Pulmonary Hypertension Treatment Explained – Guidelines

By Adem Lewis / in , , , , , , , , , , , , , , , , , , , /

well welcome to another MedCram
lecture we’re going to talk about pulmonary hypertension treatment and
there’s actually several different types of treatments remember there are
different waho groups there’s Group 1 2 3 4 & 5
now group 1 we said was idiopathic pulmonary arterial or hypertension and
the other ones for instance the collagen vascular diseases the drugs the toxins
those sorts of things number one is where most of the work in terms of drugs
specifically for pulmonary hypertension have centered there’s also one that is
FDA approved for four which we’ll talk about
but remember why this is is because group two is almost exclusively is
exclusively due to left ventricular failure Group three has to do with lung
disease and group five is kind of a grab-bag so these already have their
treatments specifically for them one has a whole bunch which we’re going to talk
about and there is one that is indicated for for remember for is for venous
thromboembolism or chronic pulmonary hypertension or as it’s officially
called chronic thromboembolic pulmonary hypertension and that’s abbreviated c t
e p h okay so let’s talk about number one and the different types of
treatments okay so there’s some things that all
groups would do well to start and we’re going to make this as simple as possible
and explain it as clearly as possible as we normally do so because of the fact
that they get this lower extremity diuretics is something that you want to
do the other thing that you want to do if they need it is oxygen therapy and
they’ve noticed on autopsies that there are a lot of blood clots not just in
group chronic thromboembolic pulmonary but also in the other groups after many
studies anticoagulation was found to be definitely something that you want to do
in group 4 and maybe in group number one other medications that might be
beneficial is digoxin and exercise definitely beneficial so these are ones
that can go really to just about all classes let’s talk about group one now
and the different classes now before we start with the advanced medications that
some of you are familiar with we have to do something called a vaso reactivity
test and the reason why we do this is because those who respond to the vaso
reactivity test are more likely to respond to ordinary medications like
calcium channel blockers like the dihydropyridine and diltiazem calcium
channel blockers so if they respond if that’s a positive response calcium
channel blockers if it’s negative then we go on down to the more advanced
medications so what is the Razr reactivity test looking at well there’s
a number of ways you can do it you can use nitric oxide to see whether or not
the patient’s mean pulmonary artery pressure drops by 10 millimeters of
mercury the other thing that you can do is you can use equal pross on all finally the other thing that you can use
is adenosine so all of these are medications that can be used to see
whether or not the patient is reactive and once again it’s considered positive
if the main pulmonary artery pressure decreases by at least 10 millimeters of
mercury and goes to less than 40 millimeters of mercury this is assuming
that the cardiac output actually gets better or it’s unchanged and as we
mentioned patients with a positive reactivity tests are ones that could
improve with calcium channel blockers those that are neg
will not respond to calcium channel blockers so once we do this and I’ll
tell you it’s a very small percent are actually reactive most of these are
going to go into the negative category and so most of them are going to be
having to be put on advanced medications if we’re dealing with a group one so
let’s talk about those things but before we do the thing that’s going to
determine what medication they get is to determine how sick they really are and
that’s determined by a w-h-o again functional class and so there’s
functional class 1 which is the most mild and they typically don’t need
medications they can be monitored and then there’s class
– and there’s class 3 and these are where most of the medications are
actually started and then there’s class 4 which is the most severe and these are
usually given IV so the way I remember it is that class IV should be given IV
class 1 doesn’t need to be giving any medications orally and the ones in the
middle are given Pio meds and those Pio meds we’ll talk about very shortly most
of these medications haven’t been around for more than 15 years that’s how new
they are and if you’re lucky enough to have a patient who is responsive to a
phaser reactive medication then you can just give a calcium channel blocker
which is dirt cheap and has been around for many years if on the other hand they
don’t respond and they’re a w-h-o class 2 or 3 then you’re going to be starting
