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 * Patient Site

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 * Treatment Guidelines
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 * Resources
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 * Overview
   * Pathophysiology
   * The Right Heart in PAH
   * Diagnosing PAH
 * Risk Assessment
   * Prognosis
   * Risk Score Calculator
 * Treatment Guidelines
 * Treatment Pathways
   * Prostacyclin-Class Therapy
 * Resources
   * PAH Curriculum
   * ICD-10 Codes
   * Published Studies in PAH




PAH RISK SCORE CALCULATORS

Home / PAH Risk Assessment / PAH Risk Score Calculators

 * Aim for low-risk status
 * 30-second risk calculation
 * Discuss risk with your patients


AIM FOR LOW RISK TO HELP IMPROVE YOUR PATIENT’S CHANCE OF SURVIVAL1-4

According to PAH treatment guidelines, each patient should receive an objective,
multiparameter risk assessment at diagnosis, and as often as every 3 months
thereafter.5

These online risk calculators can help you quickly calculate your patient’s risk
score with point-and-click ease. You can choose from among 5
calculators—including the 4-strata COMPERA 2 method introduced in the 2022
ESC/ERS guidelines for follow-up risk assessment—depending on which works best
for your practice.

Use these online tools to calculate your patient’s risk status and determine
whether treatment escalation is needed to reach low risk.


2022 ESC/ERS GUIDELINES

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REVEAL 2.0

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FRENCH NONINVASIVE CRITERIA

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COMPERA 2 4‑RISK STRATA

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REVEAL LITE 2

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INSTRUCTIONS ON HOW TO USE

Download Instructions (PDF)


CAN RISK CALCULATION BE DONE IN LESS THAN 30 SECONDS?

The value of formal risk calculation is well established, but some clinicians
are concerned about the amount of time it might take to complete a risk
assessment. Online risk calculators can help expedite the process. In this
podcast, Dr. Raymond Benza notes that tools available today make it possible to
rapidly perform a formal risk assessment.


HEAR FROM AN EXPERT

“We’ve made a lot of headway in some of the EMRs in programming them to
calculate risks on the fly. So I think the nature of the calculation is getting
easier and easier for practitioners to perform. But like you mentioned, doing a
risk calculation using REVEAL Lite 2, or the French method, that can be done in
less than 30 seconds. It really is very, very easy to do. And in my practice, my
nurse coordinators calculate the risk before I even walked into the room, and so
it’s readily available for me to talk with the patient about and discuss and
make critical decisions on.”

–Dr. Raymond Benza
The Ohio State University

Your browser does not support the audio tag.
Click to expand transcript

Dr. Raymond Benza:

Welcome to the Pulmonary Arterial Hypertension Initiative podcast. This podcast
is sponsored by and the presenters are being compensated by United Therapeutics.
In this series of podcasts, we will talk about how PAH treatment guidelines and
risk calculation drive treatment decisions in pulmonary arterial hypertension,
as well as their own experience in treating our patients with PAH. My name is
Dr. Raymond Benza. I'm a practicing cardiologist in the state of Ohio and
currently serve as the division director for Cardiovascular Medicine at the Ohio
State University. And I have the pleasure of being with one of my longtime
colleagues, Dr. Vallerie McLaughlin.

Dr. Vallerie McLaughlin:

Hi Ray. It's great to be here. I'm looking forward to this podcast. I am also a
cardiologist and endowed professor at the University of Michigan, and I direct
the Pulmonary Hypertension Program here.

Dr. Raymond Benza:

Thanks Val. In today's program, we will be discussing the importance of risk
assessment and pulmonary arterial hypertension management. According to the PAH
treatment guidelines, risk assessments should be driving our initial therapy
choices as well as any changes in therapy. So let's get started. So Val, can you
describe to me what risk assessment is all about in pulmonary arterial
hypertension?

Dr. Vallerie McLaughlin:

Yeah. Ray. I think that we as cardiologists have really incorporated risk
assessment into really many different diseases. We use different scores for
NSTEMIs and STEMIs, and CHA2DS2-VASc for AFib. We live this, this is really
something that is important to objectively care for patients. And over the
years, we have learned about the important parameters that are prognostic in
patients with pulmonary arterial hypertension. We've learned from many databases
that have included thousands of patients, the parameters that assess a patient's
risk or the severity of their illness and what their likelihood is of having an
event over time. I think the multiparameter approach is really critical because
this is a very complex disease and there are many different predictors,
including how the patient is feeling, whether or not their right ventricle is
failing, what their symptoms are like, syncope, how dyspneic they are, what
their Functional Class is. Those are all clinical assessments of the patients.
Echo has not been incorporated as much as I would like to see it.

