Pots Sub-Types?

[MommaBear]

I have been reading a lot about the sub-types of POTS here and there and have a few questions.

Does anyone know of a place where the sub-types are listed as well as the criteria/symptoms that makes them unique?

What tests are done to test for each sub-type?

Are some sub-types associated with a viral beginning vs not.

We have been struggling with this for several years with my now 16 year old daughter, but just got a POTS diagnosis on April 2. The neuro is marching down the line of drugs for the past several months, which seem to have a lot of side effects and nothing working. We have just seen a tad bit of improvement with the start of Mestinon 4 days ago. But when you are already bed ridden and a drug makes you even worse, its pretty bad off as you all know. I guess I am trying to put some logic to this process and see if we can narrow down our focus as opposed to just trying all the drugs associated with POTS and seeing what works... is that even possible - logic seems absent with this syndrome, but I do believe it is there somewhere.

Thanks for any help.

[arizona girl]

Hi, because of your daughters age she falls into the classic age group of patients being first diagnosed with pots, there is more research in this age group and it states that many in this age group recover. I hope that for your daughter. I also presented even younger then your daughter, though no one knew at the time, because I didn't tell anyone. I am now 55 and the cause of my pots symptoms are autoimmune and a defective immune system.

So, it really depends on what may be causing it. Are her symptoms only a rise in heart rate on standing or does she have other seemingly unrelated symptoms that are governed by the autonomic nervous system. Like digestion issues, or sweating issues, or moisture issues, eye problems. You get the idea anything the autonomic nervous system does automatically for us. These are questions I would put to your doctor.

Have they looked for any type of cause or done any type of testing to rule out other conditions that might be contributing. A real easy one that causes high heart rate is anemia, correct the anemia and the heart rate settles down. So the treatment depends on the cause. Many doctors right now are only treating symptoms, which is also good too, to calm them down.

However if the cause is from something like mast cell, or autoimmune, etc, treating only symptoms means that the primary cause is not being addressed and the body will continue to decline, which is what happened with me. Only in the last 5 years have they been able to diagnosis and start treating me properly. What I have is chronic and not going away, but it can be managed. Also there are no perfect medicines out there many have unwanted side effects. So you have decided what you will tolerate and what you won't. Some on the forum have decided to live with it as is, because they couldn't tolerate the meds.

I tolerate most of my meds pretty well now, after a lot of trial and error. Everyone reacts to meds differently, so the only way to know is to try. I think sometimes we may give up on a med to soon, as sometimes the symptoms will dissapate as the body gets used to it. But if after a fair run like 3 months and side effects still aren't tolerable, I'd talk to my doctor and try something else. Though there are some side effects where you should stop the drug immediately, you need to ask your doc and pharmacist about them.

Hope this is helpful and this is just my opinion.

[McBlonde]

But (as I understand it) knowing you sub-types of POTS, does not bring a cure in most cases, right? Or do I have that wrong?

[McBlonde]

I should have added... this is one of the things that confuses me most. I see people that know their sub-types, but they are still sick.... Does that makes sense? I have a lot of brain fog today #Sorry

[jpjd59]

MommaBear:

This is an interesting article (from Europe) that talks about the different sub-types.

europace.oxfordjournals.org/content/13/3/306.full

[arizona girl]

Posted the article since the link didn't work. I don't think ivabradine is available in the USA yet. Is that right anyone?

As far as the subtypes go the research that is being done on treatments for symptoms is now starting to define the different ways orthostatic intolerance presents. If you've been reading on the forum you will see all these types presenting differently in the forum members. So, how you treat someone who gets hypotensive on standing may be different then someone who becomes hypertensive on standing, or someone who only gets a high heart rate. This article discribes perfectly what happens to me when vasovagel collapse happens, I am super high in hr/bp and then I suddenly tank when my body can't take it any more or there is an additional stressor added in, then a collapse and can't get up.

So these treaments as you see from the article may or may not help. It is important to note that this is treatment for symptoms only and does not go to defining what may have caused the orthostatic intolerance to begin with. I hope that is clearer. I think I've got that part right. If not please chime in!

Richard Sutton& and Tushar Salukha - Ivabradine in the treatment of orthostatic intolerance Author Affiliations

International Centre for Circulatory Health, Imperial College and Imperial College Healthcare NHS Trust, 59-61 North Wharf Road, London W2 1LA, UK *Corresponding author: Tel: +44 207 935 1011, Fax: +44 207 935 6718, Email: r.sutton@imperial.ac.uk

This editorial refers to ‘Single centre experience of ivabradine in postural orthostatic tachycardia syndrome’ by C. McDonald et al., on page 427.

Attention was drawn to postural orthostatic tachycardia syndrome (POTS) in 1993 by Philip Low's team at Mayo Clinic.¹ Postural orthostatic tachycardia syndrome has remained difficult to treat despite almost 2 decades of expanding research and recognition.

The typical patient is a young female, 15–40 years of age, whose symptoms are usually multiple. In combination, the symptoms are very debilitating and those most common are fatigue, orthostatic intolerance, palpitation manifest as sinus tachycardia, oedema and discolouration of the lower limbs, and occasionally syncope.

