Chapters Transcript Video The Long and Winding Road: The Continuing Mission to Educate Our Region on Pulmonary HTN Click here to view presentation deck Course Director Dr. Michael Eggert discusses the essentials for PAH diagnosis and treatment. Why is it still a long and winding road? Well, I kind of alluded to that and we're going to um what is it, spill a little tea and throw a little shade um at some of the guidelines. Um Luckily, I have national level experts to allow me to say that yes, we need to know the most recent sets of guidelines which are derived in 2018. And you know, from the sixth world symposium on pulmonary hypertension in 2018, as well as revised guidelines from the European Respiratory Society and European Society of Cardiology um in 2022. And hopefully, hopefully, you know, Moses will come down from the mountain in 2024 and Barcelona and come down for the seventh World symposium and actually managed to put all this together in such a way that it makes more sense than kind of the melange of stuff that we've got now, um which is kind of about the best way I can put this. Now, let me put myself on a stopwatch, which often when I get to speaking is uh kind of the trouble is getting me to stop speaking. Um As some of my friends may attest these are my disclosures. So the outline of this and I wanted to print it out because I wanna come back to it, but I forgot to do that. So here we are. Um So ph, what is it? Um Ph and Pah are not synonymous. Um If I can help anybody who's beginning their journey in this process to understand that that'd be a great thing. Why do you care about Ph and Pah, you know, if you diagnose it, you know, does it matter? And what is the tempo and trajectory of the disease process? Are there different forms of it? Um Yeah, there are different forms of it as, as begged by the next next point, which is that it's still lethal. Um How's this any better? Because I got annoyed with that little chiming sound. Um So, and, and just to make sure that everybody remembers that it's not just vaso motor imbalance, facilitating this disease process. There are multiple things that will do that and I'm not going to uh actually the last part, I don't want Doctor Wing mad at me for treading on her excellent management talk. So I'm not going to necessarily tell you all about that, but I am going to hint at it a lot of the diagnostic maneuvers that they, that are suggested as part of the recent guidelines are kind of following likelihood tables and parameters, you know, do you have certain echocardiographic changes? Do you have certain symptom complexes? Do you have, you know, so on and so forth and it's still a hard diagnosis to make. We are still at times a year out from patients symptoms, two years out from patients symptoms. And by the time they get to us, um, you know, they are often, you know, uh pretty far down the road in terms of their disease process and they are easy diagnostic screening tools in this day and age. We'll talk about the gold standard for diagnosis, um which was called by a former pulmonologist who was president of the, of the American College of Chess positions was called by him at one time, a historical artifact. The tales of his death are greatly exaggerated and then we'll talk about treatment teasers, but I don't want dr twang to chase me. So we will move on to America's story. Um This is uh this is a patient, it's not the story of our country, uh which is also somewhat still long and winding, but America 27 year old nurse from across the water. Now, when we say across the water here, obviously, we're talking about the Hampton Roads bridge tunnel and not a larger body of water. Um She is new to our practice and came in uh in the setting of increasing shortness of breath and the recent birth of a two year old daughter. Um and she had already had multiple medical encounters. Um Her history is of more shortness of breath over the past year, um which she thought was related to just postpartum weight gain. Her stressful job as a new nurse and her toddler, her legs and abdomen just got bigger. Her past medical history is remarkable for psoriasis and arthritis. Nobody's, she was undergoing testing for psoriatic arthritis. But I think that's what she's going to end up having. She has systemic hypertension on one controlling medication. Her BM I shows class one obesity. She has no family or medical history for sudden cardiac death or thromboembolic events and very kind of a very cute thing I snore but I don't need one of those machines. Why are you asking me that? Um I couldn't possibly have obstructive sleep apnea? Um Anyway, so I say an embarrassment of riches with the new guidelines on the left is obviously the sixth world symposium uh kind of symbol. And then the guidelines from the European Society of Cardiology and the er s published in August of 2022 these two things build upon one another. So if you haven't kind of read them in sequence, it's very challenging to figure out how to use them and they end up reading like IKEA furniture assembly instructions. Um they're very challenging to use. And so this