We are really just more recently getting savvy about quantitating right
ventricular function. So if you look at the registries, the Echo parameters that
are included most often include right atrial area and the presence of a
pericardial effusion. I think we both know how that right ventricle function is
really critical in terms of the outcomes of PAH patients. And then of course,
hemodynamics are important. They define the disease and also reflect the
function of the right ventricle. And so when we talk about a multiparameter risk
assessment, we're looking at many of those different parameters and trying to
assess whether we think our patient is at low, intermediate, or high risk over
the ensuing years. And we learned from each registry that no matter what the
treatment is, if we can get a patient to a low-risk status, their 5-year
mortality is very minimal and that should be our goal for the patients to
reassess their risk and to drive them into that low-risk status. Ray, is this
your approach as well?

Dr. Raymond Benza:

It is my approach. And I think you really eloquently described the available
nuances that we take into consideration when we do these assessments of risk,
including very importantly, the multiparameter approach. We never hang our hat
on just one variable. And I also wanted to really emphasize the piece about
imaging because, as we'll talk about it a little bit later, many of the
contemporary tools that we have lack sophisticated imaging parameters as part of
them. And I think the tools that we have and that we use and that we will
describe later have to be really used in conjunction with some of these other
newer things that are coming out that we think are important, but achieving low
risk and using the multiparameter approach, I think of the 2 salient issues that
I really would like our practicing clinicians to take away from this.

Dr. Vallerie McLaughlin:

Yeah. I agree. Let's talk about how often we should be performing those risk
assessments. What's your approach to that?

Dr. Raymond Benza:

I think at least at baseline is probably one of the most important parts of risk
stratification. Really, when a patient comes to your clinic for the first time,
they may not have been on therapy. They're just newly diagnosed. They're really
assessing their baseline risk; it’s very, very important. Not only for the
treatments that we decide to place them on, but also for the patient's
information and their own means to plan their lives for the next year or 2. And
then after that initial assessment, and I think at minimum, every 3 to 6 months,
we should be evaluating our patients, but I'd like to get your opinion on a more
detailed approach and maybe peaking at a patient every time we see him in our
clinic. What do you think about doing risk assessments even in our routine
clinic?

Dr. Vallerie McLaughlin:

Oh, Ray. I think that's really key. I do that every single time I see a patient,
I think pretty much every encounter we have with a patient, it's an opportunity
to assess their risk and consider whether or not we've gotten them into that
low-risk status because that's where we want them. So we can look forward and
have confidence that they're going to do well. So Ray, there are lots of ways to
assess risk. Maybe we should go on and talk about some of those methods and you
were really the genius behind the REVEAL risk calculators. Why don't I talk
about the ERS approach, ESC approach, and the French approach? And you talk
about REVEAL, if that's okay with you?

Dr. Raymond Benza:

That's perfect Val.

Dr. Vallerie McLaughlin:

Yeah. The ESC and ERS guidelines published in 2015, this table, the very famous
table. Green, yellow, red stoplight sort of table about a number of different
prognosticators in pulmonary arterial hypertension and cut points that put them
at low, intermediate, or high risk. Most of these are derived from registry
data. Some of them are derived from gestalt, right? Like syncope is really a
gestalt thing and an observation that we've made, but not really included in
registries. So the important determinants include Functional Class. So we know
patients who have Functional Class 4 symptoms do very, very poorly and
Functional Class 1 and 2 do well, and Functional Class 3 sort of in the middle.
We have discussed hall walk already, the exercise tolerance, objective exercise
tolerance is something that's very important and that can tell us about a
patient's prognosis. So greater than that sort of magic 440 number, patients do
well, less than the mid-100 patients do poorly.

The Echo data and well, this is different; something I actually would like to
talk to you about. The Echo data in all of these is really very limited. The
presence of a pericardial effusion, which has been published a few times and the
size of the right atrium—that's really the data that we have from registries
that go into these scores. And I think that's a bit of a pity actually and
personally, when I do an Echo on a patient, I go look at that right ventricle.
It really is very meaningful to me and, I think, anyone who has experience in
this disease. I think that it's unfortunate that many registries don't have a
quantification of right ventricular function. Not all Echo labs quantify right
ventricular function. And so really we have very limited imaging of the right
ventricle in any of these risk scores, but I think it's so, so important. And I
guess sort of in my head, I incorporate that when I see patients as well. I just
thought maybe you'd want to comment on that, Ray, before we move on.

