Orthostatic Hypertension

[bjm0360]

Hello All,

I am wondering if anyone out there has Orthostatic Hypertension.

I have many many issues, but specifically I just saw Dr Barboi at NORTHSHORE Neurological Institute in Chicago, and he diagnosed me with Adrenergic Hypersensitivity.
He says that I have very low vagal tone and overactive sympathetic nervous system.

I think he was actually caught off guard that my blood pressure went up instead of sinking like a normal POTS patient.

Any input would be appreciated.

 

[Sushi]

I am wondering if anyone out there has Orthostatic Hypertension.

You have come to the right place --many, many of us have some form of OI, whether orthostatic hypertension, POTS, neurally mediated hypotension or some other form. If you do some searches or look at the threads in this section you will find a lot of information and experiences.

 

[Dmitri]

I have orthostatic hypertension that occurs intermittently, sometimes with a raise in heartbeat as well. It correlates strongly with abdominal burning pain. Was there a test for vagal tone and sympathetic activity?

 

[Countrygirl]

After about 30 years of typical OI with crashing BP and very narrow pulse pressure, it suddenly switched. The first changes were damaging bouts of malignant hypertension superimposed on the hyoptension and then rapidly developing to stage 3 hypertension which worsens when on my feet. It has now resulted in crescendo angina and encephalopathay symptoms which are remarkably similar to the ME brain symtoms

 

[halcyon]

Yes, I do have intermittent orthostatic hypertension and POTS.

I think he was actually caught off guard that my blood pressure went up instead of sinking like a normal POTS patient.

If you read the medical literature, strictly speaking, BP is not supposed to drop much in POTS patients. At least not right away. It should remain close to normal or increase:

Patients with POTS typically maintain (or even increase) their BP on standing.

POTS is defined as the presence of chronic symptoms of orthostatic intolerance (at least 6 months) accompanied by an increased HR ≥30 bpm within 10 minutes of assuming an upright posture and in the absence of orthostatic hypotension (a fall in BP >20/10 mmHg) 1.

The postural tachycardia syndrome (POTS) is characterized by a sustained heart rate increment of ≥30 beats/min within 10 min of standing or head-up tilt in the absence of orthostatic hypotension.

I found this passage in the recent David Kem autoimmune POTS paper interesting:

We have recently demonstrated an exaggerated catecholamine response in postural tachycardia among patients with syncope and dysautonomic cardiovascular response to orthostasis. The catecholamine surge may be seen as a compensatory mechanism to override the a1AR malfunction associated with the proposed autoimmune blockade as present in POTS patients...

My guess is that this is one plausible explanation for the increase in blood pressure in POTS patients.

 

[ryan31337]

Hi @bjm0360,

Yes I have the same issue, POTS with a hypertensive tendency, captured on beat-to-beat BP monitoring in tilt table and active stand tests. My POTS specialist also considers it unusual for us & is planning to send me to a hypertensionologist if standard investigations/treatments don't help.

I have orthostatic hypertension that occurs intermittently, sometimes with a raise in heartbeat as well. It correlates strongly with abdominal burning pain.

Have you been investigated for MCAS @Dmitri? One explanation offered for my hypertension is a mast cell reaction, recognised in the literature as causing hypertensive/tachy epsidodes in hyperadrenergic POTS patients.

Ryan

 

[bjm0360]

The Dr that diagnosed me with Adrenergic Hypersensitivity essentially offered me nothing as far as treatment options.

Northshore has an autonomic lab. I did the following tests:
Valsalva Maneuver ( failed stage 4 )
Sweet Test ( passed )
Tilt table ( blood pressure increased and blood pooled in my feet )

After that he said that essentially I have low vagal tone and an overactive sympathetic system. He offered me to try a med called Guanfacine and that was it. When I asked him what else there was to be done he said basically nothing. He said it could potentially clear up or not.

I really felt like he blew me off once he saw that I did not have classic POTS

 

[taniaaust1]

POTS is defined as the presence of chronic symptoms of orthostatic intolerance (at least 6 months) accompanied by an increased HR ≥30 bpm within 10 minutes of assuming an upright posture and in the absence of orthostatic hypotension (a fall in BP >20/10 mmHg) 1.

