedreams, the pandemic and PoTS: is the post-COVID-19 era a turning point for PoTS services?

Br J Cardiol 2022;29:5–7doi:10.5837/bjc.2022.007 Leave a comment
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First published online March 8th 2022

The paper by Gall et al., published in this issue (see https://doi.org/10.5837/bjc.2022.003), is timely and important; the largest case series from the UK, and among the largest globally detailing the clinical characteristics of patients affected with postural tachycardia syndrome (PoTS) developing after a COVID-19 infection. It brings empirical stature to the anecdotal reports of PoTS developing post-COVID-19. It articulates that this presents in a form indistinguishable from PoTS precipitated by other events.

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While the aetiology of this complex condition remains unclear, PoTS has been associated with other conditions, including the Epstein-Barr virus, autoimmune conditions1 such as Sjögren’s syndrome and antiphospholipid syndrome, joint hypermobility conditions,2 and chronic fatigue.3 PoTS, as is the case for most syndromes, presents uniquely in everyone in terms of its thumbprint of symptomatic expression. Similarly, these post-COVID-19 cases, in addition to the diagnostic presence of abnormal response by the autonomic nervous system to upright posture (diagnosed by a sustained increase in heart rate of over 30 bpm in adults within 10 minutes of standing4,5), present with a variety of other associated complaints. Clinical experience suggests this distribution of additional symptoms, such as migraine, breathing pattern disorder, gastrointestinal complications, fatigue and brain-fog, is similar to those seen clinically in the wider PoTS population.

This paper gives scientific credence to the assumption that PoTS is likely a significant contributor to key debilitating symptoms seen in some long-COVID presentations. The National Institute for Health and Care Excellence (NICE) already recommends an active stand test for those presenting with orthostatic intolerance in long-COVID, to routinely screen for PoTS. We would argue that, in addition, services developed for long-COVID should be expanded to meet the unmet need in the under-resourced PoTS population. Good news stories feel needed more than ever as we emerge from the challenges of the pandemic. This association between COVID-19 and PoTS offers hope that the aftermath of the pandemic phase of COVID-19 can mark a turning point in the recognition, treatment and investment in PoTS. Pre-pandemic we know that most healthcare professionals were unaware of PoTS, even though it is estimated to have affected 0.2% of the population.4 Further research is needed to clarify if that percentage has now risen post-pandemic.

In 2020, the PoTS UK charity, on their 10th anniversary awareness day, asked leading practitioners around the world to share their wishes and visions for the next 10 years. These included that:

  • All healthcare practitioners and the general public would have awareness of the condition.
  • There would be equitable access to treatment.
  • Specialised multi-specialty/multi-disciplinary treatment centres would be funded.
  • There would be vastly increased investment in PoTS research.

At the time these wishes appeared to be improbable pipedreams; what a difference a couple of years can make. Today, as we detail below, there is significant hope for change on all fronts.

There are clear sensitivities to capitalising on a collectively traumatic time where we have witnessed such extensive grief, loss and multiple long-term sequelae to well-being, mental health, the economy, catastrophic delays to standard services, and far-reaching impacts on global health. However, if among this, there are finally some positives for a long-suffering patient group; surely this can only be welcomed.

Hopes

Hope for people with long-COVID

In addition, for the potential benefit to the pre-pandemic PoTS populations, we feel that the Gall et al. paper offers hope for people with long-COVID. Clinicians and charities working with people experiencing PoTS have a great deal to offer in understanding the needs of the long-COVID population, for whom we would argue adequate services are yet to be developed. Reputable news sources suggest many clinicians feel under-skilled and overwhelmed by these patients’ needs.6 Yet there is a wealth of relevant knowledge in PoTS specialists to tap into and accelerate the development of effective services. Despite NICE guidance recommending testing for PoTS in people with postural symptoms, this does not always occur; and, as yet, this experienced network of clinicians with relevant expertise has been underutilised. We have an opportunity to combine the enhanced understanding of complex post-viral conditions brought by the pandemic, with the experiences from managing PoTS, and begin a long-overdue reinvention of healthcare provision for those with long-term conditions.