them on some Pio meds which we’re going to talk about right now the first
category is the prostacyclin agonists so this is like equal process on all IV
which is indicated for stage 4 IV as we talked about there’s tree procced
Annelle which can come IV sub-q or inhaled there’s Isla Pross which has
comes inhaled and then there’s these prostacyclin agonists which are not
really prostacyclin like celexa peg which all of these things stimulate the
prostacyclin receptor and in effect caused an increase in cyclic am P and
therefore vaso dilation okay so those are the prostacyclin
agonists the next group are the endo feelin receptor antagonists so endo
feelin one is a hormone that basically circulates around and is extremely
potent vasoconstrictor so if we could possibly block these receptors
potentially we could get some vasodilation that’s exactly what we see
there’s two types of receptors is the a and B and so both senton and massive
1010 is basically a drug that blocks these receptors it’s non-selective and
it seems to reduce the PA pressures it also like a lot of the other medications
improves the quality of life extends the length of time before decompensation and
increases exercise capacity one of the selective ones is amber senton so these
are the receptor field and receptor antagonists these are a list of
medications that are fda-approved for pulmonary hypertension specifically
sildenafil and Tel dalla fill the purpose of these things is to inhibit
the breakdown of these medications which basically increase the amount of nitric
oxide so there is cyclic GMP and that cyclic one of the monophosphate is
broken down by phosphodiesterase well these medications inhibit the ability of
this phosphodiesterase to break down the cmp and so these are why they’re called
pde5 inhibitors and so what happens is cyclic GMP goes up which stimulates an
increase in nitric oxide and it’s nitric oxide which is a vasodilator okay so
there’s a couple of other things that also increase vasodilation and that
would be like an alpha blocker or a nitrate so you should not be on these
medications at the same time these PB v inhibitors cause vasodilation in the
pulmonary vasculature by increasing nitric oxide and they do it specifically
by inhibiting the breakdown of cyclic GMP
the last mechanism that we’re going to look at is the guanylate cyclase direct
stimulants and this riociguat is one of the medications that is fda-approved is
the medication that is FDA approved to do this and it’s a direct stimulator of
the nitric oxide receptor so it increases nitric oxide just like the
phosphodiesterase inhibitors do but they do it in a different way and they have a
dual mode of action not only do they increase the nitric oxide receptor they
also increase the sensitivity of the SGC to endogenous nitric oxide which is a
pulmonary vasodilator and so they also directly stimulate the receptor to mimic
the action of nitric oxide so this is a little different and not only is it
approved in a pH group w-h-o group number one but it’s also approved for
waho group number four which remember is the chronic thromboembolic disease so
just be aware of that now all of the medications that we’ve
just talked about can be used in combination but you’ve got to be careful
make sure again that you are using these in combination after you have done a
right heart cath and made sure that they are not reactive so you could add for
instance tadalafil and amber senton you could use sildenafil and both senton you
could use both senton added to either Ipoh process and all or triple process
Adele or you could do triple Prasanna added to either both sent an or
sildenafil so there’s different ways of doing this and this is very similar to
how we increase blood pressure medication for systemic hypertension we
can also do this for pulmonary hypertension so the key points again are
that if you suspect somebody of having pulmonary hypertension make sure that
you classify them in a w-h-o group if they are Group 1 or 4 there may be
medication specifically designed for these types of diseases if it is group 2
you need to look the underlying cause of the left heart
disease and treat that if it is three you have to look at the underlying cause
for the hypoxemia and treat that whether it’s lung disease or sleep apnea for you
need to anticoagulate them generally and they may need to go to surgery where
they actually have the clot removed or if that’s not feasible they may have to
consider a medication like we just talked about here and then five is kind
of the grab bag it should be looked at in terms of the other causes so that is
a very brief primer in pulmonary hypertension how it is diagnosed how it
is looked at how it is treated and and what are the medications that are
involved so thanks for joining us

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