is, this talk is more about concepts and not necessarily kind of numbers as cut offs, but more about what is Ph what is Pah, first of all, what do you, what do you think about when you hear about Ph well run? Ok. No, don't run. Um It's not that bad. Um You think about, you know, fundamentally, pulmonary hypertension is increased pressure in the pulmonary vascular bed. Now, that can be on the pulmonary arterial side, that can be on the pulmonary venous side. But fundamentally, you should be thinking about increased pressure in the system. Then depending on the type of pulmonary hypertension that you have is the resistance through part of the pulmonary circulation. Again, either pulmonary arterial or pulmonary venus, pulmonary arterial being much more likely than pulmonary venus. Although the possibility of pulmonary venous resistance is is increasing. And what is the clinical context? Because the guidelines, what they are asking from us is to match the physics and I know physics at 8:10 a.m. it's a bit much but to match the physics of what's happening in a system that has a certain flow and therefore a certain amount of resistance to flow naturally may be more resistance to flow because of distortion of the system. And then in what clinical context is that happening? And when you put the two of those things together, all of a sudden the process starts to make sense and you don't have to worry so much about artificial guidelines and numbers and how these things are designated. So, so what is the definition now? The first thing you're going to note is that when you go out and talk to people about pulmonary hypertension, they may tell you, ah, yes, I diagnosed my patient with pulmonary hypertension based upon an echocardiogram that showed a, you know, estimated RB systolic pressure of 50. Well, no, you didn't because it's always based upon a cat. It's always based on, uh, a right heart catheterization where pressures are assessed invasively. Um, but PH is simply a pressure elevation pah, which is a disease that we all have some experience with. Um it's a disease resulting in restricted blood flow through pulmonary arteries due to increasing resistance, the increasing resistance that the right heart is forced to push against to move blood and flow it through the pulmonary vascular bed, it overloads the RVS work capacity. Now, I'm gonna say this again. Obviously, the RV is not designed to the RV, myocyte at from the time of embryo logic differentiation are not designed to help construct a, a vigorous system. This is a system, it's a low power. The RV is a low power pump. It's not supposed to pump uphill, it's not supposed to pump against increased resistance. So what, so this is a historical definition. He and hemodynamically of PAH from 2018 where you see mean PAP or mean pulmonary art of pressure as defined by right heart catheterization of 25 millimeters of mercury, a wedge pressure of less than equal to 15 millimeters of mercury and a PB R of greater equal to three wood units, which is one way to measure it. So our European colleague got involved and they didn't. Then again, II I say this, you know, somewhat, you know, kind of somewhat poking at them. There were reasons for doing this but um I I it it really does take a disease of resistance and kind of start make you start to scratch your head. So the mean pressure at which we now call or which we can make a diagnosis of pulmonary hypertension is now 20 millimeters of mercury. The wedge has remained the same while the PB R has been lowered to greater or equal to two wood units. And some would suggest that gosh, are we going to make this into make pulmonary hypertension or pulmonary arterial hypertension? Uh theoretically a you know, at least an unusual if not rare disease into more of a public health problem. Um I don't believe so, but you know, you do start asking questions about why there is such a change? Why we allow for less pressure and a reduction in the resistance of gosh a third. So why were the, why were the hemodynamic definitions of pulmonary hypertension refined well in resting healthy individuals? The normal mean pulmonary pressure is about 14 plus or minus three and the upper limit of normal beyond the upper limit of normal weight, you know, two standard deviations from the from the mean is about 20 millimeters of mercury. And, ok, that's fine. But what's in a number? And is it relevant to your patient's outcome? And can you capture it this year? At chest? There was a procon debate between Doctor Raha from Cleveland Clinic in Florida and Doctor Hat Farber who's uh has been in pulmonary hypertension. Um gosh, since probably I was in college and they had this wonderful debate where um they debated exactly this question. Where are you ever going to get a drug manufacturer or a pharmaceutical company to look at the difference in patients who were captured when their PV R was two versus when their PV R was three? And look at how they did uh over time, how easy is it to capture someone with a PV R of less than three? But a PV R greater than two in terms of, are they preclinical, do they show