When science and AI converge: the evolving landscape of Pulmonary Arterial Hypertension (PAH) care
Organized by the Alliance for Pulmonary Hypertension
Robert Pleticha
Welcome everyone, and thanks for joining this Alliance for PH webinar. My name is Rob Pleticha, and I’m a director at the Partners4Patients Foundation. We’ve been very happy and proud to support the Alliance for PH as the secretariat, and would like to thank Pisana Ferrari for helping to organise this webinar. Today’s webinar is titled “When Science and AI Converge: The Evolving Landscape of Pulmonary Arterial hypertension Care”. This is number two of four quarterly webinars that the Alliance for Pulmonary Hypertension is organising in 2026. We’ve been organising their webinars for a few years now, so you can look back on our YouTube channel and see if there are other topics that interest you. Today we look at practical digital tools and approaches that are already available and already making a difference for people living with pulmonary arterial hypertension, from remote monitoring and home-based tests to staying connected with clinical teams and accessing mental health support online.
The webinar will last about one hour, with questions towards the end. If you want to put a question in the chat anytime, you’re welcome to. The recording, the slides, and the transcript will be available later this week on the Alliance’s Knowledge Sharing Platform, as well as on the YouTube channel. Just let me know if you need any assistance throughout. You can send me a chat message here and I would be glad to help you. I’d like to thank our speakers for sharing their time so generously with us. We have quite a nice panel today.
With that, I’d like to introduce our first presenter, Ciara McCormack. She is an Assistant Professor of Exercise Science and Health at Maynooth University in Ireland, and she’ll be presenting first today. Thanks very much, Ciara. Go ahead.
Ciara McCormack
Good morning, good afternoon, or whatever, as I know we’re all in different time zones. Thank you to the Alliance for Pulmonary Hypertension for having me here today to speak on this topic. I won’t dwell too much on the different forms of, I suppose, remote digital telehealth that we have, but what I want to talk about over the next few minutes or so is specifically focusing on telerehabilitation. As we all know, we’re all online today. When we think about telehealth or telerehabilitation, we’re thinking about taking that from the hospital to the home. It’s often supported by digital technology. I’m going to present why telerehabilitation is important in the area of pulmonary hypertension, and then talk you through some of the work we’ve done here in Ireland on remote-based exercise interventions for pulmonary hypertension patients, and findings from that research as well.
So, just to set the scene, when we think about exercise rehabilitation up until recently in pulmonary hypertension, it was mainly hospital-based under supervision, or community-based. But in the last couple of years, we’ve had more research done in moving it from the hospital to the home, and that has been done in many different formats, using phone calls, app-based interventions, the internet, video calling, and a number of different ways. Really, the benefit of moving it from hospital to home is that accessibility part, that I’ll touch on in a moment. But before we get into telerehabilitation, I wanted to set the scene for those who may not be so familiar with the background of exercise in pulmonary hypertension. Since 2006, when the first-ever exercise study was conducted in pulmonary hypertension, the field has grown.
In 2019, the European Respiratory Society (ERS) released a statement to say that exercise training was effective, safe, and cost-efficient for those living with pulmonary hypertension who were stable and under supervision during exercise training. So this is really important research that has helped advance the field. However, the problem with exercise training in the hospital, as many of you will know, is that many of the centres – and I understand this diagram, or this map, doesn’t pinpoint every pulmonary hypertension centre of excellence – are located in one specific area in each European country. For us in Ireland, we only have one centre, based in our capital city, Dublin. My challenge was to develop an exercise programme that was suitable for our population of pulmonary hypertension patients living in Ireland, and that was accessible. What we found were some of the barriers to doing this in hospital: the travel time that would be required, as many of our patients live very far away.
There was the time it would take them to get there, the time within the hospital to identify space, the fatigue, the physical and mental side of having to travel to the centre, and then the geographic spread. So what we looked at was this model of care that would move from the hospital to the home. But first we wanted to understand, well, what did our patients want? We conducted some qualitative research, and what we found was that patients said there was a lack of physical activity knowledge provided to them. They actually wanted a home-based setting. So that was very interesting for us to find, that the preference for them living in Ireland was home-based. They wanted accountability and monitoring while doing it, and they wanted clinician-delivered physical activity information and guidance, in the sense that they wanted their clinicians to point them in the right direction and provide them with information, support, or services that they needed to be physically active.
And so I developed the Physical Activity for Pulmonary Hypertension home-based exercise intervention, or FAB for short, that you’ll hear me refer to. This was a remotely delivered exercise intervention that included virtually delivered induction sessions. We had three education sessions. We had one-to-one health coaching sessions that took place on a Zoom platform, like we’re doing now. We developed physical activity manuals and logbooks specifically for our pulmonary hypertension patients. We provided them with specific exercise videos, a stationary bike, and a Fitbit wearable tracker that some of you may be familiar with or already have as a device yourself, and a chest-worn strap so that we could remotely monitor their six-minute walk test. Our intervention included both males and females, the average age was 50, and we had patients who were living almost 200 kilometres away from the centre engaged in this programme.
