CMAJ Podcasts

Recognizing and treating POTS

March 28, 2022 Canadian Medical Association Journal
CMAJ Podcasts
Recognizing and treating POTS
Show Notes Transcript

Postural orthostatic tachycardia syndrome (POTS) is a disorder that can profoundly affect patients' quality of life. Its main characteristic is tachycardia on standing without a drop in blood pressure. Patients complain of lightheadedness and palpitations when upright, which sometimes leads to syncope. This can cause substantial functional disability, which may be economically devastating.

Despite these serious consequences for patients, diagnosis can be delayed up to 6 years.

In this episode, Dr. Satish Raj, author of the recent CMAJ narrative review article Diagnosis and management of Postural Orthostatic Tachycardia Syndrome talks to our hosts about difficulties in making the diagnosis of POTS, its complex range of comorbidities, how patients are typically affected by the syndrome and treatment options.

Join us as we explore medical solutions that address the urgent need to change healthcare. Reach out to us about this or any episode you hear. Or tell us about something you'd like to hear on the leading Canadian medical podcast.

You can find Blair and Mojola on X @BlairBigham and @Drmojolaomole

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The CMAJ Podcast is produced by PodCraft Productions

Dr. Mojola Omole:

This is Mojola Omole-

Dr. Blair Bigham:

And I'm Blair Bigham. This is the CMAJ podcast.

Dr. Mojola Omole:

So in this episode we are discussing a paper that was published in the recent CMAJ by Dr. Satish Raj looking at diagnosing and management of postural orthostatic tachycardia. He's a cardiologist out in Calgary.

Dr. Blair Bigham:

Now, Jola, safe space here. Have you ever heard of postural orthostatic tachycardia? Because I had not.

Dr. Mojola Omole:

This is always a safe space, and no I have not.

Dr. Blair Bigham:

At least not as a syndrome.

Dr. Mojola Omole:

Not even as anything. It's like all the words put... I've heard of the words orthostatic, posture, and tachycardia, but not all together. So this was a really fascinating read.

Dr. Blair Bigham:

Absolutely. This paper makes it sound probably much more common than it's actually diagnosed, and also sort of a bit tricky to catch because a lot of the symptoms are similar to other diseases or are sort of vague and maybe get blown off early on.

Dr. Mojola Omole:

Yeah, and it seems that this is debilitating, and the age of onset is very young, and so I feel like it has a lot of psychosocial implications for patients who have this chronic disease, and as you mentioned, like with other chronic diseases, oftentimes what people say is that “everyone thought that it's all in my head”, those type of comments. So I'm curious to see if other patients have had that similar experience.

Dr. Blair Bigham:

Absolutely, and our guest who we were hoping to have on today had symptoms that began at age 12, but she wasn't diagnosed until she was 24 years old, and unfortunately she's actually having a POTS flare-up today and isn't able to join us.

Dr. Mojola Omole:

Yeah, and it sounds, just from what she was describing, to just be awful, and so I'm really interested in digging a little bit deeper to educate myself, and also to educate our listeners so we will be able to serve our patients better. So let's get started. Dr. Satish Raj, thanks for being with us today.

Dr. Satish Raj:

Thanks for having me.

Dr. Mojola Omole:

So we were planning on having a guest today, a person who has POTS but, last-minute, they became unwell and they're not able to join our recording. So is this typical for patients who have POTS?

Dr. Satish Raj:

It can be. One of the challenges with POTS is that there's an unpredictability to it. There are problems that occur and certainly good periods and bad periods. I often describe the course as being like a game of snakes and ladders. If you go two steps forward and one step back, you're doing well. But sometimes it's two steps forward and three steps back, and that is part of the challenge.

Dr. Mojola Omole:

And how long would these symptoms last?

Dr. Satish Raj:

It can vary a lot. It can go through cycles where someone especially overdoes their activity on a certain day. Maybe for a few days they're really wiped out and out-of-sorts. I think patients will feel worse for longer periods of time than you or I who don't suffer from POTS might after predictable stresses. So, for example, if they have a viral infection, we all, certainly COVID has been the dominant one, but even pre-COVID, viral infections are part of living in Canada, and I think we are accustomed to feeling unwell for a few days and you pop up, and in many cases for POTS patients that may be several weeks…

Dr. Mojola Omole:

Oh wow.