Dr. Raymond Benza:

Oh no, I agree with you 100%, Val. I think all the tools that we have developed
were developed in eras when imaging wasn't as prominent as a known risk player
in prognosticating. And I agree with you that as these risk scores and equations
evolve, that I would love to see more imaging data in the scoring systems. And I
think the important thing about that is if you really look at the spectrum on
how a patient evolves or progresses with PAH, we have changes in pressure that
then lead to changes in the right ventricle. And these changes can occur very
early, even before many of the other symptoms or variables that are accounted
for in these 3 scoring systems happen. So it even might add a level of greater
sophistication and predictability for early decompensation by including these
imaging parameters, entities, contemporary scoring systems that we have.

But ultimately, I think that it's the combination of these things that are
important, as you mentioned earlier, the ESC and ERS guidelines and even the
French method, which use a number of variables that are within the guidelines.
There are some differences between the calculators that we have developed in
REVEAL, but I was curious if you might want to go into a little bit more about
some of the French methodology for calculating risks before I went into what we
do with REVEAL.

Dr. Vallerie McLaughlin:

Yeah, for sure. In fact, let me just make a couple more comments on ERS/ESC. So
we talked about the syncope, right ventricular failure, Functional Class, hall
walk or cardiopulmonary exercise testing PCO2 is listed. Biomarkers are also
included, BNP less than 50, NT-proBNP less than 300. Very good prognostic
indicators puts the patient in a low risk. Whereas if the BNP is greater than
300 or NT-proBNP is greater than 1400, that puts the patient in a higher risk.
And then of course, hemodynamics are also important in terms of risk. And we've
known for many years, and this point is emphasized in the risk assessments. It's
not what the pulmonary artery pressure is. It is how the right ventricle is
coping with that pulmonary artery pressure. So, right atrial pressure, cardiac
index, and SvO2 are the hemodynamic parameters that are most predictive. So,
that's the ERS/ESC approach.

Now the French approach, which I really like, is very simple. They took a
different approach. They basically said, these are the 4 factors that are most
important to us. And it has to do with being in Functional Class 1 or 2, having
a hall walk over 440, having a cardiac index greater than 2.5, and a right
atrial pressure less than 8. And so those are the 4 factors using the invasive
French approach that they look at. And if you have 3 or 4 of those 4 factors,
your prognosis is very good. Whereas if you have less than that, the prognosis
is quite poor.

And then when they had a subgroup of patients that also had biomarkers and they
can incorporate the BNP or NT-proBNP, when you put that into the multivariate
analysis, the right atrial pressure and cardiac index falls off. And so the
noninvasive French is really just Functional Class, hall walk, and biomarkers.
And at least for me, that's something I do in clinic every time I see a patient.
I have all 3 of those parameters every time I see a patient. So that's the
noninvasive French approach. Ray, you want to tell us about REVEAL?

Dr. Raymond Benza:

Yeah. Thanks Val. So REVEAL calculators are in essence, very similar to the
ESC/ERS guidelines and the French method in that they both use very similar
variables. And that's good because that tells us that all the contemporary risk
stratification scoring and systems that we use are complementary. And we could
feel safe using them because many of the variables are shared. REVEAL takes it
just a little bit further down kind of the statistical pathway, than the
guideline or French method in that REVEAL methodologies are really derived from
very standardized statistical modeling. And very importantly, that the variables
that are used in these scoring systems are weighted against each other so that
you know the relative importance of one variable versus another. For example,
the Functional Class may be more or less important than an NT-proBNP level. So
it kind of gives you a way to really summate the risk with a little bit more
sophistication than some of the European methods.

So REVEAL Lite 2 is the derivation of an earlier model called REVEAL 1.0, and
what REVEAL 2.0 did is, it took a lot of the same variables that we had found in
the original REVEAL 1.0 calculator, which contains both demographic vital signs
and other more objective measurements and added to that hospitalization and the
risk that is imparted by recent hospitalization for pulmonary hypertension and
heart failure. It gave us a more objective means of measuring renal function,
which we know comorbidities are very important part of risk stratification and
renal function is one of the very important ones that have been highlighted and
uses a GFR and then set of a subjective assessment of renal insufficiency. And
also changes some of the scoring points and actually adds a more of a dynamic
nature to the changeable risk factors like BNP and the 6-minute hall tests.

So it takes those variables and then weights them, and you can calculate a score
that very nicely discriminates between low, intermediate, and high risk. Now,
the difference between REVEAL 2.0 and REVEAL 2.0 Lite is just the number of
variables that's required to make the calculation. The parent score, which is
the REVEAL 2.0, scores 13 variables that are associated with it. And these
contain some immutable risk factors. The factors that don't change over time,
like a patient’s type of pulmonary hypertension that they have and their gender
and age, even though age does change the risk points, here it is a kind of a
combination of age and gender. And then it has a number of easy-to-discern
variables, including vital signs like low blood pressure or high heart rate
combined with the Functional Class NT-proBNP levels in which there are several
cut points, and 6-minute walk distance, which also has several cut points.