Hope for improved recognition and awareness of PoTS

Despite the publication of PoTS guidelines by the Heart Rhythm Society 2015,4 and the Canadian Cardiovascular Society 2020,5 there remains scepticism about whether the condition is ‘real’. Shocking as it seems, the perspective that PoTS is a title given to neurosis in middle-class females is still occasionally perpetuated, even in some mainstream literature, and certainly in patients’ journey to diagnosis. Up to 85% of PoTS patients are told that their symptoms are ‘all in their head’ and given psychiatric labels of anxiety and depression, despite the fact that research shows that PoTS patients are no more likely to have psychiatric disorders than people experiencing other chronic health conditions with a similar impact on quality of life. We hope that now that we have reached a time when NICE recommend testing for PoTS in their long-COVID recommendations,7 and the Royal College of General Practitioners (RCGP)8 and National Health Service (NHS)9 resources also issue advice about the condition, any idea that PoTS is a factitious condition can now finally be put to bed.

Pre-pandemic the mean time to diagnosis post-symptom-onset in the UK was seven years.3 As information and education about the condition increase, we hope that this disheartening statistic will improve.

Hope for the long-COVID association meaning the development of much needed specialist services

As things stand today, many patients with PoTS are denied access to NHS specialists and treatments. Management is often complex and requires an informed physician. Some regions of the UK have no specialists. Services in large centres (often in London) are overwhelmed with referrals. Children are especially affected by poor NHS healthcare provision for PoTS. Combining services for people with long-COVID and PoTS would help to plug those unmet needs, while also bringing together the very relevant multi-speciality and multi-disciplinary expertise developed within the community of practitioners interested in PoTS.

Hope for increased compassion and recognition of quality of life as important, beyond mortality rates

We hope that the pandemic has increased understanding that health is not binary; there are many shades of suffering between ‘ill’ and ‘well’. We hope that the pandemic has accelerated the recognition that long-term conditions and disability need to be considered as we assess our societies’ record on health, and not just mortality rates. Additionally, we hope that there has been increased compassion about the impacts of the loneliness precipitated by health-imposed isolation, and grappling with the identity changes that come with illness. Surely stigmas and assumptions are shifting as people in the public eye, clinicians and leaders who have suffered from long-term sequelae have spoken openly. It is only now, perhaps, becoming widely realised that no one is immune from developing debilitating long-term conditions, with symptoms including fatigue and brain-fog.

The disability caused by PoTS, and, therefore, we may extrapolate long-COVID, is severe – equivalent to congestive heart failure and chronic obstructive pulmonary disease (conditions that usually affect older people).10 Community recognition, compassion and support is key.

It is worth noting that among the PoTS community, many of whom are suffering from the psychological effects of feeling unsupported and marginalised, in many cases for years, there has been distress and disgruntlement that long-COVID has been a passport to more care, belief and acknowledgment than they have been party to. We empathise with this frustration, but are committed to looking forward and putting energy into improving this, rather than labouring the old, albeit objective, injustices.

Recognition of the human and economic costs of COVID, hopefully, will improve recognition that giving PoTS patients access to treatment makes economic and humanitarian sense

We need an army of income earners for the country to recover from the pandemic’s long-term social and financial burden. We hope this provides some governmental impetus to get those suffering from conditions, like PoTS, functioning and contributing as they would like to. PoTS can occur at any age, but the most common age for PoTS to develop is young adulthood: 50% of patients with insufficiently treated PoTS cannot attend work or school. The costs are enormous, both economically to the country, and personally to sufferers and their families, given that many educational and career opportunities at this critical stage are irrecoverably lost. Over 90% of patients will respond to treatment, thus, heightening the tragedy of delays in diagnosis and treatment. People with PoTS are often heavy users of our already teetering NHS emergency services until they can access appropriate diagnostic tests and treatment, another urgent reason to prioritise developing access to the available treatments.

The NHS Long Term Plan11 set bold ambitions to harness digital technology for health, which have benefited from the pandemic having swept red tape and barriers to implementation when solutions urgently needed to be put into place. Digitalisation can help many people with PoTS overcome the challenges disability brings, particularly with mobility and accessing services. In our experience of supporting this largely young demographic, they generally have excellent technological literacy, and uptake of online appointments and resources is large. PoTS UK welcome ongoing acceleration in this area, and hope that this will continue into other services allowing flexibility and improving accessibility.