up? Um you know, obviously from some much earlier data in the PH world, we want to treat this disease as early and quickly as possible. But can you find these people, are you ever going to be able to validate these numbers? Are they more than a statistical, are they more than a statistical in uh uh are they more than of statistical interest? And so, you know, the munch scream is definitely I think appropriate here, which is why I don't want, you know, there's a bunch of numbers here which I don't necessarily want you to spend a lot of time on, but I want you to think about pressure. I want you to think about increased pressure in the system facilitated by an abnormal alteration of the pulmonary, the precapillary pulmonary arterial system which to maintain the same flow is gonna require that the pump. In in this case, a pump that is a low power pump and not designed to function uh as we need it to in this case, why that pump is gonna have to work harder and generate more pressure. So this is one of the, this is one of the survival curves from which the new recommendations were validated. And you'll see that the even a mild elevation of pulmonary pressure is a predictor of mortality even as low as a mean pap of 17. And you can see the statistical cut points there. Um I mean pulmonary pressure of 17 to 26. Uh and I'm gonna see if I can use the pointer here and get ambidextrous. Uh Let's see right here. You're gonna see that even 1726 makes a difference versus grade 126. So you know there is a reason for doing this, but the issue is can you test whether or not you can you there appears to be a mortality difference here but can you capture the patients prospectively and then treat them? Um Doctor Farber did not feel that that was the case and I think a lot of us tend to agree. So in addition, can you establish consensus? You know, these, these guidelines came out and consensus had been established amongst the people present? But I don't know that there have been any real world vetting. We talk a lot of the literature and pulmonary hypertension talks about real world practice and what's happening on the ground with the patients and how they're being treated and there hadn't been any real world vetting of these new guidelines. And so there is, there is much work to do as a new better work. Um decreasing the guideline. Um the PV R for PAH, do we still think this is the disease of resistance? Well, obviously that's pejorative but and it is a disease of resistance but taking that PV R down, that seems a bit extreme. The same thing for a mean PAP for Ph again, is this disease still a disease of pressure? And as it, you know, as it relates to fluid dynamics. In addition, something I didn't even really talk about is exercise induced pulmonary hypertension. There is a formula for that within the new 2022 SESC guidelines. Has that been clinically validated as meaningful to a a process that we can look at in our clinics and in our offices perhaps within scleroderma. But where does it fit within diagnostic and therapeutic algorithms if you see this? What do you do with this? And I think it adds a level of, you know, it adds a level of poten it, it, it increases your knowledge base. But again, I, I have some problem with knowing what to do with it and I'm happy to pause for a second and, and wake up my expert panel and say, does anybody know what to do with the new definition of exercise, induced pulmonary hypertension? Does anybody use it as yet? Mhm. Say that it's an abnormal response to exercise? That's all I think I can say. Yeah, that typically we had, uh, really treated it a little bit more as unmasked group one rather than its own separate entity. I'm not really exactly sure what to make of the formulas and cut off. And I think that the, you know what Dr Elling was saying about, you know, it's an abnormal response to exercise. Does that qualify it as, you know, as a form fruster group? One? I have a, I abandoned that and I don't know what to say about it other than it is, it is an abnormal response to exercise. You know, the way I look at it is, um, we all think of it. We all have been thinking about it since I think. When did they have the last, um, definition of exercise that was in 2013 or before, before that. So, in this period of time, since the last definition before they took it out of the who or wsph whatever they want to call it back then was um that at the, at least then everybody was thinking of it as the same thing. So I'm not sure of just putting something out there as a kind of a foundation to see where we go more like a working formula, not a living formula that can now change, but just to start somewhere. That's how I look at it. Maybe hypothesis generating. Yeah. And I think, you know, there's data, I think even scleroderma, right? And that these patients that have exercise induced ph have a higher risk of progressing. Actually, that's real deal, precapillary p you know, ph at rest. And so, yeah, I think it a it's a good start. Um You know, I think personally in terms of therapy here. Uh Yeah. You know, what, what do you do? I think uh in