And then add to that the Functional Class and Echo estimates of pericardial
effusion that you mentioned earlier, and then hemodynamics, which include
importantly, the right atrial pressure and the pulmonary vascular resistance.
And then REVEAL Lite, takes the parent calculator and really strips it down to
the easily obtainable, changeable variables that you can assimilate in each
clinical encounter. Very similar to the French method in that it uses vital
signs, Functional Class, the hall walk test, the NT-proBNP levels, and the vital
signs. But the big difference, as we mentioned earlier, is that these factors
continue to be weighted against each other, such that the discrimination index
with these more statistically derived methods are a little bit higher than those
that don't incorporate weighting. Well, I hope those descriptions of the
algorithm and calculators were helpful to the audience, but I'd like to ask Dr.
McLaughlin, if she has any further opinions or statements that you wanted to
make about the formal risks calculations that we make?

Dr. Vallerie McLaughlin:

I think that was a really elegant discussion of how REVEAL was developed. And
one thing I want to highlight is the difference between 2.0 and 2.0 Lite,
including the nonmodifiable risk factors. I think when we have nonmodifiable
risk factors in a risk calculator, it’s an excellent way to predict prognosis.
In fact, I think there's probably nothing more accurate at actually predicting
prognosis than REVEAL 2.0. But sometimes when we think of driving people to low
risk, we get a little challenged because there are so many nonmodifiable risk
factors in it that, sometimes our patients are going to be at very high risk, no
matter what we do. And that I think is one of the things that we've discussed
over the years about using calculators and driving patients to the low-risk
status. And I think REVEAL 2.0 Lite very nicely addresses that issue and really
includes the risk factors that you can treat with our medical therapies and have
success at trying to drive patients into a low-risk status.

I think that's a really important differentiation and in fact, REVEAL 2.0 Lite
and French are very similar with the exception of the additional parameters of
vital signs and kidney function. I think we've discussed 4 different tools and
they're all very good. They're all very meaningful. If you get into low-risk
status with any of those tools on therapy, no matter what the therapy is, the
patient's prognosis is good and that's what we're looking for—to improve the
patient outcomes. And so that leads us to the next discussion point. We have
this really wonderful data from all of these risk calculators that show if we
can get them to low risk, they do well. So let's talk a little bit about why
it's so important to use a formal risk calculator rather than just gestalt. Do
you have any comments on that, Ray?

Dr. Raymond Benza:

Yeah. Thank you Val, for pointing that out. I think that is really important.
And as we mentioned earlier in the talk, these tools are very complementary to
each other, and I think that can be used together—a more formalized, detailed
risk assessment with REVEAL 2.0, perhaps at baseline and maybe at a year. And
then when you're doing the peak of patient, like you mentioned earlier, watching
them at each one of their clinical visits, maybe that's the time where you can
implement the French method or REVEAL Lite. To get those on the fly risk
assessments, just so that you make sure that you are actually plotting the
trajectory correctly. Clinical gestalt, they think can really mislead us in
certain circumstances. Now we even have a recent study that compared clinical
gestalt and the formal risk calculation, and by reviewing patient's charts from
clinicians who treat patients with PAH. And both clinical gestalt and the
calculated risk were aligned in less than half of the 365 charts that they
examined.

I found that very surprising; 80% of the patients that are estimated to be low
risk by clinical gestalt were actually reassigned to a higher risk category
after formal risk calculation. That's really important because underestimating
risk is where patients can really be hurt because that's where you wouldn’t use
as an intensive mode of therapy, as opposed if they were really low risks. So
that's a really important thing is just not to miss the intermediate or
high-risk patients by utilizing a less nuanced way of predicting risk. And I
think there have been other studies that looked at formal risk calculation in
these earlier patients functional two class patients.

And I believe a retrospective chart analysis of 153 Functional Class 2 patients
who were either on mono- or dual combination therapy. And more than half of the
Functional 2 patients were classified as intermediate or high risk when their
risk was calculated. So again, these are patients whom if we use the single
variable of assessment like Functional Class or even clinical gestalt, we would
have underestimated their risk and perhaps not put them on the intensity of
therapy that the patients deserved to be on.

