PAH Initiative Events

How Specialist Use Echo To Monitor Your PAH
Lana Melendres-Groves, MD:

Welcome to the PAH Today National Broadcast Series. My name is Dr. Lana Melendres-Groves, and I'm the medical director for the pulmonary hypertension program at the University of New Mexico. It's my great pleasure to be here today to have the opportunity to talk about how specialists use echo to monitor your PAH. Now, first, let me provide the following disclaimer. This presentation is sponsored by, and made on behalf of, United Therapeutics. Healthcare professional speakers are compensated by UT.

Not all drugs are appropriate for all patients, so please speak with your healthcare professional to determine which treatment plan is right for you. All right, now some of you savvy individuals out there may be thinking, 'Gosh, this topic sounds familiar,' and in that case, you'd be right because this happens to be an extremely important subject when we talk about PAH, the echocardiogram and the right side of the heart, we felt that it was so essential to revisit this topic because new information about how we utilize an echocardiogram and how the right heart looks is coming to light every day.

For instance, we now better understand how the right heart has important signals that it sends to us to tell us how someone's PAH might be doing and where it might be heading. We time and time again are seeing that our treatment guidelines further emphasize the role of frequent echocardiograms to monitor PAH. We continue to expand the set of right heart measurements that we utilize as PAH specialists to be able to determine someone's risk status. With that in mind, let me give you a little bit of an overview of the presentation for today.

First, we're going to talk about the heart and the heart in PAH. Then we're going to take a little bit of a deep dive into echocardiogram. And last, we'll continue to talk about how we can learn more. First, let's talk about the heart in PAH. I always think that this is a tremendously essential portion of any of these sessions, because understanding how the heart works and how the disease PAH affects the heart I think really helps us to understand what we might be able to do and how we might be able to monitor our patients.

In this situation, what the real issue is is that the blood vessels in the lungs start to become narrowed. These blood vessels eventually make it more difficult for the right side of the heart to be able to pump blood into the lungs. As it becomes more difficult to pump that blood into the blood vessels of the lungs, the right side of the heart starts to enlarge and it becomes weakened. This becomes important because the changes that occur in the heart are frequently seen prior to someone's symptoms worsening or changing.

The goal would be let's monitor the heart so that a healthcare provider might be able to adjust their treatment plan for an individual before those symptoms ever become problematic. You may ask, how are we going to do that? We have a couple of ways that we routinely monitor patients with PAH. The first is going to be what we call a noninvasive form of evaluation, and that's with an echocardiogram. The other is with a right heart catheterization, which tends to be a more invasive way of gathering information on how the heart is doing, how it is functioning, and how those blood vessels are overall functioning as well.

I think first, let's talk about a right heart catheterization. I know that for many of my patients, this is a really scary time when somebody is talking to them about having an invasive procedure. Understanding what it does for the provider and how that might provide you information as a patient or as their caregiver I think becomes essential in then saying, 'Let's move forward.' A right heart catheterization measures the pressure in the heart and the blood vessels of your lungs. When higher pressures are occurring in the heart, it's typically a sign that the blood vessels may be further narrowing, therefore indicating worsening of somebody's PAH.

With a right heart catheterization, we usually use it for two different things. The first is a definitive diagnosis for PAH. This is the only way that we are able to tell somebody that they absolutely have this disease and this diagnosis, and that from here we can then move forward with recommendations on next steps. The other way that we utilize a right heart catheterization is monitoring somebody's disease. And typically, as we are adjusting or changing treatments, we may need to further assess what those measurements are.

Some of the measurements that we are looking at when we do a right heart catheterization include something called the pulmonary vascular resistance or PVR. I just want to break it down a little bit. Pulmonary, that's talking about the lungs, vasculature, that's talking about the blood vessels, and resistance is how difficult it is to be able to get blood through the blood vessels. That becomes extremely important because it's the resistance that the heart on the right side has to overcome to be able to get blood through the lungs.

One of the other measurements that we look at is going to be the pulmonary artery pressure or PAP. This is the pressure in the artery that is between the heart and the lungs. One of the other measurements is going to be the right atrial pressure. We have two chambers, two muscle chambers, on the right side of the heart. One of them is called the right atrium and the other is the right ventricle, and looking at the pressure in the right atrium helps give us further information about how our patient is doing.