Dr. Satish Raj:

... to sort of get back to baseline. So there are certainly elements of that, and complicating all of this is that there is a lot of heterogeneity. POTS is a syndrome. It's a collection of things, and not always the same thing in everyone. So it's not the same underlying pathophysiology in everyone, and that manifests in different ways. So some patients have a lot of pain components, in addition to the tachycardia. Some have a lot of GI components, either they're having trouble keeping food down or a lot of abdominal pain and cramping and bowel motility, and so these different things can flare up and it's compounded by the fact that there is an overlap, certainly in the significant minority of patients, where they have a very strong tendency to allergies to various things.

Dr. Mojola Omole:

Oh, okay….


Dr. Satish Raj:

Environmental, food, other issues that can also, that may relate to inflammatory stress that may make overall symptoms worse, but that may be the acute trigger.

Dr. Mojola Omole:

And so how common is it for POTS patients to present with other complex conditions?

Dr. Satish Raj:

To some extent I think it depends on how hard you look. So some people argue that everyone with POTS has things other than the cardiovascular manifestations, and I'm not sure if that's true for everyone. But I think a high percentage probably do have at least some non-cardiovascular manifestations. In terms of diagnoses that are seen fairly frequently, about 30% - across different publications, and including in a big patient survey that we have led - seem to have been diagnosed with a form of Ehlers-Danlos syndrome, which is a connective tissue…it's actually not a, it's a family of connective tissue disorders. And somewhere around probably 15 to 20% seem to have very strong allergic components with that, and sometimes it's the trinity, the three of them go together and contribute different aspects, and there's still a lot of work that needs to be done and people are trying to do it to try and understand what the relationship is between those different modalities.

Dr. Mojola Omole:

How difficult is it for POTS patients to get diagnosed?

Dr. Satish Raj:

It's a challenge. I think we have seen with a lot of disorders we go through cycles of recognition. There's an internist at Yale who has written articles for the New York Times and books, who I heard speak maybe five years ago, and she was talking about how at Yale they're developing a general internal medicine subspecialty fellowship that they were colloquially calling the House Specialty, the House Fellowship, after Dr. House. And the goal was to try and send people, their trainees, to different clinics around the U.S. that specialize in uncommon things, and the reason was because she said, I think correctly, that the best predictor of a doctor making a diagnosis is that the doctor has seen it before, and that's really the challenge in POTS, right?

Dr. Satish Raj:

If you don't know it to think about it, you'll never diagnose it. In fact, even more basic is it's hard to diagnose a disorder related to orthostasis, either orthostatic tachycardia or orthostatic hypotension if you don't measure orthostatic vital signs, and that's something that, at some level, everyone that's gone through medical school probably has heard of orthostatic vital signs. But the truth is, most of us don't do it with any regularity, and so you have to then think should I do it in the circumstances? And that's the challenge.

Dr. Blair Bigham:

Satish, listening to this I'm trying to figure out, how could I pick up POTS in the emergency department? It's not uncommon that people come to the ER and say, "I've been to multiple specialists. Nothing's working. I'm still not feeling well," and they're looking for sort of that second opinion, and I feel under the pressure to come up with some sort of unifying diagnosis, and with all of these symptoms that are part of the syndrome kind of crossing different body systems, what are some of the clues or the red flags that might help someone go, "Oh, I should be thinking about POTS." I imagine that, depending on the dominant symptom, people could end up at multiple different specialists or in their GP's office multiple times. What sort of sets off that, "Oh, you know what? This is what we should consider."?

Dr. Satish Raj:

So I think to the extent that POTS is the diagnosis that we're going for, the key thing that should trigger the thought are symptoms that patients describe that are worse when they're upright and they get better when they lie down, because the patient's not going to necessarily tell you that my blood pressure's low or my blood pressure's high or my heart rate’s low or my heart rate's high. They may say "I have rapid... My heart races," because people can feel palpitations and may say that. But it may not be that simple. It may be a presentation of “I get really short of breath when I'm upright,” or, “I can't be up for very long and I feel better lying down.”

