i'm dr david weissman and today we're going to talk about something that's i'm very passionate
about which is called dysautonomia and pots postural orthostatic tachycardia syndrome
and i titled this talk the first key to understanding is awareness i'm a cardiologist and i've
sub-specialized in cardiac electrophysiology which makes me a electrical heart doctor a heart rhythm
doctor and you know i want to i want to kind of
start off this talk um with a little story and you know like
many other doctors we didn't really get an education on
dysautonomia or pots in medical school at least i didn't and i remember right after i got out of my training being on call on the weekend
and seeing a a 21 year old girl in the icu who had just undergone brain surgery to
remove a meningioma and meningiomas for for those of you uh that don't know is a
benign brain tumor most of the time does not need to be removed and i'd gone
to sign out on this patient and i'd gone to see her you know was not intending for you know
me to add anything uh all that relevant and the reason as cardiologist we were seeing this
patient is she had a fast heart rate so in talking to her i realized
by digging in the in the depths of my brain that she had had pots and i remember the one line that i had
read in my text as a medical student about what pots was and so i i had her
she was laying down in the bed her heart rate was you know 70 or so
which is normal and then i had her stand up and she went up to about 170 and she quickly became symptomatic and i said to her is this why you had the brain surgery
and she said yes they couldn't figure out why her heart
rate was going so fast they attributed
it to her meningioma which was benign
and didn't need to be removed
and after this i began realizing and
seeing more and more patients over the
years with dysonomia in pots and i don't
know if it's becoming more prevalent
i've just become more aware and have
just learned a lot more but
i think it's very important it's very
under-appreciated and not really well
understood by the medical profession in
general
so today we're going to talk about
dysautonomia in pots
What is POTS
and to give you a little background
about where this comes from
there's a real failure of the medical
system to diagnose and appreciate
the severity of symptoms clinical
symptoms are supported
by biological mechanisms they are real
but yet they're still not very well
completely understood and there's a lot
of research that's being undergone
people who suffer from dysautonomia and
pots are often embarrassed
feel socially marginalized and made to
feel psychologically very vulnerable
and the worst part of it is time to a
diagnosis
often can be years patients will go
doctor to doctor
ologists to ologists and many times are
more likely to
be referred to a psychiatrist than a
medical doctor
for a real medical issue
Heart Rhythm Society Guidelines
so here we have the hrs guidelines the
heart rhythm society guidelines
the last guidelines on pots was written
in 2015
and the first thing i want to highlight
here that they state
anxiety and somatic vigilance are noted
to be higher in pots
why do i think that's important where i
think here's where the crux of the
problem is
you know when a patient comes to a
doctor and they have a diagnosis of
pots or they have a diagnosis of
dysautonomia and there or they're
suspected of having that they're
automatically labeled as being
anxious or hyper vigilant but in the
next quote you'll see in this same
guideline
that detailed physiologic and
psychometric studies have
shown that although anxiety is commonly
present in patients with pots
the heart rate responds to orthostatic
stress meaning from
a laying to a standing position is not
is not caused by anxiety but is instead
a response to an
underlying physiologic abnormality and i
think this is really important we're
going to go into some more detail here
in a second
POTS Treatment
so here we have the dysautonomia
guidelines
to my knowledge no guidelines have ever
been written
about dysautonomia so let's talk about
pots
in doing my research i came across a
review article from the american college
of cardiology
titled non-pharmacological treatment is
the mainstay of therapy for pots
and they go on to list a number of
different typical treatments that a
patient
might be prescribed starting with
exercise conditioning
exercise conditioning with a recumbent
bike a rowing machine or
swimming allows patients to exercise
while
avoiding an upright posture
theoretically i think this is
excellent i think initially most
patients this is really not
a feasible a regimen they come too
symptomatic unless their symptoms are
more on the mild side
and so initially an exercise program
might not be suitable for them
medications medications for severe
symptoms
medications like propranolol, midodrine,
pyridostigmine, and fluorocortisone
POTS is a syndrome of orthostatic intolerance characterized by a heart rate increment of ≥30 bpm, often with standing heart rates >120 bpm, within 10 minutes of standing or head-up tilt, and in the absence of orthostatic hypotension (a decrease in systolic blood pressure [BP] of ≥20 mm Hg and/or decrease in diastolic BP of ≥10 mm Hg).
It is the most common form of orthostatic intolerance in young people (predominantly premenopausal women). Presyncope is much more common than syncope in POTS, but it is not infrequent that POTS coexists with episodes of neurally mediated (reflex) syncope.