Collecting all of these measurements together will help us to understand where our patient is at that time and what may be expected as we move forward. This is also considered an invasive process, because we are going into somebody's blood vessel with a catheter. Now, an echocardiogram is different in that it is collecting essential information about the heart and the function of the heart, but it's done in a noninvasive way and it is able to potentially monitor changes that may be occurring in the heart.

Now, some of you may look at this and see this picture on the right side of the screen and say, 'That just looks like an inkblot test. There's black and there's white and there's gray, and it doesn't mean very much.' What you have to understand is that we have the capacity through ultrasound to use sound waves that recreate a picture of the heart. And that all the black and white and gray when you kind of understand what it's showing you can be very meaningful. It is used to assess the structure and the function of the heart, and it is the most widely used tool to do so.

What do these echos show us as healthcare providers? Well, it can show us how PAH is affecting a patient's heart at the time that they're diagnosed, and then it can be used to assess how current treatments might be improving or affecting the way a patient's heart is working. These are all essential in determining the treatment regimen that somebody might most benefit from. And that takes us into really looking at that echocardiogram. Let's try to understand what all that inkblot test that I just showed you means.

From the standpoint of a healthcare provider, we're looking at the size and the shape of the heart in terms of the right side and those chambers of the heart. We want to know how well is the heart squeezing or the functioning of the heart, and then looking at the response to therapy. You may have noticed I flipped the heart over. And that's exactly what our pictures look like, an upside down picture of the heart.

We've taken the ventricles, which normally point downward toward the ground if we were standing up or sitting up, and the atriums, which normally sit up toward our head, we've now flipped them upside down. Hopefully it's a little bit more meaningful when we look at this picture. We've labeled the ventricles. You can see that there's the right side and the left side and the right atrium and the left atrium. This is a picture of a patient who does not have PAH and would have what we consider a normal appearing heart.

What happens if you don't have PAH? What are we supposed to see? Well, the right ventricle is smaller than the left ventricle. You can see that that's pictured based off of the arrows, the blue arrow indicating the size of the right ventricle and the red arrow indicating the size of the left ventricle. Now, some of you may be familiar with what an apex is, but it usually means sort of the top portion or kind of a triangular or pointy area. In the right ventricle, that's the top portion that you see on your screen and that indicates the apex of the right ventricle.

What we expect to see is that that's very thin walled in terms of the muscle and narrow. The other piece that we look at are going to be the atria. You have the right side and the left side, and they typically are quite similar in size and as you can see, quite a bit smaller than the ventricles. Now, let's take what we've just learned in terms of a normal appearing heart and move into a patient who has PAH. The first thing I want to let you know is that we've presented what would be a very advanced severe PAH patient's echocardiogram.

Each individual patient's heart is going to look a little bit different, but this I think helps demonstrate some of the changes that we might expect or see in patients who have PAH. First, the right ventricle you can see has gotten tremendously large, much bigger than the left side of the heart. You can see our blue arrow has increased in its length and our red arrow has shrunk. That apex that is supposed to be nice and narrow and pointy, you can see is now quite a bit larger.

The white aspect that surround it, that's the muscle wall and that's started to thicken. And last, you can see that the right atrium, which used to be about the same size as the left atrium, is now substantially enlarged. By monitoring the right heart with these echocardiograms in this noninvasive manner, we are able to see some of those changes that might be occurring in terms of the atrium, the ventricle, the apex, the squeeze.

And by seeing that information, we may be able to proactively or preventively try and come up with recommendations that could prevent our patients from then later developing symptoms, meaning things like the walk test that we have you do or talking to you about functionally how you're feeling every day. Most importantly, trying to prevent the hospitalizations that affect how people do long-term. The echocardiogram is also such an important part of assessing someone's risk.

I know that this isn't per se our topic today, but I do think that understanding risk status is just essential to every person who has PAH. The chart that's up on the screen is indicating some of the variables that we look at to try to better understand how our patient is going to do long-term. One of those variables is the echocardiogram. Before I talk about echocardiogram in the realm of risk status, I want to remind everybody that we look at risk status in sort of a green, yellow, and red zone.

Our green zone, which is the goal based off of our guidelines for all patients, whether we're able to get them to low risk, that is individual, but our goal is to get them to low risk, and that's our green zone. Our yellow zone is our intermediate risk in terms of how a patient will do long-term, and then our red zone is our high risk patients where we're really having to be thoughtful about how do we get those patients in an intermediate or high risk zone down into that low risk status and how do we keep them there.