Dr. Satish Raj:

Often, the patients that I'm seeing, and maybe there's a spectrum and I might be seeing people that are on the more severe end of the spectrum, they have challenges not with not being able to… not that they're getting short of breath when doing their 5K, they get short of breath when they're standing at the stove in the kitchen trying to do some cooking. They get challenges, perhaps, walking the dog. They get challenges with basic housework. It does involve being upright, right? These are activities that are hard to do if you're not upright.

Dr. Blair Bigham:

Once you have it on your differential, what is the next step aside from just saying it's a diagnosis of exclusion? Are there any specific lab tests or clinical tests that can help me narrow down on this?

Dr. Satish Raj:

Yeah. So I don't think it's a diagnosis of exclusion. It's a syndrome, and so I think the first thing is…the syndrome largely requires two things with caveats, right? So the two things are it requires excessive orthostatic tachycardia, an excessive increase in heart rate on standing. And so for adults we define that as at least a 30 beat increase on standing from lying down within 10 minutes, and we're not trying to pick up that the heart rate goes up at one minute and comes back down. There are different aspects of physiology on standing. But the idea is with prolonged standing it gets worse. It doesn't just happen and then get better if you keep standing. That's a different disorder altogether. The other is that patients have to feel unwell, and we refer to that as orthostatic intolerance.

Dr. Satish Raj:

In fact, when the Canadian Cardiovascular Society came out with a position statement on POTS, as well as the broader issue of orthostatic intolerance, we tried to put this on a Cartesian plane to try and make this visually impactful for people that you need to have both excessive symptoms, as well as lots of tachycardia, and that may seem like a trivial point but these days more and more patients have Apple watches and have Garmin monitors and heart rate monitors. Their monitor's telling them that they're unwell, right? And that's, there's a spectrum of physiology and people can have a bit of excess tachycardia, and that's not necessarily a problem. But in POTS patients it is, because they feel horrible with it. So those are the two cardinal issues, and it has to be chronic, right? So some have said three months, some have said six months. In the Canadian Cardiovascular statement, we settled on three months. But the underlying premise is that it has to be chronic… it's not an acute illness because there's a good chance, with acute illness, if you give it a few weeks it'll get better.

Dr. Satish Raj:

Once you get to three months, I guess there's a chance, but it's less likely, and then we want to exclude other obvious incriminating and potentially reversible causes, and by that, that could be some basic medical conditions. So for example, Addison's disease. If you actually weren't able to produce cortisol and you weren't holding onto fluid for that reason and were hypovolemic, that could certainly give you a tachycardia, orthostatic tachycardia presentation. But we don't want to just give someone an extra label of POTS. It does no one any good, right? What you want is to find the Addison's, and there is a treatment for Addison's disease, and with that treatment these tachycardia-related symptoms should get better. So there are a handful of things like that that should be excluded. There aren't that many that are clearly the underlying cause, but things like that, and then the other things to be careful about are drugs. A lot of patients I see, for example, are co-diagnosed with ADHD, and certainly stimulants raise heart rate, and even things that we don't think about as raising heart rate can raise heart rate in susceptible individuals.

Dr. Satish Raj:

So, for example, some of the more common antidepressants these days are not SSRI's, but SNRI's, the serotonin norepinephrine reuptake inhibitors, and the norepinephrine reuptake actually promotes an increase, effective increase, in sympathetic tone and promotes excessive tachycardia, and so we actually have data that pure norepinephrine reuptake inhibitors can actually make POTS patients feel worse and make the tachycardia worse. So you want to make sure that there aren't drugs contributing to it. You want to make sure, obviously, there's not acute bleeding, acute hypovolemia, contributing to it. And then the other-

Dr. Blair Bigham:

Now you're talking my language.

Dr. Satish Raj:

Yeah, and the other thing that we see sometimes, thankfully not too commonly, is prolonged bedrest, and there's lots of good data - much of it funded by NASA interestingly - that prolonged bedrest will cause orthostatic tachycardia. It causes sort of a POTS phenotype. So you want to make sure people aren't spending 20 hours a day horizontal. They need to be exposed to gravitational stress.