So really, we were able to reach people at the very end of our small country here in Ireland. We also conducted the assessments remotely, and I know we’ll hear later on today more about the remote assessment models, but we were able to monitor the six-minute walk test remotely in real time. That allowed us to make sure the patient was safe, allowed us to capture the walk distance that they were doing, their step cadence, their heart rate, and their SpO2, or oxygen saturation. We also conducted strength testing via Zoom, and then we posted out any additional questionnaires, which nowadays we probably would have done online or via an app as things have changed. The intervention was conducted at home, in the patient’s own home, at a time that was suitable for them. They were given a programme that included a mixture of walking, cycling, resistance exercises for the upper body and lower body, and some respiratory breathing exercises.
The patients would work through this on their programme, which was individualised to them based on their baseline measurements and how they were progressing over the course of the couple of weeks. This would be decided on during their health coaching session, which took place on a biweekly basis via Zoom, as I mentioned earlier. What was important was that the patients’ progression was as tolerated. It was individually prescribed, and it was progressed based on the frequency, intensity, time, and type of exercise they were doing.
We were also obviously measuring safety measures. They had a pulse oximeter and were working off and monitoring their Borg scale, which they logged in the logbook. What we saw – I won’t dwell too much on these – but what we were really interested in this particular study was looking at whether it was safe and feasible to conduct this type of exercise intervention remotely, to do the testing remotely, to deliver the intervention remotely, and for patients to work on their own and report back with the monitoring. Then we wanted to see the impact it had on patients on a number of different markers like physical activity, quality of life, and psychological constructs. We’ve published our research, but the main thing I think is important here is that, in this cohort, what we found was that it was safe for patients to exercise at home. We had no adverse events. It is important, though, to note that obviously this was a research project, so there were inclusion and exclusion criteria, and patients were screened prior to taking part in this.
That’s an important part when we think about remote delivery of telerehabilitation, because it may not be for everyone. But what we found was that, over the course of the 10 weeks, we saw a significant increase in the quality of patients’ reported quality of life, their six-minute walk test, their daily step count, their sleep duration, their confidence in their ability to exercise, and a reduction in fatigue levels. We found that it was feasible and was implemented as per plan despite challenges during the pandemic.
Patients remained highly engaged and adhered throughout the programme. They reported it to be very enjoyable and acceptable, and they perceived it to be very practical and usable, in that they were able to exercise in the comfort of their own home. I also always like to think about the patient’s feedback. I won’t read out this whole quote, but I think what’s really important here is that this programme gave this particular patient the opportunity to engage in an activity that they felt in control of, and they had seen benefits despite living with pulmonary hypertension for many years. One thing that really stood out here is that they really wished this was something that had been provided for them earlier. What we’ve seen in the last number of years is that there’s a real push towards patients wanting to be more in control of their life, and to engage in other activities, such as exercise, physical activity, and the day-to-day things that they like to do.
Exercise can be a really important tool to help them do the things that they want to do. Importantly, we got some patient feedback from engaging in the Physical Activity for Pulmonary Hypertension trial, and what they found was that it was convenient and accessible. Because they had to do the exercise themselves, they were using their watch to monitor the symptoms, writing in their logbook, and reporting back to the health coach. They were able to develop exercise self-regulation skills, so they understood when they were pushing themselves too much or too little and could understand their symptoms if they had any. They really valued the support and accountability that they got from the health coaching sessions, and they also talked about the physical and overall wellbeing improvements that they noticed over the 10 weeks. Really, what we’re trying to say is that we now notice that exercise and telerehabilitation, like many things, have become very important for patients.
But I do recognise that there are still unanswered questions when it comes to telerehabilitation in pulmonary hypertension: which patients would benefit most, what’s the optimal way to deliver it, and how can we increase accessibility, particularly for those who may not have access to devices or phones. We still work in this space, and we’ll try to push and continue to increase that accessibility. Just to finish off, where do I see the future going? I really think that wearable devices – and I’m sure we’ll hear more today – are providing a really great opportunity for clinicians and healthcare providers, and for patients, to help manage and improve their physical activity. Apps are on the rise, particularly pulmonary hypertension-specific ones, and there’s some work we’re doing in that area. Our healthcare is getting more connected, and my hope is that all patients who are suitable, stable, and medically cleared to exercise at home will have the opportunity in the future to do so.
Thank you very much.
Robert Pleticha
Thank you very much, Ciara, for that excellent presentation. Next up, I want to welcome Steve. Steve van Wormer is the president of phaware global association, and he’s going to show us a very exciting app today called “HeartWorks”. Steve, please go ahead.
Steve van Wormer
Thanks so much. I want to talk to you about a new pulmonary hypertension mobile research and resources app called HeartWorks. This is an app that I am happy to say is available in every app store that has a pulmonary hypertension advocacy group across the planet. So that’s 40-plus app stores – or 40-plus countries, rather – in 25-plus languages. Let me tell you a little bit about phaware, just very briefly. We’re a nonprofit organisation. I’m talking to you from Los Angeles. We’re dedicated to raising global awareness and accelerating research for pulmonary hypertension through technology like mobile apps and storytelling that amplifies the voices of patients worldwide. So what is the HeartWorks app? It’s basically a mobile wearable health data collection system. It’s got full data privacy protection, Health Insurance Portability and Accountability Act (HIPAA)-compliant servers, and real-world patient experience data. It really was born out of the idea of doing out-of-clinic or hybrid research studies, much like when a pulmonary hypertension patient goes to a clinic and might do a six-minute walk test once, twice, or three times a year, and that really kind of determines their course of treatment.