Dr. Vallerie McLaughlin:

I think those are great points, Ray, and I think we always have to have some
common sense, some gestalt in medicine, but this is an example where risk
calculators are helpful. But I might point out Functional Class, as we've talked
about, is subjective. Maybe a patient sounds Functional Class 2 because they've
limited their activities so much that they don't get short of breath. A really
skilled historian can try to dig that out, but sometimes patients just adapt.
And it also leads to the next topic of discussion is relying too much on one
thing such as Functional Class. I mean, for many years before we had all of
these additional tools and all of the additional data about BNP and what have
you, Functional Class was the holy grail and it's something that's easy. It's
inexpensive, we do it every single time we see a patient. But it's not enough,
is it?

Dr. Raymond Benza:

No, it's not. And it's like you said, we were both weaned on Functional Class,
as cardiology fellows. And it's been ingrained into the way that we evaluate our
heart failure patients, but it is, the bottom-line, subjective. It really relies
on patient input, their memory, and even their honesty at some point. And so
this subjective evaluation really has to be weighted and thought of in a
different manner. The formal risk assessment is objective. We're talking about
multiple parameters. Some that are objectively measured, but not subjectively
measured like hemodynamics or vital signs or pericardial effusion on an echo or
an NT-proBNP level. So, balancing these subjective with objective factors I
think is very good, but I don't want to shortcome the Functional Class because
even despite its subjective nature and every risk calculator and every
assessment we have done, it's always peaked its head as something that is
important. It may not be as discriminatory as the others, but it certainly
remains important.

Dr. Vallerie McLaughlin:

Yeah, no question. Now, some people who are critics of this say, "Oh my gosh, it
takes so much time. You have a limited amount of time with a patient appointment
and Epic or the electronic medical record makes it all so complex. I don't have
the time to do this over the course of a visit." And I think that's really not a
very good excuse to do something that is so important and so prognostic for a
patient. A French invasive or noninvasive is really 4 or 3 variables. It really
doesn't take that much time. And Ray, I know that you've actually gone to a lot
of trouble trying to develop apps to make the REVEAL calculation easy as well.

Dr. Raymond Benza:

Yeah. So, there are apps that are currently available. There are websites that
are available that can calculate the risk for you. We've made a lot of headway
in some of the EMRs in programming them to calculate risk on the fly. So I think
the nature of the calculation is getting easier and easier for practitioners to
perform. But like you mentioned, doing a risk calculation using REVEAL Lite 2 or
the French method, that can be done in less than 30 seconds. So it really is
very, very easy to do. In my practice, my nurse coordinators calculate the risk
before I even walk into the room. And so it's readily available for me to talk
with the patient about and discuss and make critical decisions on.

Dr. Vallerie McLaughlin:

Yeah. Ray, I think you've contributed so much to this area. It's really been a
delight to have this conversation with you. I think to wrap up, we've really
emphasized that formal risk calculations are important to help us monitor our
patients and choose the appropriate therapy for them. So it's been such a
pleasure and this concludes our first PAH Initiative podcast. Please join us
next time when we will discuss initial therapy choices based on risk
calculation.

If you would like more information on risk assessment, PAH treatment guidelines,
or to use an online risk calculator, please visit pahinitiative.com/hcp.


DISCUSS RISK WITH YOUR PATIENTS

Help your patients become more invested in achieving low risk. Use one of the
tear pads below and give each patient their score at every visit so they can
track their own risk score.


RISK CALCULATION TEAR PAD: REVEAL 2.0 & ESC/ERS GUIDELINES

Use this double-sided tear pad to quickly calculate your patients’ risk score
during their visit.

Download PDF
Order Copies


RISK CALCULATION TEAR PAD: FRENCH NONINVASIVE CRITERIA

Use this tear pad to determine the number of low-risk criteria your patient has
achieved while facilitating a discussion on risk assessment.

Download PDF
Order Copies


IF YOUR PATIENT IS NOT AT LOW RISK, TREATMENT ESCALATION MAY HELP IMPROVE
PROGNOSIS5


See Medication Classes

ESC/ERS=European Society of Cardiology/European Respiratory Society.References:
1. Boucly A, et al. Eur Respir J. 2017;50(2):1700889. 2. Hoeper MM, et al. Eur
Respir J. 2017;50(2):1700740. 3. Kylhammar D, et al. Eur Heart J.
2018;39(47):4175-4181. 4. Benza RL, et al. Chest. 2019;156(2):323-337. 5.
Humbert M, et al. Eur Heart J. 2022;43(38):3618-3731.
 * Overview
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   * Risk Score Calculators

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 * Treatment Pathways
   * Prostacyclin-Class Therapy
 * Resources
   * PAH Curriculum
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   * Published Studies in PAH

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