A note of caution

In caution to this optimism is the looming reality that we are facing a crisis without investment, specialist services, and changes to our healthcare system. Waiting times, already far too long (up to a year for secondary and tertiary care), look set to soar. NHS England has agreed that clinicians’ awareness of PoTS is low and that services for affected patients were inadequate before the pandemic. As the Gall et al. article cites, in the US, the American Autonomic Society anticipates an increase in referrals to already overwhelmed services, way beyond their capacity to deliver adequate, timely services. As rates and recognition accelerate, scarce services become even more overwhelmed and waiting lists longer.

Turning hope into action

Like people experiencing long-COVID, and their healthcare providers and carers, are experiencing now; people with PoTS have become despondent before and during the pandemic about the quantity and quality of research, awareness, compassion, treatment pathways, and services. It would be wonderful if, as well as the swathes of destruction caused by the virus to people with PoTS and without; the aspirations explored above no longer need to be considered pipedreams.

With renewed vigour, PoTS UK remains committed to advocating to:

  • Ensure that PoTS patients have equitable access to healthcare.
  • Raising further awareness among healthcare professionals and the general public.
  • Supporting research into the causes of PoTS, the impact on patients and developing effective treatments.

Be part of the change! For more information about the organisation and to be informed about upcoming study days for healthcare professionals see www.potsuk.org.

We are always keen to expand our outreach to interested clinicians and encourage you to sign up for our newsletters via the website, and otherwise get informed and involved.

Conflicts of interest

MO and MN are both Trustees of PoTS UK.

Funding

None.

Editors’ note

See article by Gall et al. https://doi.org/10.5837/bjc.2022.003 from this issue.

References

1. Vernino S, Stiles LE. Autoimmunity in postural orthostatic tachycardia syndrome: current understanding. Auton Neurosci 2018;215:78–82. https://doi.org/10.1016/j.autneu.2018.04.005

2. Roma M, Marden CL, De Wandele I, Francomano CA, Rowe PC. Postural tachycardia syndrome and other forms of orthostatic intolerance in Ehlers-Danlos syndrome. Auton Neurosci 2018;215:89–96. https://doi.org/10.1016/j.autneu.2018.02.006

3. Shaw BH, Stiles LE, Bourne K et al. The face of postural tachycardia syndrome – insights from a large cross-sectional online community-based survey. J Intern Med 2019;286:438–48. https://doi.org/10.1111/joim.12895

4. Sheldon RS, Grubb BP 2nd, Olshansky B et al. 2015 Heart Rhythm Society expert consensus statement on the diagnosis and treatment of postural tachycardia syndrome, inappropriate sinus tachycardia, and vasovagal syncope. Heart Rhythm 2015;12:e41–e63. https://doi.org/10.1016/j.hrthm.2015.03.029

5. Raj SR, Guzman JC, Harvey P et al. Canadian Cardiovascular Society position statement on postural orthostatic tachycardia syndrome (POTS) and related disorders of chronic orthostatic intolerance. Can J Cardiol 2020;36:357–72. https://doi.org/10.1016/j.cjca.2019.12.024

6. Lovett S. ‘Only a fraction’ of long Covid sufferers able to access NHS support clinics. The Independent 12 September 2021. Available from: https://www.independent.co.uk/news/health/long-covid-nhs-clinic-support-treatment-b1917924.html

7. National Institute for Health and Care Excellence (NICE), Scottish Intercollegiate Guidelines Network (SIGN) and Royal College of General Practitioners (RCGP). COVID-19 rapid guideline: managing the longterm effects of COVID-19. London: NICE, 2020. Available from: https://www.nice.org.uk/guidance/ng188

8. Royal College of General Practitioners. Long term effects of COVID-19 and post-COVID-19 syndrome. eLearning website. Available at: https://elearning.rcgp.org.uk/course/view.php?id=492

9. National Health Service. Supporting your recovery after COVID-19. Your COVID Recovery website. Available at: https://www.yourcovidrecovery.nhs.uk/

10. Grubb PB. Postural tachycardia syndrome. Circulation 2008;117:2814–17. https://doi.org/10.1161/CIRCULATIONAHA.107.761643

11. National Health Service. NHS Long Term Plan. Available from: https://www.longtermplan.nhs.uk/

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