Our big idea is that a patient with a mobile phone does a hundred six-minute walk tests a day, every day. So it was really about big data and getting more information to researchers and doctors for this disease. This app is not just for patients and caregivers; it also has resources for health care providers, certified proessionals, medical professionals, and some Continuing Medical Education (CME). It really is meant to be used by the entire pulmonary hypertension community. As I mentioned, it’s in basically every app store on the planet, and it is available in native languages for all those various locations. There are clinical trial matching tools and clinical trial finders. There are global pulmonary hypertension news feeds from across the planet from many great organisations. There’s a podcast player, which we’ll get into, and more importantly, a curated AI system and a patient experience portal, and we’ll talk about that as well. But first I have to say, we couldn’t make this without a really core collaboration with many great organisations, many trusted organisations, I should say: places like PHA Canada, Team Phenomenal Hope, the Stanford Wall Center, who did 3,000-plus six-minute walk tests to help validate this app, PHA Europe, and Global BioNews Services, which includes Pulmonary Hypertension News, Pulmonary Fibrosis News, Scleroderma News, and PPHNet, which is a paediatric conglomerate of 15 or 16 hospitals across North America.
PHA UK, Alliance for Pulmonary Hypertensio (AfPH), of course, TBX4Life, Vanderbilt, which is running a clinical trial, and the University of Alberta, which is also doing a study called CRAVE with us. Again, just some top-line information about HeartWorks. I said it’s a wearable device that works with your Apple iPhone, your Apple Watch, Android devices, and Android smartwatches. You don’t need to have a watch, but if you do have a phone, that’s basically what you need to do these six-minute walk tests. What it’s really doing is simplifying and allowing clinical research participation remotely, and helping to push the science forward. As I said, it delivers personalised education for patients, caregivers, clinicians, and advocates anywhere, anytime, across the planet. Let’s see what we have here. This is an example of how it was really born out of the idea of a remote six-minute walk test. These are just a few screenshots showing what you will see once you download the app: some pre-walk information, some pre-walk questions and answers, and then you have a six-minute walk route. That information is stored locally on your phone. Let’s say you are not in a trial; you’re just a user, a patient or non-patient user. That data is stored on your device, so you can see those trends in reports and then share that with your medical professional when you see them and say, “Hey, I’m walking further.
I’m walking less,” so you can determine that. I’d be remiss if I didn’t mention our podcast series, which really has helped us amplify the patient voice over the last decade. All these podcasts – 600 of them, rather – come out weekly, and we’ve been recognised by two third-party individuals or organisations as the number one pulmonary hypertension podcast on the planet based on relevancy, social followers, and freshness. It’s really about delivering information to patients and the community, because there are a lot of people who live remotely and are isolated: isolated geographically, isolated mentally, isolated financially. Maybe they have insurance; maybe they don’t have insurance. So what we do is bring the best minds on the planet in a weekly podcast series, in the hope that no one gets lost and missed in the shuffle. This is really kind of the heart of the app as well: every interview we’ve ever done, which is over 600; every video we’ve ever made; every webinar, poster session, or white paper we’ve ever been involved in has been fed into an AI system, and it creates the ultimate patient lived-experience portal.
We do not believe that there is a larger library of patient experiences than our podcast series. A couple of weeks ago we were at the American Thoracic Society (ATS) Respiratory Innovation Summit, and somebody was talking about, “Oh, we could make a chatbot for a website that can emulate a patient.” This isn’t emulating a patient; this is actually real-world patient experience. I’m proud to say we’ve asked many people to try to stump it, and that hasn’t happened yet. Basically, you can ask questions about pregnancy, about transition, about transplant, et cetera. Again, it’s in native 20-plus languages.
Daily news is brought to you from all across the planet. Trusted organisations like Pulmonary Hypertension News, PHA Canada News, PHA Europe, Alliance for Pulmonary Hypertension (AfPH), and PHA UK. No matter where you are, you’re getting news updated basically daily. This just shows you, when you download the app, how the experience works. You’re going to say, “I’m a patient,” “I’m a healthcare professional,” or “I’m a community supporter.” You answer a few questions. It takes about 30 seconds to put this together: male, female, when were you diagnosed, which type of pulmonary hypertension you have, and some basic demographic information. The same goes if you’re a healthcare professional, just so we know what specialists are using this thing: “I’m a cardiologist,” “I’m a pulmonologist,” “I’m a nurse practitioner,” “I’m in academic practice” or “community practice.” Here I’m just showing you a few dashboard screenshots of some of the information I went over: podcasts, which I mentioned; the six-minute walk test, which you see there on the left; and reports, where your six-minute walk tests will land.
You can find pulmonary hypertension specialty centres. This is US-based. You can find every patient organisation worldwide: email, URL. In the resources tab here, you see another breakout of various other groups related to PH, TBX4Life, and clinicaltrials.gov. We have publications from all the World PH Symposiums, all the guidelines, and videos as well. This just shows you a little bit more. The phone knows where you are once you register. “Oh, I’m from Colorado.” Boom. It shows you all the specialty centres that are in and around that state. In the second screen here, you see the podcast. You can search there by anything you want to search by: by a speaker, by transplant, chronic thromboembolic pulmonary hypertension (CTEPH), paediatrics, and all the ones specific to that need basically show up. Here’s just a screenshot showing you PHA Europe’s newsfeed. On the far right, as I mentioned, is a clinical trials finder. You answer a couple of basic questions and choose a search radius, and it will actually, for all intents and purposes, pre-match you to clinical trials that you’re likely to qualify for, as opposed to just going to clinicaltrials.gov and trying to weed through hundreds or dozens of sites.
