Does POTS need a new name?

The term ‘POTS’ (postural orthostatic tachycardia syndrome) was coined in 1993 by physician Dr Philip Lowe. This initial definition related to chronic orthostatic intolerance, in association with excessive increase in heart rate with standing, but without a fall in blood pressure. 

It’s a condition that affects more women than men, with many patients spending years (an average of seven) searching for a diagnosis.

Dr Susan Corcoran is a Cardiologist at Bayside Health (Alfred and Caulfield Hospitals) and has worked in the research and management of conditions associated with orthostatic intolerance over the last 25 years. 

She says that the current label is not serving patients well, and in some cases, isn’t serving doctors. 

“A shift away from a specific label would lead to a better understanding of the frequency of orthostatic intolerance in the community, and early validation of POTS symptoms, and implementing simple strategies to manage the condition,” says Dr Corcoran.

What is POTS?

A consensus definition coordinated by the Heart Rhythm Society in the US defines POTS as a clinical syndrome usually characterized by:

• frequent symptoms that occur with standing, such as light-headedness, palpitations, tremor, generalized weakness, blurred vision, exercise intolerance, and fatigue; 
• an increase in heart rate of >30 (bpm) when moving from a recumbent to a standing position (or >40 bpm in individuals 12 to 19 years of age); and 
• the absence of orthostatic hypotension (420 mm Hg drop in systolic blood pressure).

Other associated symptoms include, bloating, nausea, diarrhoea, abdominal pain, sleep disturbance, and migraine headaches.

“POTS is now often inappropriately used to describe anyone with frequent symptoms when upright. I am seeing patients with a profound fall in blood pressure when they stand, who may have a degenerative neurological disorder, being told they have POTS,” says Dr Corcoran.

“Further difficulty comes with the broader use of the term POTS in people with orthostatic intolerance, who also have other, overlapping conditions that aren't related to the cardiovascular system.”

“A patient may hope a cardiologist can help with management of the complex overlapping conditions. Cardiologists feel they can’t manage these patients as they don’t have the training.”

A complex condition

40% of women and 25% of men will faint at some point in their lifetime. There are many causes of orthostatic intolerance. Currently the most common referral question for someone with symptoms when upright, is POTS. 

“In fact, vasovagal syncope, vasovagal presyncope and orthostatic hypotension are much more common than POTS,” says Dr Corcoran.

“There is significant overlap in orthostatic symptoms and also heart rate changes between POTS, Vasovagal syncope, and orthostatic hypotension.”

Dr Corcoran says that the focus on heart rate also means patients who may have conditions unrelated to the cardiovascular system, such as Persistent Postural-Perceptual Dizziness (PPPD) or Vestibular migraine, may not have those conditions managed. 

“The NIH has described a group of overlapping conditions — most recently using the overarching term of “chronic overlapping pain conditions” (COCP).

“As patients with orthostatic intolerance often have these overlapping conditions under the umbrella of (COCP), they can be disappointed that management of the cardiovascular manifestations does not improve other overlapping disorders if they consider these conditions under a POTS umbrella.”

Dr Corcoran says that including chronic orthostatic intolerance under the COCP umbrella may be a useful way forward.

“In the Victorian context, we've recently had the investigation into women’s pain. That enquiry didn’t include orthostatic intolerance, because it's not perceived as pain. But it is discomfort.”

The frustration is real

For patients wanting answers, the frustration is common, and valid. Health care professionals understand, but are also frustrated.

“If you look on Reddit, chats, and social media, it'll say things like, ‘A doctor didn't want to give me a diagnosis of POTS.’ And I'm part of that,” says Dr Corcoran.

“Why don't I want to automatically give someone a diagnosis of POTS? Because I think it's often too simplistic.”

“I'm trying to give that person a sense of control. To say, ‘OK, this is what I see is happening with you. Let’s talk about strategies we can use in different circumstances.’”

“But we need to work through a process.”

“We're not looking at the complexity of mechanisms that might be at play. And a lot of those mechanisms we still don't understand.”

“The area that we don’t understand, yet, is epigenetic mechanisms. Epigenetics is a rapidly expanding area that we still don't have enough understanding.”

“It’s really difficult for patients, and it's difficult for doctors. In medicine we are not very good at sitting with uncertainty. It’s really hard saying we don't know.”

Dr Corcoran says that a name change may lead to better knowledge for doctors, and better service for patients. 

She cites the recent change from PCOS to PMOS as a good example of the need for a change.

“The acronym polyendocrine metabolic ovarian syndrome (PMOS) for the former polycystic ovarian syndrome (PCOS) takes the focus off the ovaries, and reflects the broader presentation of the condition.”

“Irrespective of whether they have those heart rate changes, if someone has symptoms such as light headedness, palpitations or dizziness when upright, those symptoms warrant management and advice.”

Growing body of research

Claire Seeley is a research Fellow at the South Australian Health and Medical Research Institute and CEO of The Australian POTS Foundation.

“Our foundational research has been trying to establish and understand how impactful POTS is. It is a significantly terrible quality of life; 58% of people who get POTS don’t return to work. It’s a really disabling condition,” says Dr Seeley.

"If you don't have clinicians who understand that nuance, these patients don’t get the treatment or recognition they deserve — or the validation. Patients don't get taught how to manage the condition. And they often don't get offered the medications that might support them in improving their quality of life.” 

"Problem number one — not only just POTS — but autonomic disorders are not taught well to GPs. Our medical system, our education system, has failed to really support our GPs and clinicians."

"We did a survey of 2 300 GPs, and this article is still under review, but only 3% had ever had formal training in POTS."

"Women take double the time for diagnosis compared to men, despite being 90% of the cohort. 60% of these people present to emergency departments multiple times before they get a diagnosis."

The Australian POTS Foundation has resources for GPs regarding POTS.

Dr Corcoran says specialists at the Alfred would love the funding for a network of interrelated clinics to serve patients with diverse POTS symptoms.

“The Functional Gut Clinic at the Alfred is an amazing clinic, and that’s one of the overlapping conditions. And they have doctors, psychologists, and dietitians. What we actually need is a service integrating the functional gut clinic, the headache clinic, the pain clinic, so that rather than duplicating, people with symptoms can go to one service where we can look at all of these services and how they relate to each other.”