Now, the way echo plays into this is that many of the things that we have already discussed today are talking about what is the size of the right atrium, how does the muscle squeeze, and what is the output, meaning that function of the right ventricle. Is there potentially fluid around the heart? Those are all aspects that we know play into someone's risk status. We also know that by assessing someone's risk every three to six months, we have the ability to proactively try and change treatments and adjust therapies and our care and recommendations to what we're seeing in someone's risk status.

Our PAH treatment guidelines, as I mentioned, indicate that reaching that low risk status is an extremely important goal and one that we are attempting for all of our patients. All right, I know that was a lot, but we continue to learn and I hope that you've stuck with me thus far. One of the things that we've looked at is how has utilizing echocardiogram on a more frequent basis potentially helped our patients? What was done in this research study was to actually take patients who were at a functional class two.

Now, I know we haven't talked about functional class today, so I just want to give you a brief refresher. Functional class is how you feel. What can you do? What is your exercise tolerance or your functional ability? In a functional class two patient, those individuals are somebody who really are able to maintain all of their activities of daily living. But if they push it a little bit hard, if they have to run to that bus or get out on a hot day to the mailbox, they may notice that they're a little bit more short of breath or a little bit more fatigued.

That's the group of patients that were looked at. Their charts were then pulled and the frequency of their echocardiograms reviewed. What was found was that the physicians who performed echos every three months were able to better assess a patient's disease status than their counterparts who were only getting echos at every seven to 12 months. This brings up the point of why it's essential to find a PAH specialist who is right for you. Because PAH is a rare disease, not every cardiologist and pulmonologist has expertise in treating it.

PAH experts have experience in reading and understanding those components of the echo that we've talked about today and more specific to each of their individual patients with PAH. They also use those echos to interpret how the treatment plan may be impacting the right side of the heart. Thankfully, we have the ability to look for PAH specialists through search tools and other means. We've provided one today at findapahdoctor.com.

Please, if you feel that you're in need of somebody with more expertise or you haven't found that person yet, this may be an opportunity to find somebody who's just right for you. In summary, monitoring the right heart is important in PAH. Changes in the heart are among the first signs of worsening in PAH. An echo is an easy, noninvasive way to look at the structure and function of the heart. Today, echo is an important part of risk assessment.

Monitoring the right heart allows healthcare providers to be more proactive with treatment toward preventing symptoms before they happen and can help patients lower their risk status. If you're interested in watching more, learning more, please visit our PAH National Broadcast Series and don't forget to secure your spot to join our next broadcast event, which we'll be discussing the five things you need to know before your next PAH appointment. You can do this at pahtoday.net.

Also, consider joining the PAH Initiative at pahinitiative.com, where you can stay informed, stay motivated, stay focused on your future. I'm hopeful that you all have questions because there's always more to learn, and please feel free to submit those in the question and answer box at the bottom of the screen. While you have an opportunity to think about that and type in your questions, I think from my standpoint, I get frequently asked questions and I thought, what a nice way for me to provide you with information that I'm able to give to many of my patients in clinic.

For instance, one question that I frequently get is, does my insurance company decide how often I can get an echo? Now, I can't speak to every insurance that each person has across the country, but what I can tell you is that because of our guidelines within PAH, we have the ability to request an echocardiogram based off of our patient's needs. That means if they're feeling different, if we're changing treatments, if they've been hospitalized, if there are other aspects of their presentation that are concerning, we as their provider can order a new echocardiogram to reassess, and that our guidelines indicate that that's appropriate and that insurance should be required to then cover that.

Now, please make sure that you ask your provider and case managers. If you need prior authorizations, don't hesitate to follow up on that. But from my standpoint, if we don't have the information, we cannot do the best for our patients. I definitely fight the fight often against insurance to make sure that each of my patients is able to get what they need. All right, moving into another viewer question that I frequently get is, if my symptoms have not changed much over the last year, does that mean my echo results will improve?

I think that this is a really complicated question, even though it seems like it should be so simple. Because if you feel the same, shouldn't everything else either look the same or maybe it will look better? I think that as many things in PAH, this is very individualized and it may depend on what those symptoms are. If somebody's symptoms are significant and they have tremendous amount of shortness of breath or fatigue, then that may be reflected in what the echo looks like and we may not expect to see improvements.

However, if somebody says, well, my symptoms haven't changed and I have been feeling good and been able to do my activities as I would like to be able to do them, that doesn't mean that your echo may not be looking more normal. I think the only way to really assess that is not just through an echo, but through several other tests that all play into what does somebody's sense of how they feel along with the testing that is objective, meaning it is just what it is, tell us. All right, let's go ahead and move on to another question.