Dr. Mojola Omole:

And what's the overlap with POTS and things such as chronic fatigue syndrome, and what's the other one? Myalgic...

Dr. Satish Raj:

Myalgic encephalomyelitis.

Dr. Mojola Omole:

Yeah. Yeah.

Dr. Satish Raj:

I'll answer that in two ways. I'd say somewhere around almost 100% of POTS patients are chronically fatigued. It's a universal complaint. Several years ago, when I was still at Vanderbilt in Nashville, Tennessee, a colleague looked at our data and took a cohort of patients and tried to map them against the CDC criteria at the time for ME or CFS, and the challenge is the criteria evolve over time, and so it may be different based on more current criteria that are being used. But it was around 50, 55% of our patients actually met CFS criteria.

Dr. Mojola Omole:

Wow.

Dr. Satish Raj:

But 45% didn't. So it's not, so I'd view these issues as overlapping Venn diagrams. I think the issue that I pointed out with POTS that's a challenge, is that is that it's a syndrome and it's heterogeneous and not due to one underlying pathophysiology. That issue is true for myalgic encephalomyelitis… chronic fatigue syndrome, and it's worse because I think it's a bigger IF... Not everyone likes Venn diagrams - I'm a big fan - but the chronic fatigue myalgic encephalomyelitis circle would be a lot bigger than the POTS circle.

Dr. Mojola Omole:

Okay.

Dr. Satish Raj:

And then parts of those circles overlap is how I would think about the disorders.

Dr. Mojola Omole:

Is there a demographic that tends to be more affected by POTS?

Dr. Satish Raj:

Absolutely. It's basically women of childbearing age, right? So we've done a big survey, a patient oriented survey to try and understand sort of the challenges and who gets it and why and when, and the mode. The most common age of onset was 14, and when we're talking about female, male, 90... I think it was around 93 or 94% at that survey, the exact numbers depend on each study and how you sample, but it's certainly north of 80% female to male. So it's much more common in women.

Dr. Mojola Omole:

And this seems like it's a massively disabling syndrome.

Dr. Satish Raj:

It is. There's a spectrum, obviously, and, but it absolutely is. In fact, there was a paper that we've published recently out of that survey trying to quantify some of the economic impact. But absolutely, there's a huge amount of inability to do things. I was going to say inability to go to work. Actually, with the most common age of onset at 14, the first issue is school.

Dr. Mojola Omole:

Yeah.

Dr. Satish Raj:

Right? So there's a very high burden that we found of kids that had missed school. That was almost universal, kids that had missed some school because of this illness. But what was more troubling is that a very high percentage actually had to be pulled out of school for a while, or had to be homeschooled because they weren't able to physically sit in school and to learn, but-

Dr. Mojola Omole:

Because of fatigue, or...

Dr. Satish Raj:

It could be, we didn't ask exactly why, but I suspect it's a combination of fatigue, a combination of orthostatic intolerance… you have to be able to sit.

Dr. Mojola Omole:

Yeah.

Dr. Satish Raj:

You're not necessarily standing all the time, but you're sitting vertical and that can be a problem as well. There may be other related issues, but through a combination of this, there are a lot of people that had to be pulled out of school, a lot of people that had either had to take a break in university or not to do the things that they would do, and the challenge there is it's not just the acute loss, but… I view that decade from, say, 13 to 23, as one where you're really determining the trajectory for your life in some ways. That's when you're getting a lot of your education done that may determine how you do socioeconomically, not just for that decade, but for the next four or five decades.

Dr. Mojola Omole:

And also your social interactions. It must be massively isolating to be a 14 year old who is stuck at home because they're unwell.

Dr. Satish Raj:

It gets, it's worse than that. So part of it is it's isolating because you're not interacting in the same way, and part of it is that one of the challenges, and there are many, with POTS is that it probably falls under this umbrella of invisible illnesses. One of the challenges, when you look at these patients, they don't necessarily look sick, and they may even be able to be up and around for a few minutes, and then it wipes them out and they're not able to and it's hard to look at them and necessarily understand why, and I think that that adds to the difficulty, and at one level you can say, "What do strangers know, and who cares?"