As I wind this up, I just want to say this app is more than just a digital health tool. It’s a global call to action for patients to really take control of their patient journey and share in their rare disease experience. With this platform, we’re uniting patients, researchers, and clinicians in a shared mission to accelerate and improve outcomes for the pulmonary hypertension community. Based on a lot of conversations we’ve had over the years, people get information from all over the place and they have to go to this website and that website, and here, there, and everywhere. What we’re really trying to do is go to the best organisations, pull in the best content, and make it a one-stop shop. God forbid someone is diagnosed with this disease: our hope is that when they walk out of that first clinic visit or that first referral, by the time they’re walking to their car, they can download this app and be listening to or reading about this disease, hearing other people who share that experience, looking for doctors, finding a centre, and really connecting with their disease.
I’m going to leave this last screen up here for a second. Again, if you want to learn more about it, it’s on our website, phaware.global/heartworks-app. If you take 20 seconds and take a screenshot, the QR code on the left is for Apple App Stores, and the one on the right is for the Google Play app stores. It just takes, like I say, 30 seconds to register, and we really hope that the community can find this useful. I’ll say in conclusion, I’ve mailed out about … I’ve mailed out about 2,000 of these flyers to various clinics, hospitals, and doctors over the last week. So we’re doing it one support group, one doctor, one hospital at a time. Thank you so much.
Robert Pleticha
Awesome. Thanks so much, Steve. I downloaded the app just as we were getting going today, and I’m excited to explore further in it based on what you told us today. I can also assure the audience that it is as easy to get started as Steve mentioned. Thanks so much for sharing that. Paula is going to present next to us. Dr. Paula Appenzeller is a clinical research fellow at the Royal Papworth Hospital NHS Foundation Trust and the Victor Phillip Dahdaleh Heart and Lung Research Institute in Cambridge. Paula will speak about remote monitoring technologies, and your slides look perfect. Please go ahead.
Paula Appenzeller
Thank you very much for the introduction, and also thank you very much for inviting me to talk, and to the previous speakers for sharing their exciting and new technologies. My talk is a little bit more of an aerial view of why we as clinicians and researchers think that remote monitoring technologies are important, and what possibilities we currently have as a pulmonary hypertension community. So the first question is – and Steve already answered that – what is remote monitoring? We understand remote monitoring as using digital devices to manage and monitor disease outside of the hospital and in a patient’s home and environment. We are trying to see if a disease improves or gets worse, and we can do this by using a smartphone, as was seen in the previous talk, or by smartwatches, also as we saw in the first talk.
But we can also use some more specific medical devices, such as implanted monitors that collect the data. The data is then shared remotely with the clinical care teams, and they can take decisions, for example increasing or decreasing diuretic medications, or upping the dose of pulmonary hypertension medication remotely, so that the patients don’t actually have to come into clinic. The data we collect could be step counts, heart rate, daily weight, specific tasks, or even questionnaires, and we can really use this data to monitor the disease in the same way that we would do in a hospital, just in a remote setting. We might ask ourselves: why do we think this matters? Why is it important? In recent years, and especially since the pandemic, there has been increasing pressure from regulators and from the public to move traditional care from hospital into the patient’s environment and to use digital devices.
I’m going to give you an example that is a little bit off topic, but it’s a perfect example of how digital devices can improve disease management. The example is diabetes, and for people living with diabetes, it’s really important to measure blood sugar levels several times a day and then to titrate and adjust the dose of insulin they need accordingly. Traditionally, people have done this by using finger-stick measurements, so they’re taking their blood, measuring blood glucose levels, and then adjusting the insulin dose. But now we can do this a little bit more elegantly. If we imagine the shaded area on the graph to be the optimal range for blood sugar, single time-point measurements before each meal might look like something like this. We can see, at first, this all looks really well; everything is in order. But what we don’t see is that the blood sugar levels are actually much more varied, and there are some unnoticed highs and unnoticed lows that go missing if we only look at single time-point measurements.
In 1999, the US Food and Drug Administration (FDA) approved a continuous blood sugar measurement device, a sensor on the arm of the patient that will measure the blood sugar each minute, and today many patients have automated insulin delivery systems that rely on these continuous measurements. This is a perfect example to show you how, if we just measure something at single time points, we might miss something and it is not really representative of the whole course of a disease. We can take these examples to many more areas. We could, for example, take normal arterial hypertension, where we have known that white-coat hypertension is a real problem, and patients generally have higher blood pressure if we measure them in clinic or in the general practitioner setting compared to when we measure them at home. That’s also why, for example, in hypertension, we have introduced 24-hour blood pressure measurement at home before we can properly diagnose hypertension, and using digital devices is just an extension of this concept.