Please continue to send them into me. They're piling up, and I'm going to get to them, I promise. Next question is, how accurate is an echo at estimating pressures? Now, this is one of those questions that I love for my patients to come with because it means all of you guys are thinking ahead. If you're getting a test and you're spending the time doing it, can we rely on the information?

Over the past several decades, our echocardiographic technology continues to improve and enhance the images that we see. So that these days when we look at some of the trials and research that has specifically looked at how accurate the echo information is, that we feel very confident when we have a technician who has the ability to give us and get the views of the heart that we need.

We have individuals who specifically are looking at the aspects that some of which we talked about today, that the estimated pressures tend to be extremely accurate. And when comparing them to what we get on a right heart catheterization, we feel very confident that they will mirror what we're seeing on our invasive testing. All right, we're going to go through a few more, and then we will get to yours from today. Next question is, how do you evaluate a patient's pulmonary pressure in comparison to other test results?

Ah, this is a good one, and this is good because a number seems like that should be most accurate and that should give us what we need. But what's interesting about a number is that that can shift throughout a day and throughout time and doesn't necessarily reflect exactly how the heart is feeling. Sometimes a pressure can actually go up when the function of the heart improves, because now it can squeeze better and increase the pressure that it is sending blood through those blood vessels.

Yes. Do I take into account what someone's pressure is being measured out on echocardiogram? Absolutely. But more important to me is, what does the heart look like? Are those muscle chambers normal in size and function? And if they're not, what do we need to do to try to get them there? Our next viewer question is, is elevated pressure acceptable if all other factors appear to be okay? Well, I think that this one is tough. It's tough because I'm not sure what I would consider acceptable.

Acceptable to me for my patient is a normal appearing heart, a low risk status. Their symptoms are completely manageable so that if they're not completely normal, they're able to do everything they need and want to do throughout the day. If we're talking about somebody who's sitting in that green zone of low risk and overall feels good and is able to enjoy and participate in their life and the other aspects of their echo look good, then even if that pressure is elevated, I may not use that to make changes, but I'll keep it in mind.

How frequently should I have a right heart catheterization? Like everything else in PAH, this is completely individualized. This is about you. What do you need as a patient? Or if you're the caregiver, what does the person that you're taking care of need? A right heart catheterization can be used for so many different reasons. We mentioned, of course, diagnosis, but a right heart catheterization may be needed if we're changing someone's medication to reassess what that looks like. It may be needed if someone's symptoms have worsened.

It may be needed if there are other things going on so that we're not able to get walk tests because somebody needs a surgery and had a broken hip, or somebody has pain, or they weren't able to get to the echo, or for some reason we're not able to get good views on an echo. There are many different reasons that we may recommend a right heart catheterization. This is where I think having a specialist who is in tune with you and discusses what your results from your testing are, that's how you make a decision on when a right heart catheterization should be performed.

What I will say is that if I haven't performed a right heart catheterization in a year or over a year on one of my patients, I need a reason that I'm not. And maybe that's because they're doing fantastic. I think that those are the aspects of talk to your provider. This is a perfect question for them. I know a right heart catheterization can be stressful and scary. But in the right hands, it is a very low risk procedure with a lot of information that can be gained.

Coming up to our last question before we get to all of yours that I look forward to, what does it mean if PAH is found in a right heart cath but not detected during an echo? Well, actually for me, this probably means that whoever suggested the right heart catheterization is spot on because an echocardiogram is a tremendous screening test for PAH, but it isn't diagnostic.

Often if we are screening patients because they might have an underlying diagnosis such as certain connective tissue diseases or other underlying medical conditions that might put them at risk for developing PAH, we may not see that the echocardiogram is abnormal at this point in time, but they may have other symptoms or other findings that would suggest that there's concern for PAH. And by doing a heart catheterization early on where we're finding this disease in an early state, it gives us a lot more opportunity to positively affect someone's outcomes.

We can't rely completely and entirely on an echo, but it definitely is our most widely used means of screening and evaluating our patients.

Thank you all. I really appreciate the time you've spent with me today. This is the best part of my life in terms of PAH, which is having opportunities to interact with patients.

They may not be mine in clinic, but they're mine because this is a disease that I'm passionate about and I want to provide opportunities for patients and their caregivers to continue to learn and advocate. If you want more information, please go to www.pahinitiative.com or connect with us on social media. Have a wonderful rest of your day. Thank you.