Hyperadrenergic Pots Diagnosis Questoin

[avidita]

Hi all:

I hope your holidays were fine!

I am still overseas getting tested . The doctors here are not quite familiar with the tests for hyperadrenergic POTS in particular, so I was wondering, if anyone has been diagnosed with it and how exactly. I know they take the catacholomines (spelling?) lying, sitting and standing, but what is the procedure exactly. How long to you stay in each position before they take blood, etc.? What other tests do they have for it? My doctors will do any tests I want, but I'll have to tell them how to perform it exactly, so I can use the results later... Anyone who has experience with hyperadrenergic POTS and its diagnosis in particular will be appreciated

Thanks all and I hope you have a wonderful and symptom-free weekend!

M.

In case anyone is curious about my tests so far:

EMG - normal

TTT - potsy, but surprisingly better than the beginning of the year (my pulse jump was only borderlining, like 35 beasts more than supine; actually my pulse has been normal for the past few days for some reason, like 100 walking, 65 sitting, 50 laying down, dunno why, still symptomatic...)

Very extensive blood work: mostly normal, but with low Magnesium Level, very high B12 levels (I do take supplements), and the real kicker - confirmed allergy to soy, rye and gluten and nut intolerances, which is great news for me, because that is literally ALL I eat (soy burger with bread for breakfast, soy shake for lunch, etc.), so if I modify my diet, I'm hoping for improvement ; no Lupus; Lyme's decease still not ready; Differential blood work a little off, but that will be discussed with my docs later; thyroid OK; sex hormones OK; Slightly high Chlorine in blood, which may mean dehydration; I'm sure I'm missing something, cause it's 3 pages of fine print test results, but they are fine

ENT workup - normal with the usual slight deviation in my VEMP test, which is very minor and should not be affecting me much according to the doctors

EKG - normal

Coming up next week: Holter Monitor, heart echo ( I have a tricuspid valve prolapse, just need to get a picture of it) and endocrinology tests (catacholomines in particular)

[janiedelite]

Sounds like your docs are being thorough. That's great! When I had catecholamines drawn at Mayo, they started by inserting an IV that they could draw blood from. Then I laid down in a quiet room for 30 minutes or so. Then, while I was still resting, the nurse drew some blood from the IV. Then I was told to do whatever made me feel symptomatic (standing, being upright, etc.) for 10 minutes. Then I had my blood drawn for the last time and they removed the IV.

[firewatcher]

Mine was assumed because of the responses that I had to the standard four ANS tests: poor-man's tilt, valsalva, paced breathing and ice bath. My BP spikes under autonomic stress, particularly the diastolic part. I did not have catecholamines drawn.

[avidita]

Mine was assumed because of the responses that I had to the standard four ANS tests: poor-man's tilt, valsalva, paced breathing and ice bath. My BP spikes under autonomic stress, particularly the diastolic part. I did not have catecholamines drawn.

Hmmm. They didn't do anything other than the TTT in the ANS lab, but the doctor insisted that based on my readings there is no way I have hyperadrenergic POTS. How much did your BP spike exactly? Do you remember? Was that during the TTT?

[firewatcher]

Instead of dropping when upright, my BP climbs...instantly. I don't have orthostatic hypotension, I have orthostatic HYPERtension. During my testing at one minute upright it was 148/98 with a HR of 123. It got to 148/117. If I raise my arms above my head it will go to 173/149. But, I still have the 30+ bpm increase on standing. Walking will send it anywhere from 130-180 bpm. Supine my BP is typically 98/60 with a HR of 48-60 bpm.

I had my testing at Vanderbilt University and they didn't do a Tilt table test on me, but they did the supine to standing HR and BP measurement.

Edited December 5, 2009 by firewatcher

[ramakentesh]

The tests for hyperadrenergic POTS are still confused because some major bodies in POTS research dont even accept the diagnosis as a different clinical entity to other forms of POTS.

The tests usually conducted by Toledo and Vandebilt are the standing norepinephrine levels being above 600 pmol. Orthostatic hypertension is considered to be a sign of primary sympathetic overactivity. These patients also have impaired beta 2 receptor vasodilation, hypertensive responses to fear and stress and high norepinephrine and epinephrine levels on standing, cold sweaty feet and hands during episodes and an excessive MSNA (nerve firing rate from the central nervous system) response to orthostatic and general stress.

The suggested mechanisms are:

Vandy: Impaired norepinephrine reuptake, leaving it at the synapctic cleft for longer and resulting in over stimulation from orthostatic stress (although one study where NEt was implicated did not find an excessive level of norepinephrine in patients suggesting they werent hyperadrenergic)

Impaired beta 2 receptor vasodilation - either as a primary problem or a result of excessive sympathetic overactivity. This means that epinephrine and to a lesser extent norepinephrine when activated in plasma only activate beta 1 receptors to induce tachycardia and vasoconstriction without the secondary beta 2 activity which results in vasodilation particularly in the stomach region.

Toledo: NET impairement

beta 1 receptor auto anitbodies

Receptor hypersensitivity

NYMC: NET Impairement??

Low Flow POTS - impaired neuronal nitric oxide levels as a result of increased angiotensin II levels. reltaed to a genetic defect in angiotensin II catabolism. Low neuronal nitric oxide levels result in norepinephrine potentiation - MSNA overactivity from normal levels of norepinephrine.

Implicated - parasympathetic withdrawal.

'

There are two studies that suggest that Hyperadrenergic patients are just a different presentation of the same disorder. Differences in beta 2 receptor genes coudl result in clinical differences in the setting of the same fundamental problem. Mayo also recently suggested that hyper POTS were just a different presentation of the one major underlying disorder which they conclude is autoimmune in at least half pateints - including those with HYper.