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hyperadrenegric POTS


Radha
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My understanding is that one of the main differences is that in hyperadrenergic POTS blood pressure increases on standing as opposed to no change or lowered bp on standing. I won't venture beyond this b/c my understanding is otherwise shaky. Perhaps others can give better insight!

IGNORE THIS STATEMENT AND SEE MY RESPONSE BELOW. THIS IS NOT CORRECT! <_<

Edited by MomtoGiuliana
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Guest tearose

My understanding is different.

I have hyperadrenergic pots which was dx at Mayo.

I do have plummeting bp on standing and my heart rate will take off and upwards till I eventually pass out or sit down. The reason I have the "Hyperadrenergic" label is because the chemical that is surging through my blood during the ensuing autonomic storm is called norepinepherine. It is produced in massive quantities to force my heart to beat more to get blood to my brain. I have no trouble with seratonin. I thought I got this label because of the major norepinepherine release.

let's see what the next person understands...

tearose

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I can add this ... According to the NDRF handbook's glosary, hyperadrenergic orthostatic intolerance is "a condition where an inability to tolerate standing up is combined with signs or symptoms of excessive levels of catecholamines such as epinephrine (adrenaline)."

Here's the URL to a page from the Handbook for more info: http://www.ndrf.org/NDRF%20Patient%20Handb...cA_pp59-134.PDF

It's a little confusing, but it's something!

Best,

Merrill

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I found this definition. Tearose you are of course correct that you can have hyperadrenergic POTS and still have low blood pressure on standing.

"...patients with incapacitating orthostatic hypotension can have either a "hyperadrenergic" or "hypoadrenergic" presentation. Although the latter is related to overt autonomic neuropathy, the former is proposed to be explained by appropriate autonomic responses. We hypothesize, however, that both conditions are part of a spectrum of autonomic dysfunction. "

"Two distinct clinical presentations of orthostatic hypotension have been described ... The distinction was based on their plasma norepinephrine response to standing. Patients with a blunted norepinephrine response, "hypoadrenergic" orthostatic hypotension, were considered to have true autonomic neuropathy. Other patients appeared to have an exaggerated norepinephrine response to standing, a condition termed "hyperadrenergic" orthostatic hypotension (8), for which the pathophysiology has not been completely elucidated..."

It appears that in a hypoadrenergic response, hr on standing does not increase as significantly. That's where I was getting confused, I guess.

Here's where I found the quote above. This is an article about POTS in diabetic patients, but I assume the definitions are universal.

http://care.diabetesjournals.org/cgi/content/full/26/7/2174

Hope this is more helpful than my first response!!

Katherine

The question I have after reviewing this is--isn't POTS by definition hyperadrenergic? Can you have POTS that isn't--and what would the definining symptoms be in a non-hyperadrenergic POTS patient? Certainly you can have orthostatic intolerance that is not hyperadrenergic, but POTS?

Great question, Rahda. These definitions of autonomic dysfuction conditions ARE confusing. It certainly helps to have our terminology straight when researching for ourselves and talking to our doctors!

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Guest tearose

Radha, the way they found mine was with a blood test that; a Specialized Catecholamine Test, which is done over about 1 1/2 hours. Your blood is drawn over a period of time in a quiet room. First blood is drawn upon arrival. Second blood is drawn after you have rested in a darkened room in a bed. Third blood is drawn after sitting up for a time. Fourth is drawn after standing still for 10-15 minutes.

The bloods have to be sent to a special lab for analysis. I had mine done at Mayo.

(Don't let this deter you , sometimes Mayo can teach your local lab how to perform the testing and how to send the samples in specialized containers)

They also found denervation in my legs which further supports the hyperadrenergic diagnosis. This denervation of small nerve fibers was found during the thermoregulatory sweat test. (not the same as the QUSART sweat test)

hope this answers your question

best regards, tearose

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I thought all POTS was hyperadrenegric. Otherwise, how does one explain the increase in heart rate that is usually the symptom that is relied upon for diagnosis.

If the body was not over producing norepinepherine or the body was not over sensative to the norepinepherine present upon standing, the sympathetic nervous system would not be overactive, therefore the symptoms of POTS (tachycardia, tremolousness, etc) would not be present. The tachycardia is a direct indicator of sympathetic overactivity or accessive norepinepherine levels.

If there is an obvious decrease in BP upon standing, according to my doctor, this is Postural hypotension, which he feels is quite different from Postural Orthostatic Tachycardia Syndrome. Accordingly, POTS patients can often have completely normal blood pressure upon standing (or even hypertension), but due to the vasoconstricting properties of accessive norepinepherine and sympathetic overactivity, blood flow to the brain is decreased to such a level that it causes dizziness. Thus you get dizziness, tachycardia, tremors, mental confusion, etc.

My doctor told me that most POTS patients have an everactivity of their sympathetic nervous system upon standing upright for some time that is five times above that measured in normal patients.

Lastly, as part of my TTT i had an electrode measuring my sympathetic nervous system and also a pipe into an artery measuring my BP and norepinepherine levels. It was all done at the sametime.

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ramakentesh

I agree with your line of thought b/c I also thought that all POTS is hyperadrenergic (see my last post in this thread). I was hoping someone more knowledgeable, or someone with a non-hyperadrenergic variant, would respond to clarify.

Anyway, here is a quote from the recent article on mast cell activation disorder in some POTS patients. This states there are (at least) two variants of POTS--one neuropathic and one hyperadrenergic. This sentence is in the introduction of the article:

"In the neuropathic variant, the

primary defect is thought to be a partial autonomic denerva-tion

that compromises lower limbs with exaggerated ortho-static

venous pooling,1 and perhaps the kidneys with low

levels of plasma renin activity.2 Patients with the hyperadren-ergic

variant are thought to have centrally driven sympathetic

activation.3

Here are the references to the above statements from the article:

1. Jacob G, Costa F, Shannon JR, Robertson RM, Wathen M, Stein M,

Biaggioni I, Ertl A, Black B, Robertson D. The neuropathic postural

tachycardia syndrome. N Engl J Med. 2000;343:1008 ?1014.

2. Jacob G, Robertson D, Mosqueda-Garcia R, Ertl AC, Robertson RM,

Biaggioni I. Hypovolemia in syncope and orthostatic intolerance role of the

renin-angiotensin system. Am J Med. 1997;103:128 ?133.

3. Jordan J, Shannon JR, Diedrich A, Black BK, Robertson D. Increased

sympathetic activation in idiopathic orthostatic intolerance: role of systemic

adrenoreceptor sensitivity. Hypertension. 2002;39:173?178.

The answers to Radha's questions *might* be available in these articles, if no one else can completely answer them. I think tearose has already explained the conventional testing for the hyperadrenergic variant. If someone can't stand at all though--as Radha states--how do you do this test?! Can it be partially done (just not the standing part?) This is probably a question for a specialist.

Katherine

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