The postural tachycardia syndrome (POTS) is characterized by a sustained heart rate increment of ≥30 beats/min within 10 min of standing or head-up tilt in the absence of orthostatic hypotension.

@halcyon Originally it was thought with POTS that one had to have OH (orthostatic hypotension) too. They clearly are just defining it there to make it clear there that POTS isnt defined etc by having OH with it. In fact now if you have the OH with it as many do, you now get given two different diagnoses. One of POTS and one of orthostatic hypotension.
................

Lots of us here including myself have orthostatic hypertension. It's one of the 5 different kinds of dysautonomia that ME expert Dr David Bell says happens with ME. A ME person may have just one of these kinds to being like myself and having ALL of these so its quite possible to have both orthostatic hypertension and orthostatic hypotension (both when standing I can have 197/136 at one minute of standing or go to 40/0 (that's a lay NOW reading for me) with my BP readings. I never know what my BP and heart is going to do when I stand.

So be aware though orthostatic hypertension has been found for you, it is very possible you could also have other of the dysautonomia types too.

Im on clonidine for the orthostatic hypertension part of my POTS. Clonidine works differently to other high BP drugs as it works by helping keep the noradrenaline down which causes the BP spike in this kind of issue.

there has been a very good scientific article on this kind of POTS (if Im remembering correctly it was in some Indian Journal). Note this kind of POTS, when those who have this were studied, they found they were extremely likely to have a mast cell disorder (it was over 80% ..I cant remember how many now but it was extremely high, of those who have it have been found to have a mast cell disorder).

 

[taniaaust1]

I really felt like he blew me off once he saw that I did not have classic POTS

been there. I was refered to see a professor for mine at one of our main city hospitals who specialised in POTS. It turned out that this professor who specialised, didnt even know that one could get high BP with POTS and told me that couldnt happen. (she didnt get me tilt table tested or anything and just ignored another doctors BP readings on me when I stood).

 

[taniaaust1]

Here's something by Dr David Bell on the 5 types of dysautonomia in ME. Note it is old so the diagnostic criteria for POTS has changed very slightly since then

 

[Dmitri]

Hi @bjm0360,

Yes I have the same issue, POTS with a hypertensive tendency, captured on beat-to-beat BP monitoring in tilt table and active stand tests. My POTS specialist also considers it unusual for us & is planning to send me to a hypertensionologist if standard investigations/treatments don't help.


Have you been investigated for MCAS @Dmitri? One explanation offered for my hypertension is a mast cell reaction, recognised in the literature as causing hypertensive/tachy epsidodes in hyperadrenergic POTS patients.

Ryan

I am sure that I have some level of MCAS because of the obvious symptoms (incl. anaphylaxis) and because a family member has confirmed MCAS and POTS. I didn't get a chance to have it investigated deeply because my tryptase level was normal on two occasions. Histamine and prostaglandin tests are unavaliable where I live, which is unfortunate, since a "proven" MCAS diagnosis would help me a lot.

On a related note, I still don't understand how hyperadrenergic POTS is diagnosed. Does blood have to be taken from different positions, or is it a constant disbalance?

 

[taniaaust1]

On a related note, I still don't understand how hyperadrenergic POTS is diagnosed. Does blood have to be taken from different positions, or is it a constant disbalance?

I dont know if there is any set diagnostic criteria for hyperadrenergic POTS but it should be easy enough to tell if you have this form eg having orthostatic hypertension on top of having POTS would lead to this diagnoses.

I have abnormaly high adrenaline tests in blood tests. (had several so high that my test results from 2 different labs were dismissed by the labs as they must be errors.. with the labs not knowing that another lab were getting same kind of results and hence putting their results for me down as being errors too. In the end a lab accepted that an abnormally high result which on that occassion wasnt highly high, was a true result, makes me wonder just how badly high the dismissed results were.

 

[taniaaust1]

I am sure that I have some level of MCAS because of the obvious symptoms (incl. anaphylaxis) and because a family member has confirmed MCAS and POTS. I didn't get a chance to have it investigated deeply because my tryptase level was normal on two occasions.

same here, two results with normal trypase with mast cell disorder in my family. My uncle had to have his tryptase tested THREE times before he got his systemic mastocytosis diagnoses diagnosed (it took 10 years I think it was for his to be diagnosed though his had gone lifethreatening, his had gone so bad that he needed resusitation on multiple occassions). The third time the issue showed on his blood test.

