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Hello, I'm Kelly Chen from UT Southwestern in Dallas, Texas, and today we'll be talking about rethinking pulmonary arterial hypertension. My disclosures are here, mostly related to research work in pulmonary hypertension, either on steering committees or clinical research that we do at our institution. All right. Starting with myth 4, escalation can wait until worsening. So the learning objective here is to develop personalized treatment plans that integrate guidelined directed therapy, pathway-based rationale, and patients specific factors to optimize outcomes. So first with a question. In patients with confirmed PAH who show partial improvement on initial dual oral therapy but continue to have intermediate low-risk features as shown, which of the following best reflects guidelined directed next steps to optimize long-term outcomes? A Continue current therapy unchanged as long as symptoms have improved. B. Delay escalation until clear clinical worsening or hospitalization occurs. C. Switch both oral agents to alternative drugs within the same classes. D. Discontinue one agent to evaluate whether symptoms worsen, and E, perform comprehensive reassessment and consider escalation to add-on therapy. All right. Let's start with the case. So, this is a 42 year old woman who I met some time ago. She has a history of rheumatoid arthritis on infliximab and she comes in with dyspnea. She's able to do her ADLs and shopping, but she has dyspnea at faster pace walking or with stairs. So she undergoes a workup starting first with the dyspnea eval and then working into pulmonary hypertension when nothing's found on her, um, lung evaluation. She also has a normal BQ. Her echocardiogram suggests pulmonary hypertension and she's referred for right heart catheterization, which is shown on the right, and this confirms PAH. She has a PA mean of 48, normal cardiac output with an index of 2.6, and a very high PVR at 8.7, as is pretty typical of newly diagnosed PAH patients. She initiates guidelined directed therapy with oral combination therapy with a PD5 inhibitor and an ERA, and she undergoes follow-up 6 and 12 months later. She tells you she's feeling better, and you can see here her functional class, 6 minute walk distance, and NTro BMP. Her functional classes improved. Walk distance has improved as well, but just a bit, and her NTPro BMP, um, initially improves but then loses ground. So where do we go from here? She's doing OK. She's not declining, um, she's even a little better. So, the best next step is to do a formal risk assessment using a risk calculator. And there are two main risk calculators that I use in, in my practice and that have been um comprehensively developed. The first comes from the ESCERS guidelines and sometimes goes by Compera, which is one of the registries where it's developed. And we'll use it to start with. Um, it is quick and easy, and I have memorized the low risk criteria, so very quickly, I know when looking at a patient if they're low risk or not. She is functional class 2, but her walk distance and NTrobiumP are above these thresholds which put her into the, if you average this, which is how this works, into the intermediate low-risk range. We could also use Reveal, and on Reveal, she has a slightly low systemic BP, and that gives you a point which puts her into the intermediate risk range. There, it also uses functional class walk and and Tro BMP. So, now, um, I have some concerns based on her non-invasive testing, but I need a heart catheterization and an echocardiogram for a more complete picture. And these are needed because these non-invasive tests sometimes underestimate overall severity, I think particularly in young patients who really would be Normally having a much higher walk distance and much lower NTR BMP and then also sometimes overestimate because non-pulmonary hypertension risk factors, comorbidities, obesity, arthritis, so on can also affect these non-invasive measures. So, on echocardiogram, we got a lot of information. We're very focused on the right side of the heart and I'll show the um report information that we had and then we'll take a look at the pictures in a minute. So she has a baseline echo, a six-month echo, and a 12. A month echo. And it's kind of a mixed picture. So I think you could look at this and conclude, well, some things got better and some things not so much. Maybe we're doing OK here. But in reality, this is a very much not at all OK echocardiogram. The pressures did get a little bit better and the report on the function is that it improved. But you have to be very careful with RV function. It is dependent on whether there's significant tricuspid regurgitation, and if you have a lot of that, and she does, then so much what's going backwards that in a sense you get an overestimate of overall function. And the most important and concerning thing on her echo is size. Her right atrium is very enlarged, which is highly concerning. And her right ventricle is enlarged and getting better and in fact, um, at the very bottom, we get a, a comment on our reports, how does it compare to prior and the reading cardiologist says this is getting worse, the, the RV is getting bigger. What does that look like? Here is her echocardiogram. We have right atrium, right ventricle, left atrium, and left ventricle. And you don't really need much more than that to say this is not at all OK. Just very, very large right side of the heart. Which is a bit discordant from her um walk test and NTRBMP but we do sometimes see that in younger patients. On heart catheterization, um, at baseline, we've already looked at that, severe pulmonary hypertension. 6 months looks very similar. Her pressures and PVR had not improved very much, and then by 1 year, we have the highly concerning finding of a drop in cardiac index where it has, has come down, um, below the borderline low of, um, 2.6 and 2.7 that she had. Putting it all together can be a little bit tricky because it's not always clear how to adjust points for um the imaging in particular. There's a lot of different things to look at, but at the end of the day, unless the heart size and function are both completely normal, a patient is not at low risk based on their echocardiogram. So, this is the ESC ERS guidelines bigger table with All of the different ways that are commonly used to assess risk and um based on this, this patient would be at at least intermediate risk with the echo showing some very high-risk features. So next steps, um, prior to that catheterization, she had started having some fluid retention, which is also a concerning finding. She was started on diuretics. We, after the catheterization was done and saw the overtly low cardiac index, started talking about parenteral therapy, which is what we were recommending, but discussed options and She was still thinking about it, interested in probably trying an inhaled prostacyclin. This was some years ago, so these were the only options available at that time. And prior to initiating anything, she had a very severe decompensation. She was admitted um with fast heart rate, with some um SVT volume excess and um required ICU admission to the point that um urgent initiation of an IV prostacyclin was undertaken. So how did she do from there? Well, I've been following her for a very long time. I actually recently saw her for her 15-year follow-up where we had done another catheterization. We hadn't done one in some time. She's been doing so well. You can see where we initiated IV therapy, what her PA pressures and PVR did from there, um, big drop in PA pressures, PVR came down well under 5, which is one of our goals, and her cardiac index normalized. On echocardiogram, you can see on the left, the one just before IV therapy. This was about a year after IV therapy. Still some right-sided dilation. We did some initial, some additional up titration of her IV therapy. And then this was her echocardiogram at 15 years. And it looks pretty good. Um, the RA and RV are still slightly enlarged but not very big. Interestingly, she's now 62 years old and starting to show some left-sided changes. Her left atrium is a little borderline and she's got some mild to moderate mitral regurgitation. So, how did we get there and what would we do different today in this patient? Well, so she did get upfront ERA and PDE5 therapy, but we were a bit slower to add a third agent. Um, so we went from here to This, I think, could have been considered for sure at 6 months. She had not achieved low-risk criteria and maybe we would have avoided the decompensation. So take points from this case are that intermediate low isn't truly low risk, but lower relative to other patients. Non-invasive testing provides our best assessment of one year.