Dr. Satish Raj:

But part of the challenge is it's not just strangers. It's often family, sometimes close family, sometimes extended family. For patients that are a bit older who are in relationships already or married, this is a huge stressor, where you have to… it changes the dynamics totally.

Dr. Mojola Omole:

Of course.

Dr. Satish Raj:

And it's not, I don't want to make it sound like that's unique to POTS. That's probably true of a lot of chronic illnesses, but it certainly has a very real impact on their lives.

Dr. Mojola Omole:

Massively, and so what are some of the treatments that are available and how effective are they?

Dr. Satish Raj:

So I think we treat some symptoms better than others. I think the cardiovascular issues, the excessive tachycardia, the issues maybe with soft blood pressure, I think, I believe that I can make a lot of people function better. I can't cure anyone. The truth is I can't do these three things and make it all go away. But I think with sort of a multi-modality treatment, a lot of people can function better than they were. The goal is to get them to function as best as they can, get them back to work if that's what they want to do, and that's not always possible fully but usually we get them better than they were. There are other symptoms that, quite frankly, I do a really poor job of treating. Fatigue, which you brought up, is one of them. The patients will often describe a debilitating fatigue, and in some cases that's what limits their ability to go back to work and function, or function at the same level they did before, and there is a great need to try and understand that better.

Dr. Mojola Omole:

If a family physician is able to diagnose POTS, what do they do next?

Dr. Satish Raj:

In these patients with excessive tachycardia, I think treatment, I would argue, starts with a foundation of non-pharmacological approaches, and then we layer upon that pharmacological therapies as needed. So the tachycardia is the hallmark finding. That's the low hanging fruit. We all can measure heart rates fairly easily in clinic. But if you want to get to why they're tachycardic, a lot of POTS patients - and this has been shown by our group and other groups - tend to have a fairly low stroke volume, especially on standing, and part of the issue is there's not enough, we think there's not enough blood getting back to the heart, and that lowers cardiac venous return, lowers stroke volume.

Dr. Satish Raj:

So we've done, recommended clinically and done some work to look at the effects of compressing the lower body to try and minimize those fluid shifts, and in fact there was a nice proof-of-concept study that one of my PhD students published last year in the Journal of the American College of Cardiology where we looked at segmental compression, looking at the full lower body compression from the belly down, to bicycle shorts compression versus sort of a calf compression, versus no compression with repeated tilt tests where we had people lying flat and then tilting them up to look at the orthostatic tachycardia. And what we showed very nicely was that it was in an almost dose dependent manner.

Dr. Satish Raj:

The more you compressed, the lower the heart rate increase was. So, heart rate still went up, but it went up... you blunted that increase. And, importantly, that actually decreased the standing symptoms as well. So we rated symptoms at the end of each stand, and so the higher the heart rate went, the worse the symptoms were. So if you could blunt that, you actually improve symptoms, and so we, these were proof of concept garments. It's something that no one would actually wear in their right mind in the real world, but we recommend-

Dr. Mojola Omole:

Have you seen women's clothing?

Dr. Satish Raj:

But we, what I was going to say is that we actually are doing a study right now with commercial garments. So, one, you can prescribe pantyhose-style stockings that are hot, tight, itchy, and ugly, and hard to get on. But for some people they do it because that keeps them functioning. But, before going to that, I often recommend things like triathlon tights, compressive garments that are commercial, that don't look as ugly as medical grade things that our young patients are more likely to wear, and that may be what you're referring to.

Dr. Mojola Omole:

Yeah.

Dr. Satish Raj:

But things like Spanx where we focus on compression from the sort of upper thighs to the ribcage, focus on the abdominal compression or the bicycle shorts style compression, because that's actually where a lot of the fluid sits. 


Dr. Satish Raj:

And then the other nonpharmacological treatment that we found to be important in the tachycardia group in patients with POTS is an aerobic reconditioning program, and this is somewhat controversial because in the chronic fatigue myalgic encephalomyelitis literature, there is a sense that exercise will harm patients and because of the overlap with fatigue, that there are some patients that'll push back saying they've heard it'll cause permanent damage. But in the tachycardia, certainly in this group with excessive tachycardia, it does help. So we recommend things like rowing machines or recumbent cycles or swimming, and for the aerobic benefit they have to do it frequently.