So we are using something to monitor diseases in higher granularity and with a little bit more complexity, but it will give us a better understanding. Essentially, what we want to achieve is improved disease management. Digital devices can give us objective data to complement what a patient tells us in clinic, or what a patient feels, or what the symptoms actually mean. They allow us to have continuous monitoring between in-person visits, and they also monitor the patients and the people living with the disease at home, in an environment that they are familiar with and in an environment that matters much more than a clinic environment. So we can see how the disease affects people in their everyday life, and not just when they’re in hospital. Then, of course, using AI and machine learning, which are now becoming more and more popular, we can use all the data we collect and use something called pattern recognition to detect improvements or deterioration early, and then also adjust treatment in a more personalised way.
Last but not least, now that we have the opportunity to collect data in a much more granular way, we will gain a better understanding of how the disease naturally improves or deteriorates and how people with the disease live in their daily lives. So this is more general, but how can we actually use it in pulmonary hypertension? In 2024, the PVRI, the Pulmonary Vascular Research Institute, conducted a global patient survey that some of you might have participated in. We have seen that 93% of patients with pulmonary hypertension have a smartphone, a phone with internet access, and 50% of these already use it to monitor their health in some way. The easiest way to approach digital monitoring solutions is to take something that we are very familiar with and translate it into the digital space. The survey also asked if patients would feel able to complete a digital six-minute walk test outside of the hospital using a smartphone or an app.
Again, we could see globally the answers were really high at 80%, and we could also see that it didn’t really vary across the different regions of the world. Together, this data is very encouraging. It shows that remote monitoring is definitely feasible, and it shows that patients and people are willing to engage with it. It also shows, and we have seen this before, that the six-minute walk test is an easy first step. We have heard a lot about the six-minute walk test in the previous two talks, but this is the concept that is the most developed in pulmonary arterial hypertension. Over the last decade, if we go back to the literature, the pulmonary hypertension community has developed several different versions of a digital six-minute walk test. That means patients can perform the test that they usually would perform in hospital, in hospital corridors supervised by nurses or researchers.
They can now perform it at home in their gardens, in their houses, or in their environments just outside their houses, using a smartphone app or a smartwatch. The distance walked is measured through step-counting algorithms or GPS signals, and it’s stored on an app platform where it can be shared with clinicians. The table on your right gives you an overview of how many groups over the last 10 years have developed some version of the digital six-minute walk test. There are lots of different methodologies behind it. Some are indoors, some are outdoors, some use GPS, and some use steps, but overall, we can say as a community that we’re quite confident that the digital six-minute walk test is something that is safe and feasible, that patients really like it, and that it’s also quite accurate. You can see this on the graph in the middle, which is a correlation graph.
You can see the standard six-minute walk test measured in hospital on the horizontal axis versus the digital six-minute walk distance on the vertical axis. You can see that the points are really close together, meaning that they’re very closely related, meaning that we are quite confident in saying that what we measure with the phones or the smartwatches is what we would measure in clinic when we just counted the laps someone walks. So we can definitely use a six-minute walk test as a monitoring device outside of the hospital. The community has even gone further and is now exploring different versions of the six-minute walk test. Some of them are shorter. We have a digital one-minute walk test and a digital three-minute walk test that are currently being explored. This is based on feedback we got from patients saying that they would prefer a shorter walk over six-minutes.
We have looked into this and we’ve seen that shorter walks actually carry the same clinical information as a normal six-minute walk test, but they obviously have the advantage of being more convenient to perform in a home environment. Again, we have the correlation plot on the right where we can see the different colours – a one-, two-, three-, or four-minute walk test – and how it relates to the normal six-minute walk distance. We can see that they are very closely associated. Another concept that is also currently explored by a group in America is to measure something called cardiac effort. This is used by measuring the continuous heart rate during the six-minute walk test. We divide the total number of heartbeats by the distance the patient has been walking, and it gives us an idea of how intense the six-minute walk test was.
We have seen that this adds meaningful information to the six-minute walk test, and it makes it a little bit more interpretable and also comparable if we want to measure it longitudinally over several weeks in a row.
The group that’s doing this is doing it with this little patch that you can see in the picture. It’s called a BioStamp, and you can see that this little patch is measuring steps and heart rate. It’s an example of how advanced we can be in our digital monitoring, while it really doesn’t take a lot to do so. Another really exciting thing that we are doing is using implantable devices called CardioMEMS, which are paperclip-sized devices that are directly implanted into the pulmonary arteries during the diagnostic right heart catheterisation. They continuously measure the blood pressure in the lung arteries and the resistance, and the group in Sheffield that is pioneering this has been able to show that we can directly see the impact of therapy by using these devices. On the graph, you can see in red the total systemic resistance and in blue the total pulmonary resistance, which is normally elevated in pulmonary hypertension.
This is also the reason why the symptoms are like they are: the higher it is, the higher the symptoms are in general. You can see that the curve dramatically drops down after therapy was initiated, and it stays low while therapy is administered, and it rises again after the drug is withdrawn. So we can use these devices to see the direct impact of drugs on the pulmonary haemodynamics, which is what we define the disease by, and we can also use them to find the perfect dosing and to be a bit more personalised in our treatment. There are many more different examples that I don’t have time for, but to conclude: the term remote digital monitoring covers a wide range of solutions, some of which are as easy as just carrying a smartphone or applying a patch, and the pulmonary hypertension community is currently exploring very different solutions.
