PAH Risk Score Calculators

Aim for low risk to help improve your patient’s chance of survival1-4

According to the 2018 WSPH treatment guidelines, each patient should receive an objective, multiparameter risk assessment at diagnosis, and then every 3 to 6 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 4 calculators, depending on which works best for your practice.

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

ESC/ERS Guidelines

French Noninvasive Criteria



Instructions on how to use

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 excerpt, 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

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Dr. Vallerie McLaughlin:

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 complimentary 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 short come 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.

EHR tools

REVEAL 2.0 in Epic

Directions on using the REVEAL 2.0 calculator in Epic

Pursuit Lists in Epic

Instructions on how to create a Pursuit List that helps identify patients based on risk status variables in Epic

Pursuit Lists in Cerner

Instructions on how to create a Pursuit List that helps identify patients based on risk status variables in Cerner

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.

PAH Initiative risk calculation tear pad: Reveal 2.0 & ESC/ERS Guidelines pdf thumbnail

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.

PAH Initiative risk calculation tear pad: French noninvasive criteria pdf thumbnail

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.

If your patient is not at low risk, guidelines indicate that treatment escalation may help improve prognosis5

See Medication Classes
ESC/ERS=European Society of Cardiology/European Respiratory Society; WSPH=World Symposium on Pulmonary Hypertension.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. Galiè N, et al. Eur Respir J. 2019;53(1):1801889.