Cardiovascular deconditioning is a universal feature in all POTS. Overlapping pathophysiological variants that may contribute to an individual’s susceptibility to develop POTS include peripheral autonomic neuropathy, excessive venous pooling, hypovolemia in the setting of volume dysregulation, hyperadrenergic states, mast cell activation disorders, and autoimmunity.
Chronic symptoms and comorbidities that cannot physiologically be explained by orthostatic intolerance or tachycardia, but are common in patients with POTS include chronic fatigue, dizziness, syncope, migraines, functional gastrointestinal disorders, chronic nausea, fibromyalgia, and joint hypermobility.
POTS should be differentiated from neurogenic orthostatic hypotension (NOH), which can occur in disorders such as multiple system atrophy, Parkinson disease, Lewy body dementia, pure autonomic failure, autoimmune autonomic ganglionopathy, and other autonomic neuropathies. With orthostatic hypotension, there should be at least a 20-point drop in systolic BP by 3 minutes of tilt and typically the heart rate increment is minimal.
Inappropriate sinus tachycardia (IST) is sometimes confused with POTS, especially because both occur in young women, but IST occurs independent of body position. The key to making a diagnosis of IST is ambulatory monitoring; IST patients, unlike patients with POTS, demonstrate relative night-time supine tachycardia.
Formal autonomic function testing is helpful to evaluate for other types of autonomic impairment and differentiate among POTS subtypes.
Nonpharmacological treatment is the mainstay therapy for POTS and includes:
Exercise conditioning with a recumbent bike, rowing machine, or swimming. This approach allows patients to exercise while avoiding the upright position and improves tolerance of the program. (Medications can be considered in patients with severe symptoms as a bridge to help minimize some symptoms and allow patients to initiate the exercise program. Such medications include propranolol, midodrine, pyridostigmine, fluodrocortisone.)
Increasing blood volume can be accomplished by drinking 3 liters of water per day and liberalizing salt intake by ingesting 5-10 g of sodium per day.
Avoidance of large and heavy meals, alcohol, and heat exposure.
Wearing compression stockings up to the top of the thighs or higher and abdominal binders and they must extend at least to the top of the thighs and preferably to the abdomen.
Sleeping with head of bed elevated and performance of physical counter maneuvers such as leg crossing and squatting.
Behavioral and cognitive therapy may be used to obtain long-term control of symptoms, particularly when anxiety, hypervigilance, or catastrophizing behaviors are present.
these are medicines which have been around
really for many many years prescribed for other reasons used to help support patients with
severe symptoms and pots increasing blood volume and this can be
accomplished by drinking liberalizing salt intake from 5 to 10
grams per day of sodium
avoiding large and heavy metals and then
as well
avoiding alcohol intake and heat
exposure
heat can cause excessive sweating and
obviously volume loss and that might
predispose someone
for to cause their heart rate to speed
up
uh wearing compression stockings either
thigh high or abdominal binders
i would say this is one of the most
difficult things that i cannot get any
patient to do
they're very uncomfortable for some
patients very difficult to get on
and so in general i fiber i find very
low compliance
uh with these kinds of stockings and
abdominal binders
sleeping with the head of the bed
elevated and performing physical counter
maneuvers such as
leg crossing or squatting can minimize
symptoms
so the last prescribed therapy in this
review article was behavioral and
cognitive therapy for
anxiety hyper vigilance and
catastrophizing behaviors when they're
present
and when i read this i actually paused
and was
embarrassed that the medical profession
would put this in
a prescribed treatment plan for patients
with dysautonomia and pots
as a patient i would be personally
insulted to know that my doctor
felt that way about me a patient with
diabetes
high blood pressure coronary disease is
not treated in that way
we don't label them as being anxious or
hyper vigilant we treat their underlying
condition and we respect them as such
Dysautonomia
so let's get into a little bit of a
discussion about dysautonomia
so dysautonomia is a large word really
that discusses the
disease of the autonomic nervous system
the autonomic nervous system has two
parts
there's a sympathetic and
parasympathetic nervous system
the essential thing that the autonomic
nervous system does is that it maintains
a homeostasis and equilibrium it is the
unconscious
regulation of various body functions in
the body
which is involuntary opposed to our
somatic nervous system which is
completely voluntary the sympathetic
nervous system neurons are located in
the brain stem and spinal cord
while the parasympathetic nervous system
is located near the target organ system
and here we have a large kind of
complicated chart
and on one side we have the sympathetic
on the other side we have the
parasympathetic and we can really see
the interplay with how these
systems coexist with each other in our
bodies in our in our eyes for example
pupil dilation and pupil constriction
are regulated
our breathing our heart rate everything
is regulated
by the sympathetic and parasympathetic
nervous system we often think of it