Most of them are slowly making their way into clinical trials, although the clinical implementation to be actually used in a regular service is still a little way off. The overarching goal we have to remember is to use digital monitoring to improve disease management and to empower patients to monitor their disease in their own homes. Lastly, it’s very important that any potential solution going forward is developed in collaboration with people living with the disease and clinical teams together, to create something that is both informative and acceptable for everyone involved. With this, I thank our patients for participating in our trials. Thank you very much.
Robert Pleticha
Thank you, Paula. Very interesting to see the cutting edge of remote monitoring right now. With that, I want to invite next, and last but not least, Gregg Rawlings, who’s a lecturer in clinical psychology at the University of Sheffield in the UK. Gregg, please go ahead whenever you’re ready.
Gregg Rawlings
Hi everyone. Thank you so much for having me. The title of my presentation is “Online Mental Health Resources and Peer Support”. Just to introduce myself in case you don’t know me, my name’s Gregg. I have degrees in psychology from the UK. I have a PhD in neuroscience. I am a qualified clinical psychologist. I work two days in the UK National Health Service (NHS), working with adults with a learning disability, and three days as a lecturer in clinical psychology at the University of Sheffield. That’s where I do my research in relation to pulmonary hypertension. I work with colleagues at Sheffield Teaching Hospitals, which is a pulmonary hypertension specialist centre, as well as PHA UK. This is my second time presenting at an Alliance for Pulmonary Hypertension webinar, so thank you for having me again. I presented years ago, where I discussed our work looking to develop a self-help guide, a remote guide, for anxiety in pulmonary hypertension.
This is a self-help guide that can be completed in the comfort of your own home. It’s based on a type of treatment called CBT, or cognitive behavioural therapy for short, and, as I said, it helps people with pulmonary hypertension manage anxiety. If you’re interested in hearing more about the programme, there’s a YouTube link underneath, and if you are interested in the programme, there’s the link above. I suppose the reason for that work, or one of the things that helped reinforce the need for it, is this review that was published a few years ago, looking at rates of anxiety and depression in pulmonary hypertension across North America, Europe, and Asia. It involved over 2,000 patients, and what they found was that rates of anxiety and depression were much higher than what we’d expect to see in the general population. It was around 37% for anxiety and around 20% for depression.
For comparison, we’d expect to see rates of around five to 6% within the general population. So, much, much higher in pulmonary hypertension. This graph in the review is particularly important, at least in my view. This is looking at predictors of anxiety and depression in pulmonary hypertension, and I just wanted to draw your attention to the information in this red box. This is looking at severity of pulmonary hypertension symptoms as indicated by the World Health Organisation (WHO) functional class. This is the idea where functional class three and four is more severe in terms of symptoms compared to one and two. What you’d expect to see is that World Health Organisation functional class, so severity of pulmonary hypertension symptoms, is a significant predictor of depression and anxiety, when actually that’s not what we found. For it to be significant, these values here would have to be less than 0.05.
So this would suggest that treatments that aim to address the severity of pulmonary hypertension, as measured by the World Health Organisation functional class system, may not be the most helpful. There’s something else going on here that might account for why rates of anxiety and depression are so high.
The way that we can measure symptoms of depression is by using screening tools, and these are self-reports that can be completed anywhere: in clinics, via apps, or online. This is one of the most common methods of screening for depression. This has nine items that someone would respond to over the past two weeks, looking to see how often they experience some of these symptoms related to depression. The score can then help us to understand whether they’re likely to experience depression or not on a clinical level. It is only a screening tool; it’s not a diagnostic tool. However, it is a relatively easy measure to administer and complete.
Some of you may be aware – someone has already mentioned the global pulmonary hypertension survey that was published this year. This was a survey involving over 3,000 patients from 88 countries. On the right-hand side, you can see the percentage of people experiencing, or who endorse, some of these experiences. So 27.5% of people reported low self-esteem, 26.5% reported anxiety, fear, and so on. This led the authors of that paper, of the survey, to conclude that psychological support is a highly requested but frequently unmet need, reinforcing the importance of holistic care models. So, recognising that rates of anxiety and depression are high in this population, and recognising that there are multiple barriers to accessing support, we’ve already developed a resource to help people with anxiety. What we did recently was develop a resource that people can complete at home, with self-help based on cognitive behavioural therapy, targeting depression in pulmonary hypertension.
This paper is now published and the resource is available; I’ll share the link later on. What this resource is looking at is four booklets over four weeks, and each booklet corresponds to different topics. The first one, for example, is looking at depression and pulmonary hypertension, helping people to understand the symptoms of depression and how it might interact with pulmonary hypertension. Moving on to week two, booklet two, which is looking to replace inactivity, this is a key aspect of cognitive behavioural therapy for depression. It’s known as behavioural activation. It’s around helping people to change behaviours in the hope that it will help improve mood, and then what we might find is that it feeds into more positive cycles. What we’ve done is tested this intervention. As I say, it was self-help, so people could complete it at home. We had 68 people take part, and we randomised people to one of two conditions: one where they received the intervention, shown by this blue line, and one where they were in the control group.