Two sub-types of POTS have been described. In the first of these, a primary neurological defect is present, possibly triggered by a viral infection. This defect takes the form of a partial autonomic denervation leading to excessive venous pooling in the legs and, sometimes, additional affection of the renal innervation resulting in a reduced plasma renin activity. The second sub-type is considered to have central sympathetic activation, termed the hyperadrenergic type.^2,3

In 2005, a possible third sub-type was described that clinically presents facial and/or upper thoracic flushing, in some triggered by exercise, together with the typical symptoms noted above.⁴The explanation of the finding is postulated to be the release of a vasodilator from activated mast cells, supported by a raised urinary methylhistamine.⁴ These patients, as might be expected, may be worsened by beta-blockers but may respond to H1 and H2 histamine blockers with or without alpha-methyldopa. This third sub-type of POTS may present raised blood pressure at times in association with flushing and offer a differential diagnosis from phaeochromocytoma. This sub-type may only be a small percentage of those with POTS but, for them, there may be a specific therapy, anti-histamines not beta-blockade.

For the majority, therapy remains incompletely effective. Contemporary therapy includes increased fluids and salt, support stockings, counter-pressure manoeuvres,⁵ exercise, fludrocortisone, beta-blockers, midodrine, pyridostigmine, octreotide, erythropoietin, and the inspiratory impedance valve.⁶ These have been well described in a recent review in the Journal.⁷ Combinations of these measures frequently offer a greater response. McDonald et al.⁸ implied the benefit in symptomatic terms for POTS patients from the use of ivabradine and may thus be pointing us a new direction.

Ivabradine⁹ is a novel If channel inhibitor which slows phase 4 sinus node diastolic depolarization directly promoting bradycardia. The drug is approved in many countries for treatment of angina on an evidence base¹⁰ but, in the UK, it is currently not licensed for the POTS indication. By reducing or preventing the symptomatic sinus tachycardia, McDonald et al. have shown, in a retrospective case series, that control of one symptom is not only valuable in itself but might also contribute to resolution of others in this complex syndrome.

In addition to the reported benefit in POTS, there may be some in vasovagal syncope from ivabradine therapy. In the original VASIS classification of vasovagal collapse patterns, an exception to the standard ones (mixed, cardioinhibitory, and vasodepressor) was excessive heart rate response, where the heart rate was >130 bpm just prior to collapse, in a few per cent of tilt-positive patients.¹¹

At our centre, we see 1000 patients per year with orthostatic intolerance and currently have 20 patients on ivabradine therapy (10 with vasovagal syncope and excessive heart rate response and 10 with POTS). We use 5 mg twice daily in the majority and have found help in combination with midodrine in a few. Two patients have abandoned therapy because of lack of benefit, and side-effects have been minimal without serious visual disturbances. The remaining 18 patients continue therapy, with a maximum duration, to date, of 18 months. All have notable benefits in terms of palpitation and others of their symptoms.

Now seems an appropriate time to consider a randomized controlled trial so as to provide, if possible, a firm evidence base for the use of ivabradine in POTS.

[McBlonde]

Thank you for posting that for us Arizona girl!

[MommaBear]

Thanks Arizona Girl. My daughter had a virus while living in Australia, Ross River Fever - which has now been directly linked to ME/CFS. She never really recovered, suffering fatigue, dizziness and exhaustion after the viral symptoms faded. I am currently looking at the research on ME/CFS along with treating POTS, and reading a book by Dr. David Bell, Cellular Hypoxia an Neuro-Immune Fatigue. He feels it is the core to ME/CFS, Fibromyalgia, POTS/Dysautonomia, Chemical senstivities, and Chrongic Lyme Disease.

So, we are trying to wrap our heads around all of this, as its clearly complicated.

Thanks jpjd59 - great article that answers my question as to the sub-types. The article lists the sub-types of POTS as the following: (quoting the Oxfordjournals article cited above)

Two sub-types of POTS have been described. In the first of these, a primary neurological defect is present, possibly triggered by a viral infection. This defect takes the form of a partial autonomic denervation leading to excessive venous pooling in the legs and, sometimes, additional affection of the renal innervation resulting in a reduced plasma renin activity. The second sub-type is considered to have central sympathetic activation, termed the hyperadrenergic type.^2,3

In 2005, a possible third sub-type was described that clinically presents facial and/or upper thoracic flushing, in some triggered by exercise, together with the typical symptoms noted above.⁴

The explanation of the finding is postulated to be the release of a vasodilator from activated mast cells, supported by a raised urinary methylhistamine.⁴ These patients, as might be expected, may be worsened by beta-blockers but may respond to H1 and H2 histamine blockers with or without alpha-methyldopa. This third sub-type of POTS may present raised blood pressure at times in association with flushing and offer a differential diagnosis from phaeochromocytoma. This sub-type may only be a small percentage of those with POTS but, for them, there may be a specific therapy, anti-histamines not beta-blockade.

[MommaBear]

Sorry to repeat what you wrote Arizona Girl, your post didn't come up when I started writing my post.

I am wondering if there has or could be a survey done of the members on this forum as to those who know their sub-types and what their symptoms are and what therapies have been tried and worked/not worked. It would take some time, but think it would be an interesting study. I know there are drs who have studied their patients over time and looked at similar things, but this forum seems to have such a broad spectrum of people.

Anyone think that would be worthwhile?