as fight or flight eat or rest so
you know if you're if you're resting the
parasympathetic nervous system
in general slows our heart rate down
slows our breathing down
when you're getting ready to exercise or
or if you're scared or frightful
the heart rate will speed up the
breathing will will start to go a little
bit faster
and this is kind of the yin and the yang
of the autonomic nervous system
Overview
so an overview a dysfunction of the
autonomic nervous system or dysautonomia
can either be under active or overactive
and this can involve either limb of the
of the autonomic nervous system it can
be the sympathetic or the
parasympathetic
and we can further divide these into
primary and secondary disorders
primary disorders include conditions
like primary dysautonomia
multiple system atrophy and familial
dysautonomia
secondary disorders are much more common
we see them in conditions like diabetes
parkinson's rheumatoid arthritis lupus
sjogren's disease sarcoidosis
inflammatory bowel disease crohn's and
ulcerative colitis
celiac disease chiari malformation
amyloidosis
guillain-barre airlos danlos syndrome
lamborghini and hiv in line
for the most part the diagnosis of
dysautonomia is a clinical one
there are a lot of fancy tests that one
can do on the autonomic nervous system
but at the end of the day
i have not really found them to be all
that helpful and management really is
a supportive one as no cure is currently
available
Clinical Symptoms
so what are the clinical symptoms if we
think back to that chart that i showed
earlier about
how the sympathetic and parasympathetic
nervous systems are at play
you can start to think about what kind
of symptoms a patient might have
so imbalance dizziness
fainting symptoms or feel feelings of
near faintness
palpitations or feeling like your heart
is racing sweating which is due to
an imbalance of thermal regulation cold
and heat intolerance
nausea vomiting diarrhea some of these
patients will have something called
gastroparesis
forgetfulness and brain fog shortness of
breath
especially with exercise they have
exercise intolerance
chest pain fatigue insomnia
migraine headaches and sensitivity to
light noise
and looking at this list you can see
mostly symptoms are very vague and
common
you may even experience one or two of
these but the patients with dysautonomia
in pots frequently have a constellation
of these and offers suffer from many of
them
POTS
so let's talk a little bit about pots or
postural or
orthostatic tachycardia syndrome as it's
mostly called
it really is exists on the spectrum with
dysautonomia
it is not truly a separate entity it is
defined by
orthostatic intolerance meaning when a
patient goes from a laying to a standing
position
and is characterized by a heart rate
increase of 30
beats per minute or more with standing
within 10 minutes of standing
in the absence of orthostatic
hypotension or drop in
blood pressure where the systolic or the
upper number doesn't drop by 20 or the
lower number doesn't drop by 10
because in in situations where the blood
pressure drops
that might be another reason why the
heart rate is speeding up
most patients tend to be premenopausal
females
more often dizziness is the primary
complaint although
fainting can occur although it's a
little bit less common
now a distinct entity from pots is ist
or
inappropriate sinus tachycardia and this
is not related to position
cardiovascular deconditioning is common
as i said before
a lot of patients experience fatigue
inability to exercise and shortness of
breath with exercise
Cardiovascular Deconditioning
pots can frequently coexist with
neurocardiogenic
syncope a lot of these patients will
have you know as we
end the layman term vasovagal syncope as
well as other autonomic disorders
triggers can include dehydration
prolonged standing
drinking alcohol or caffeine as well as
stress and excessive heat
treatment really is multifactorial the
number one thing and we always stress
is avoiding dehydration and this
includes avoiding alcohol and caffeine
alcohol and caffeine will cause us to
urinate more and therefore depleting our
body of our volume
and make you more predisposed to
symptoms liberalizing salt and water
intake
and this is something that i've only
really appreciated in in the recent past
but salt supplementation as much as
possible can really
ameliorate symptoms very nicely without
any other prescribed medications
and we like to target at least three
grams a day really if not more
exercise one symptoms are under control
a
rigorous exercise program to build up
the skeletal muscles really
does help and i think a physical
therapist with the knowledge of these
conditions can really be helpful in this
situation
compression stockings and abdominal
binders although can work really well
again
i i find in general very low compliance
with this therapy and then medical
Medical Therapy
therapies and we're going to go
a little bit into medical therapies so
the medical therapies for dysautonomia
and pots really overlap and really
depend on their symptomatology
the mainstay of therapy are beta
blockers and beta blockers are
cardiac medicines heart medicines that
we've had for many years
and their primary reason that they're
prescribed in this situation is to
reduce the heart rate
the downside of beta blockers is that
they also can reduce
blood pressure and lower blood pressure
and patients sometimes who have
dysautonomia may have low resting blood
pressure and
so that may predispose them to fainting