So from that I know those blood tests are completely unreliable in some of us. My uncle says it shows up better (though still can be missed) from a bone marrow test. My uncles daughter has same problem, same symptoms as me and they believe she has mast cell disorder too but her trypase tests come back normal.

So from this we can know there is a lot of missed mast cell problems going on in those who have ME/CFS diagnoses. Maybe all of us with POTS and the orthostatic hypertension have mast cell disorder (either by itself or as a complication of also having ME)

 

[ryan31337]

I didn't get a chance to have it investigated deeply because my tryptase level was normal on two occasions. Histamine and prostaglandin tests are unavaliable where I live, which is unfortunate, since a "proven" MCAS diagnosis would help me a lot.

I'm about to go through this fun. Would they not consider speculative treatment? I'm hoping that's the case for me. Regardless I think some of the anti-histamines are available OTC so perhaps worth a short trial.

On a related note, I still don't understand how hyperadrenergic POTS is diagnosed. Does blood have to be taken from different positions, or is it a constant disbalance?

I don't think its something you'll get diagnosed clinically with unless you have a very academically-minded physician. I think many see the hyperadreneric state as as just one overlapping aspect of POTS (alongside neuropathy and hypovolemia), in some patients its just more significant than others. It puzzles me a bit because I'd expect the treatments for it should change dependent on primary issue but I guess there isn't enough evidence out there to guide that?

 

[sb4]

I found this passage in the recent David Kem autoimmune POTS paper interesting:
My guess is that this is one plausible explanation for the increase in blood pressure in POTS patients.

If this is the case surely pseudoephedrine would help. I have been hesitant to try as I have high sympathetic tone, but if Im right it would lower sympathetic tone...

 

[Dmitri]

I dont know if there is any set diagnostic criteria for hyperadrenergic POTS but it should be easy enough to tell if you have this form eg having orthostatic hypertension on top of having POTS would lead to this diagnoses.

I have abnormaly high adrenaline tests in blood tests. (had several so high that my test results from 2 different labs were dismissed by the labs as they must be errors.. with the labs not knowing that another lab were getting same kind of results and hence putting their results for me down as being errors too. In the end a lab accepted that an abnormally high result which on that occassion wasnt highly high, was a true result, makes me wonder just how badly high the dismissed results were.

I've had so many different blood tests that just come back normal, but I haven't had anything like a "comprehensive hormonal panel" yet. When I see an endocrine specialist, are there any other considerations I should keep in mind?

same here, two results with normal trypase with mast cell disorder in my family. My uncle had to have his tryptase tested THREE times before he got his systemic mastocytosis diagnoses diagnosed (it took 10 years I think it was for his to be diagnosed though his had gone lifethreatening, his had gone so bad that he needed resusitation on multiple occassions). The third time the issue showed on his blood test.

So from that I know those blood tests are completely unreliable in some of us. My uncle says it shows up better (though still can be missed) from a bone marrow test. My uncles daughter has same problem, same symptoms as me and they believe she has mast cell disorder too but her trypase tests come back normal.

So from this we can know there is a lot of missed mast cell problems going on in those who have ME/CFS diagnoses. Maybe all of us with POTS and the orthostatic hypertension have mast cell disorder (either by itself or as a complication of also having ME)

In my mother's case, the tryptase was raised after a severe drug reaction. The regular day-to-day leakage that MCAS patients experience seems to release mediators other than tryptase, unlike mastocytosis which I believe is associated with constantly raised tryptase.

According to Dr. Lawrence Afrin, endoscopy may be of some diagnostic utility for MCAS. Here is a quote from him I found on another forum:

Although marrow is almost always "clean" in MCAS, I have enjoyed a roughly 90%
"hit rate" on finding aberrant mast cells (either increased quantities and/or
abnormal immunophenotypes and/or genotypes) on random ("blind") biopsies taken
throughout the GI tract. In my patients who want "tissue proof," or for those
patients who will be challenged by their other physicians who won't "believe"
they have MCAS until "tissue proof" is demonstrated, I don't even bother with
marrow biopsies any more and instead just send them to the gastroenterologist
for upper and lower endoscopy. I had to persuade him the first time, but the
gastroenterologist now knows to take multiple biopsies all up and down the
tract. Similarly, I had to persuade him the first time, but the pathologist now
knows to perform special staining (typically at least CD117, sometimes also
Giemsa, tryptase, toluidine blue, CD25, and/or CD2) on *all* the biopsies. The
disease has a patchy and sparse distribution, after all, and the
gastroenterologist is "blindly" taking biopsies of tissues which grossly (to his
eye, through the endoscope) appear completely normal. In fact, without the
special staining, under the microscope the tissue will almost always appear to
the pathologist, too, as either completely normal or, at most, mildly
chronically inflamed. Yet, if the biopsies are taken in a patient who not only
has a history consistent with MCAS but also has at least one elevated mast cell
mediator level, then in at about 90% of these patients you can find at least one
GI biopsy that shows abnormal mast cells -- and I would be willing to bet
another 9% or so would be found to have it if only a few more biopsies had been
taken. Once an abnormal biopsy is found, the pathologist knows to send it for
"PCR" (polymerase chain reaction) analysis for the KIT-D816V mutation that marks
for systemic mastocytosis, but that's almost always negative in MCAS, consistent
with Dr. Molderings' recent findings on the genetics of MCAS. I continue to
hope that we will gain within the near future the ability to (cheaply,
routinely, commercially) analyze for all the mutations Dr. Molderings has found.

http://mastcelldisorders.wallack.us/yabb/YaBB.pl?num=1309376308

 

[Dmitri]

I'm about to go through this fun. Would they not consider speculative treatment? I'm hoping that's the case for me. Regardless I think some of the anti-histamines are available OTC so perhaps worth a short trial.


I don't think its something you'll get diagnosed clinically with unless you have a very academically-minded physician. I think many see the hyperadreneric state as as just one overlapping aspect of POTS (alongside neuropathy and hypovolemia), in some patients its just more significant than others. It puzzles me a bit because I'd expect the treatments for it should change dependent on primary issue but I guess there isn't enough evidence out there to guide that?

I tried ketotifen and other antihistamines and all they did was increase my inflammation. There's also cromolyn, which I didn't get a chance to try because of how expensive and difficult to obtain it is. More advanced options like xolair are even more difficult to come by. Mast cells release hundreds of chemicals other than histamine, and the antihistamines just block specific receptors, which may just cause the histamine to bind to other locations in the body with other, unblocked receptors. But many report success with H1 and H2 blockers, so it's definitely worth trying. Maybe I simply can't tolerate any antihistamines and all they do is cause even MORE histamine to be released, or my mast cells are releasing mediators other than histamine.

Even without potential treatment, a MCAS diagnosis would be useful as a quick explanation for drug intolerances and environmental sensitivities.

 

[ryan31337]

Sorry to hear that you've not had any luck with the common treatments @Dmitri. It's a very active field so hopefully they will have better tests for you soon.

 

[Jmsparky]

Hello,

I am new to this site and desperately looking for answers, my name is Jim and I am 44 years old. I do not really have a diagnosis yet but can tell you the millions of things I dont have. I feel like a laboratory test monkey for as many Dr's that I have seen. I started having chest pain in 2012 that would occur daily, I must have went to the ER no less than 20 times over the past 4 years and each time they found nothing that was wrong but yet I was having chest pain and felt an overall yuck feeling on a regular basis.

In 2013 the ER Dr found that when I stood up my Bp would go from 118/70 to 150/100, so I was seen at the University of Iowa for autonomic testing and told that there was nothing wrong with me and that I have hypertension. I was started on different meds over the years and ended up on lisinopril. The problem is that I do not need Bp control when I am sitting or lying down so my pressure drops and it becomes a crazy balancing act. I have been all over the internet for the past few years and this is the first site that talks about orthostatic hypertension. If anyone has any ideas please let me know. I am currently not taking any meds and my upright bp is high until i sit or lay down.

 

[NelliePledge]

Here's something by Dr David Bell on the 5 types of dysautonomia in ME. Note it is old so the diagnostic criteria for POTS has changed very slightly since then

hi @taniaaust1 sorry if Im being slow but I couldnt see the link you were referring to here from Dr David Bell any chance you could post it again
NPx