This is where people were allocated to a wait-list condition, so they didn’t receive anything during that period. We assessed depression before the intervention, after the intervention – so only four weeks later – and at one-month follow-up. We asked people to complete the PHQ-9, which I showed earlier. This is what we found. Both groups scored moderate depression, and using the PHQ-9 we can look at the clinical cutoff. This means that anyone scoring above this cutoff may benefit from speaking to a healthcare professional and receiving additional support. What we found after the intervention was that those in the cognitive behavioural therapy group reported a reduction in their difficulties related to depression, and at one-month follow-up that was maintained. We also asked people who took part to complete the emPHasis-10, which is a health-related quality-of-life measure. It’s 10 items asking about difficulties related to pulmonary hypertension, particularly around breathlessness, for example, or fatigue, or some of that other psychosocial impact.
So, “Pulmonary hypertension does not control my life,” “I’m independent,” “I never feel like a burden” – those were some of the questions that were answered. Again, we asked them to complete it at baseline, post-intervention, and follow-up. As you can see here, those in the cognitive behavioural therapy group, interestingly, didn’t score much difference between baseline and post-intervention. However, at one-month follow-up, they scored an improvement, and this was a statistically significant improvement. We obviously have to bear in mind that it is a relatively small sample size, so more research is needed. Nevertheless, the evidence is promising. As I say, our intervention shows promising evidence that it can help with depression symptoms, but also help with quality of life. We also asked people what they thought about the intervention, and the majority valued that it was specific to pulmonary hypertension. The majority reported feeling more in control of their depression.
As I say, relatively small numbers – 15 people responded – but nevertheless, it’s still promising data. People with pulmonary hypertension thought that other people with pulmonary hypertension should have access to the intervention. A similar amount said that it helped them a lot and that they’d recommend it to others, and there are some quotes there. People are talking about how the self-help intervention gave useful strategies to help maintain their mental health and make sure they are doing what they need to support their physical and mental health. As I say, just like the anxiety intervention, the depression intervention is now widely available via PHA UK. You don’t need to access this through your pulmonary hypertension provider or pulmonary hypertension service; it can be accessed remotely. This also contributes to the other forms of remote support that PHA UK can offer, namely the listening line. This is a telephone line where patients with pulmonary hypertension can ring and receive support from someone with knowledge of pulmonary hypertension.
It’s not counselling or therapy, but it’s someone with expertise in pulmonary hypertension, in addition to “PH Connect”. This is an online platform where people impacted by pulmonary hypertension can access and find other people who have shared experiences, whether it’s to do with medication, type of pulmonary hypertension, gender, age, or ethnicity, and reach out. That’s where you can have that connection and that peer support.
As I say, we’ve developed a resource for anxiety and a resource for depression, and they’re both remote and can be accessed as self-help. What we’re currently developing at the moment, and the intervention has been developed and is in the process of being made available, is an intervention that we’ve developed looking at sleep in pulmonary hypertension. This comes on the back of our research, but also some of the research looking at the high rates of sleep difficulties in pulmonary hypertension. For example, recently we interviewed 111 adults with pulmonary hypertension. We asked them to complete validated measures of sleep. Eighty percent were classified as having poor sleep quality, around a third reported difficulties with daytime sleepiness, and around a third reported clinical levels of insomnia. So our intervention looking at sleep will be available again with the aim of being remote and self-help. Thank you very much for your time.
Thank you for having me again. I’d like to thank everyone who took part in our research, and PHA UK and our collaborators. Thank you.
Robert Pleticha
Thank you, Gregg. Really appreciate it. If the other speakers want to come back, we have a few questions if you don’t mind answering them. I think the first one was for Ciara. How did patients do after the programme? Because she hears that it’s hard to keep doing the exercises without a little nudge after rehab.
Ciara McCormack
That’s a really good question. As probably many of us have experienced, sometimes when you’re not being followed, it’s easier not to do something than to keep it going. One thing that we did find – we have done follow-up, and we have some follow-up data to be published on this – is that many patients did actually continue to be physically active, but maybe not as highly active as they were during the intervention. What they found useful was that they still had access to the resources and the tools that they developed during the intervention, such as how to monitor and self-manage, which were really useful tools for them to keep using when they didn’t have the health coach and support. However, a lot of them mentioned that it would be useful to have ongoing check-ins. I suppose, from a research perspective, we are trying to explore the best options and what’s the most impactful and best way to continue to support, or to taper support, after being involved in an intervention that is monitored quite intensively.
So it’s very much dependent on the patient and individual. I suppose the goal with any intervention is to try as much as possible to help the individual become self-motivated and to self-manage as well. But I do think there’s a really important role that clinicians play, particularly when it comes to exercise and physical activity, in ensuring that they are at least asking patients how they’re getting on, if they are being physically active, and if they need more support. It’s a million-dollar question: how do we keep patients engaged, and how do we support or offer more support for people who may need it? Everyone’s very different, and it can be at very different time points as well, depending on maybe where they’re at with their pulmonary hypertension.
Robert Pleticha
Thanks, Ciara. Steve, I see you had a few questions in the chat that you kind of already responded to.
Steve van Wormer
Yes, I can articulate a little better. Someone asked, how do we keep the data relevant? Not just in our app, but on our website, on our homepage, there’s a vendor. It’s called Match. It’s a matching tool. Basically, if anybody has ever gone to clinicaltrials.gov, it’s just a web of all kinds of stuff, and there are dozens or hundreds of various studies going on, and the dates, and how you qualify, inclusion, exclusion. What our match tool does is basically preset for pulmonary hypertension, but you could set it for anything, or Chronic Thromboembolic pulmonary hypertension or whatnot. You answer four or five basic questions, you put a search radius, and based on your questions, it basically pre-matches you to studies that you’re really going to be qualified for potentially, as opposed to just randomly picking 20 trials. But as far as the dates and relevant information, it’s tied directly to clinicaltrials.gov.