common medicines that are beta blockers
are
propanolol or endurol metoprolol and
atenolol
next we have the calcium channel
blockers which is another class of
medicines again
medicines which have been around for
many years and similar to the beta
blockers they can also
reduce and lower heart rate and blood
pressure and and similar to beta
blockers
although they have the favorable effect
of slowing the heart rate down they can
also
lower their blood pressure and that can
sometimes make for intolerance to these
medications common medicines like this
can include medicines like varapamil or
deltaism
the next medicine is something called
midodrin and this is an old medicine
that
is given and it is there to increase
blood pressure so patients who have
hypotension or low blood pressure who
are prone to dizziness
and near fainting or may actually faint
we may prescribe mitogen
it's a very good medicine one of the
downsides to menodrin is that it's
really prescribed three times a day due
to its short half-life
the next medicine is fluid cortisone or
fluorine f
similar to menodrin it also can increase
blood pressure for the same reasons
and can be very effective the newest
medicine um
in this class is called northern or
droxy dopa
and this can be a very potent uh
medicine to increase blood pressure and
is
for the most part we use in patients who
have failed either mitogen
or your cortisone
it's an excellent medicine that can
really increase their blood pressure
and we'll go into a little bit more
detail in a few minutes
the next medicine on this list is called
the vabradine or coralinor
and this is also a relatively new drug
in the united states
and it has its only effect
is to slow down the heart rate so unlike
the beta blockers and calcium channel
blockers
there's no effect on blood pressure so
this makes it an ideal
therapy for pots patients other
therapies that i've seen
that are out there are mestanon
ssris which are kind of antidepressant
and anxiety medicines
and pacemakers i've had patients come to
me with pacemakers
but for the most part these are really
rarely indicated and uh
not really ever helpful um in general
Goals of Treatment
so what are the goals of treatment when
i see a patient with dysonomia pots
so the first thing is to identify what
symptoms they're suffering from
and how that is impairing their quality
of life and then
how can we then focus our therapy to
limit
side effects of therapy and limit their
overall symptomatology
most patients i feel would benefit from
a digital blood pressure monitor and
this is
uh fairly inexpensive it can be gotten
in any
retail pharmacy or even a supermarket
i think frequent orthostatic vitals
especially
during symptomatic episodes is very
helpful to log
to share with your doctor so that they
can help guide and tailor your therapy
i think frequent visits initially
especially upon diagnosis
and treatment is very helpful rather
than spacing them out as time elapses
and patients become less symptomatic
less visits are usually needed
again pacemakers are really rarely ever
indicated for these patients
and then sinus node modifications and
ablations are also rarely indicated i've
gotten
a number of different referrals to
ablate or burn the sinus node which is
the heart's natural pacemaker to slow it
down
these these procedures are often
not very easy to accomplish and not
successful and for the most part
symptoms can be controlled with
more simpler measures so i want to then
Corlinor
focus on
this new drug core lenore corollanor or
if aberdeen
has been available actually in europe
for a number of years and only recently
became
available in the united states it's an
amazing drug
it's really a great drug for patients
who have
pots or suffer with pots again it's
specific to the heart rate it slows the
heart rate down and doesn't affect the
blood pressure so this really makes it
an ideal therapy for those patients
it affects something called the funny
sodium channel in the heart it's
actually called the funny channel
believe it or not and so this drug
actually blocks that channel inside the
heart and that's how it has its effect
the only real issue with coraline or
that that we find is that
it's very expensive in the united states
the only indication for prescribing
corlinor is actually for heart failure
there is no indication for pots
it's not a commonly prescribed medicine
for heart failure
and so a lot of patients find other
means of obtaining this drug
frequently from overseas and mail order
Nithera
i want to shift gears and talk a little
bit about north thera
or droxy dopa and this is the
counterpoint to corollary this is a drug
that affects blood pressure it's a
potent medicine which can really uh
raise someone's blood pressure who might
feel like they're fainting or nearly
fainting
and has no real effect on heart rate um
again the biggest issue with this drug
is that we need to monitor
supine blood pressure so obviously we
want the blood pressure to
go up when we're standing but when
someone is sleeping at night
we don't want their blood pressure to be
that high and in in drugs like north
thera
that blood pressure can actually go up
quite a bit so so we want to take
vital signs at home when the patient is
sleeping to see what that actually is
and then we can adjust their therapy as
needed
the limiting factor with nithera similar
to coralinor
is cost and as i mentioned the supine
hypertension unlike the core lenoir
i've had very good success in getting
this drug approved
uh you know through prior authorizations