Dr. Satish Raj:

We try and get people up to 30 minutes, but try and really reduce the load, and the challenge is they're going to feel worse before they feel better, and it often takes, say, six weeks before they notice any benefit, and I have many patients that have gone through this and there is an improvement. There's the light at the end of the tunnel, but it's a long tunnel. If the heart rate is really high, often I'll use a little bit of low dose propranolol, a little bit of beta blocker, just to take the edge off. But the important thing is you don't want to fix the heart rate. If I correct the heart rate, if I normalize the heart rate, patients often feel worse because, again, there's a different underlying problem.

Dr. Satish Raj:

Often it's that the stroke volume’s low and the heart rate's compensatory, but sometimes if the compensation's too aggressive it causes its own problem, and that's really what we're trying to fix with things like a little bit of propranolol or a little bit of midodrine, which is a drug that is a pro-drug of an Alpha 1 adrenergic agonist. Or, more recently, there's a drug called ivabradine that's on the market that is actually approved for heart failure, slows down the heart rate a little bit like a beta blocker, but is pretty restricted to the sinus nodes. You don't have other beta blocker side effects if someone is prone to asthma or have other side effects that are specifically beta blocker-contraindicated.

Dr. Mojola Omole:

Interesting. Awesome. Thank you so much.

Dr. Satish Raj:

Thank you for having me.

Dr. Mojola Omole:

Dr. Satish Raj is a cardiologist in Calgary.

Dr. Mojola Omole:

Wow, Blair, that was quite eye opening, and I learned quite a bit. What are some of your takeaways from it?

Dr. Blair Bigham:

So I think for me, just having it in mind as a diagnosis is something that I'm going to try to have on the top of my mind the next time I'm at work. And certainly the workup sounds fairly straightforward, at least initially: the AM cortisol, making sure their TSH is okay, checking a CBC to make sure this isn't attributable to something like anemia. Those are all workups that I can do at the emergency department, I can do in a clinic, and certainly get the ball rolling.

Dr. Mojola Omole:

Yeah, for sure and, for me, as I was listening to some of the gastroenterology symptoms, maybe now, especially if I have younger patients, that before we start thinking of whether this is just IBS is just to ask them a few other questions, just to also help our family doctors who are always overwhelmed at having to know every single thing in the book, just help guide them to just help them so we can have a better idea of what's going on with a particular patient.

Dr. Blair Bigham:

Totally, and the biggest take-away for me, and it's tricky to do in real life because a lot of things are virtual or you're in a hallway in a busy emergency department, but getting those orthostatic vitals. They teach you in medical school and it's always that exam answer during an oral board, make sure you have your orthostatic vitals, but in real life everyone's so busy. The nurses will always give me cut-eye if I order orthostatic vitals, and sometimes I'll feel compelled to do them myself just to not put too much of a burden on them. But that just sort of adds to the complexity when you're in this busy emergency room or doing something virtual and you don't have space to lay someone down and take their vital signs.

Dr. Mojola Omole:

For sure. Yeah, and I think what's really key was that it's not something that you see right away, that you have to wait 30 minutes, and so I do think that there needs to be a greater emphasis on thoughtfulness around this and planning for it, and maybe, as a non-family doctor looking in, is that when you do have that young, usually female patient with this constellation of symptoms that just does not all fit, is that maybe this should be something that should be high on your diagnosis list, and to create that space for measuring the postural vitals and hopefully getting the diagnosis to help them.

Dr. Blair Bigham:

That's it for this week's episode of the CMAJ podcast. Please remember to share or review our podcast wherever you download it. It really helps us get the word out.

Dr. Mojola Omole:

And smash that like button. Little levity to the day. I'm Mojola Omole.

Dr. Blair Bigham:

And I'm Blair Bigham. Thanks for listening, and be well. Oh God, I stole your tagline, Jola.

Dr. Mojola Omole:

I know. He stole my tagline, everybody.

Dr. Blair Bigham:

You're going to come after me.

Dr. Mojola Omole:

Please put this part in. He stole my tagline. I'm saying it next time.

Dr. Blair Bigham:

We’ve got to shake it up. That's all.