So if something goes offline, or it’s filled, or whatever, it’s going to go away. But this platform just makes it a little cleaner and easier to navigate. Another question that was there in the chat, which I answered, but I’ll state again, was: does our HeartWorks app connect to other phone apps? In the case of iPhone, which I’m an Apple user, yes. Right when you log in, it will ask you your height and weight. Some people have asked me, “Why are you asking for my height and weight?” It’s because that’s if you are going to want to connect to do the six-minute walk test or connect to your Apple Health app. So it works in concert with that and with whatever the relevant version for Android devices is. I don’t know what it’s called on Android. I’m sorry, I don’t have an Android device.
I think those were the two questions that were for me, unless there’s…
Robert Pleticha
Thanks for adding that other colour to it, Steve. Those details are appreciated. Paula, the next couple of questions are for you. Suzanne was wondering: do you find that people can be more honest about their symptoms, et cetera, using a third-party tool?
Paula Appenzeller
This is a really good question, and it might need to be forwarded to Gregg as well. We have done some work on questionnaires, and what we notice is that we can see a little bit more change when we use questionnaires more regularly and people actually have to fill them out, not only once every six months or once a year, but every two weeks. In terms of what the watches are measuring, we don’t really ask for symptoms, but we can definitely see what people are doing. So in that sense, if you decrease your activity or increase your activity, the watch is just measuring what you’re doing. You can’t sort of lie – or you can … So that might be a way to be more honest. But with the questionnaires, maybe Gregg has an answer, or maybe you have done some work on this, to see if people give some more information if they’re using a tool rather than a person.
Robert Pleticha
Gregg, anything to add?
Gregg Rawlings
I haven’t done the research myself, but obviously it’s worth bearing in mind that if it is a patient-reported outcome measure to begin with – the questionnaire or way of collecting data – and you develop a new way of administering that or collecting data, it’s important to make sure that’s still validated and you’re still collecting the same data. So yeah, that’s just something to add.
Robert Pleticha
Thanks. Paula, are you able to compare the results of the six-minute walk test in hospital versus remote ones? That seems to be the biggest hurdle, to compare them.
Paula Appenzeller
Yes, that’s spot on. We are able to compare them in terms of, if we do them in a study and we measure what we measure on the ground and we measure what the watches measure or the phone measures, they are really highly correlated, so they’re really basically the same thing. It gets a bit more difficult when patients take the tool home and then start doing their tests at home, and what we can see is that the test, the difference, or the distance walked varies much more than what we would expect on a week-by-week basis. Most of the studies that have been done have just been showing that, at a single time point, when we measure it remotely and we measure it on the ground like normal, they are really highly correlated. There’s not that much data yet on how to continuously measure them over months at home, but data is coming in. We are collecting more digital device data and we are finding solutions on how to sort of overcome this problem. It’s not a big difference. We can still deal with it. So I still think this is a really good tool to use. But yes, it is a bit of a hurdle to compare them directly.
Robert Pleticha
Next question is about CardioMEMS. Please go ahead. The procedure.
Paula Appenzeller
How to insert a CardioMEMS? This is the small device that sits in the pulmonary arteries, and this is inserted during your right heart catheter examination. Normally, when you have the normal diagnostic catheter, you can insert this tool. It has two little clips on the side that will anchor itself into the pulmonary artery walls, and it swims in the middle. So it’s not a big procedure, and it’s proven to be really safe. It’s been mainly used in left heart failure, and it has been used for a couple of years there and really well explored. The team in Sheffield is doing a lot of these, so if someone’s interested…
Robert Pleticha
Great. The next question is for Gregg. Is there a difference in people who get treated with IV treatment versus oral therapy, because they always live with the knowledge that when the infusion stops, they have minutes, not hours?
Gregg Rawlings
I’m not sure if I understand the question fully, but I suppose in terms of my research, we don’t differentiate in terms of who is eligible for our interventions. So it’s all forms of pulmonary hypertension, regardless of type, functional class, or medication. Our inclusion criteria are very open. There are some limitations. For example, we won’t accept people who have got thoughts of suicide or self-harm, recognising that realistically self-help might not be appropriate. So we signpost them to their clinicians to have those conversations.
Robert Pleticha
Thanks, Gregg. Another question for you: can the programme be ordered from outside the UK as well?
Gregg Rawlings
I believe so. If you reach out to PHA UK, you’ll be able to have those conversations with them and see whether it’s something they could post out or make available via a PDF, for example.
Robert Pleticha
Great. We will make this entire webinar, as well as the four presentations that were given today, available on the Knowledge Sharing Platform and on the Alliance for Pulmonary Hypertension YouTube channel after this. I just think it was an incredible discussion today looking at technology, AI, what the future holds, and the fact that maybe a lot of these tools can help people better manage their pulmonary hypertension. With that, I want to thank you all for attending. We really appreciate your questions and your participation, and also thank you to our speakers. We couldn’t do this without you sharing your knowledge, and Gregg coming back even a second time. Hope to have some of you back other times as well. Thank you so much for your time.