and peer-to-peer reviews through
insurance companies
but it can be a challenge in some
patients additionally many insurances
will require
tilt table testing to make a concrete
diagnosis of their
dysautonomia before doing a prior
authorization
some patients with dysautonomia can also
have baseline hypertension
can have high blood pressure and that
can make treatment of their hypotension
their low blood pressure really
difficult so
they can have high blood pressure at one
minute and the next minute
it can be really low so the typical
patient could have a systolic pressure
of 150 160 and the next
minute have a systolic pressure of 80
and then faint
and so we we really have to kind of
weigh the um
the advantages and disadvantages of
these kinds of medicines when we
prescribe them so that we don't
cause their hypertension to shoot up and
cause other problems
Common Conditions
so what are common conditions uh
that of patients who have dysautonomia
so i mentioned
a long list earlier and i'm just going
to focus in on a few of them the ones
that i really see a lot
so diabetes so diabetic patients can
commonly have dysautonomia
especially patients who have long
standing
uncontrolled diabetes these are the ones
with high hemoglobin a1cs
and or maybe they didn't treat their
diabetes early on
in addition to supportive care and
treatment of the their dysautonomia
treatment of their underlying diabetes
is really critical
parkinson's disease so parkinson's
parkinson's disease is very
underappreciated i i've gotten a number
of referrals over the years
to put pacemakers or to do ablations for
arrhythmias that these patients didn't
have and patients in particular who have
advanced parkinsonism can have
dysautonomia and have very labile
very erratic blood pressures and and
faint
so focusing on that treatment can really
make their quality of life
really much better amyloidosis so this
is
a genetic condition that can affect many
organ systems in the body
including the brain the heart and the
autonomic nervous system
it's an abnormal protein deposition
which causes this
issue the only there are many different
subtypes of amyloidosis
currently the hattr subtype
is the only one actually recently that
has a treatment available for those
patients
Heirloom Stainless Syndrome
so i want to focus in a little bit about
a condition called
heirloom stainless syndrome so heirloom
stainless syndrome
is a genetic disorder with a autosomal
dominant
inheritance autosomal dominant means
that if you inherit
one copy of the gene so usually we get
one copy of the gene from our mother one
copy the gene from our father
if you inherit one of the genes you'll
have the disease now patients with aeros
dandlows or eds as we also like to call
it
can have variable expressions so
although let's say a mother may have
eds the patient may have eds but to a
different severity maybe more mild or
more severe depending on that patient
currently we know that there are 13
different subtypes although
the most common type is hypermobility
which is type 3
and unfortunately type 3 has no
identifiable gene
that is currently available on the
market there's a significant female
predilection but
males certainly can have eds
Clinical Features
so what are the common clinical features
that we see in patients with
erlos download syndrome so
the first thing that we see most
commonly is joint pain and hypermobility
these patients this this is what really
impairs our quality of life
they have pain all over in many
different joints
they have hypermobility and are prone to
subluxation and actually dislocation of
their joints
they suffer with imbalance dizziness
fainting palpitations and and
tachycardia or fast heart rate
sweating cold and heat intolerance
nausea vomiting
diarrhea diarrhea and in particular a
lot of them can have gastroparesis
forgetfulness and brain fog shortness of
breath or exercise intolerance
chest pains fatigue insomnia
migraines and insensitivity to light in
noise
in addition they can also have things
like mast cell disorders
resistant to anesthetic drugs atrophic
scarring in the skin
and soft velvety skin and on this slide
you can see that i've highlighted a lot
of these symptoms
and i bring this to your attention
because if you refer back to the
beginning when we talked about symptoms
related to the autonomic nervous system
this is identical
patients with eds almost universally
will have dysautonomia at some point
and we have to make sure that we
identify it and treat it
so that we can improve their quality of
life
Take Home Message
so what is the take home message i think
the take home message is
that the management of dysautonomia and
pots requires a very systematic approach
many patients actually feel reassured
simply by giving a name to their disease
which was previously invisible to the
medical world
while currently there is no cure there's
a lot of research being done
there's a lot of really good supportive
management and therapies which can
improve the quality of life
for many patients i
so i have two offices one is in palm
beach gardens on burns road here across
from the hospital
the other one is in west palm beach
across some good samaritan
you can also find me at
drdavidweissman.com
i'm currently offering telehealth and
virtual visits
and we're excited to see you and talk
about your condition
if you enjoyed this video i have other
videos and talks that you could
watch to learn more about other
conditions thank you
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