symptomatic patients oftentimes we try, you know, therapy to see if it makes a difference. But um you know, I think more so it's, it's hypothesis generating. It maybe starts to raise some alarm bells. Yeah. And II I, what Doctor Kang said, I, I, especially in square derma. Given that population's notoriously poor illness trajectory. I think that there, you know, it could have some value outside of that. I agree with everyone else where it's, it, it, you know, it's a signal but as far as what is a signal of, I'm still kind of struggling with that. Um All right. So moving on. Um So Poo's law and this is actually helpful and no, it's not a fish. Um which is why I put the dolphins down there because they are also not fish. But, but what I wanted to kind of put, put this out there as is as resistance increases within a fluid system, either due to a smaller radius lumen or a higher viscosity fluid. If you a greater pressure is needed to get to the same flow. And that's what the diagram shows, right. So if if flow is flow is on the y axis and pressure grade and change in pressure is on the X axis then and this kind of looks like for the fellows a bit like kind of thinking about peep um pressure gradient, the higher the resistance, the more the pressure will have to change to try and get to try and increase the flow. OK. So again, as resistance increases, more pressure is gonna need to be exerted within the system to have the same flow. OK. Which is why you know obviously that you're going to the right, the heart is going to try to maintain the same cardiac output and index pressure in the system will have to increase if the vessels are undergoing some sort of process that is making the flow through them slower because of increased resistance. OK. That's it. If you, if you understand that it makes a lot more sense, this is another way of saying it. So this is a, this is a table I took from the er sesc guidelines and kind of improved it. So, and basically what shows again is that ph is just what it says? It's a pressure elevation, not anything else, not anything, not anything harder, softer, easier, more difficult, it's just a pressure elevation. It's not diagnostic of anything because the pressure elevation can be precapillary or it can be postcapillary. It's, is it now if it's precapillary, is there, is there a resistance issue? So let me get my handy dandy here. So is it a oops, it was handy dandy? There we go. Ok. So is it a, is there a resistance issue here? And is the volume status normal if that's the case, that is probably something like pulmonary arterial hypertension isolate, postcapillary, pulmonary hypertension? That seems like a mouthful. Well, that's easy. There's no resistance there. But when I said no to a normal volume status, all that means is there's a lot of fluid without resistance and the pressure is high. That's just a bunch of fluids sloshing around in the postcapillary, pulmonary vasculature. And where do we find that heart failure of any sort? This can be from that. This can be from a hypertrophied left ventricle. It could be from micro stenosis. It can be from aortic insufficiency. It doesn't matter. This is just very simply diastolic dysfunction. This is just very, simply a lot of fluid sloshing around now this particular entity, the last one is a little more complicated where, you know, it's the same as above, except there is an incr there is a resistance component introduced. And if you find as you're trying to classify what disease process you have that you have a little resistance and the and the pressure is high and the amount of fluids high that, that should get an expert consultation. And so again, pressure resistance and what is the volume status of the patient are key concepts in trying to classify what you have in front of you. And the next slide will help. So, you know, isolated precapillary, pulmonary hypertension or postcapillary, pulmonary hypertension right here. Left heart disease. Ok. It's common ph associated with lung disease. Pulmonary hypertension is not unusual, severe pulmonary hypertension is more unusual and so that shouldn't necessarily say common, but these are the slides that er SESC had available. So I present them for your edification. Pulmonary hypertension associated with pulmonary artery obstructions in terms of chronic clot, otherwise known as CTF or chronic thrombolic pulmonary hypertension is more rare. Other things can also obstruct the pulmonary vasculature and are outside the scope of this talk group five as in 12345 P with unclear multifactorial mechanisms. It's called rare. Well, I don't know that I call it rare so much as unusual. Um kind of like uh I'm sure most uh centers with a wealth of patients of color as well as non caucasian patients. I certainly have a wealth of patients with uh sarcoidosis as well as hemoglobinopathy to include sickle cell disease in whom we find elevated pulmonary artery pressure with some evidence of increased pulmonary vascular resistance. Um who I would be happy to classify as group five. So I don't think those are rare. The rare birds are, in fact over here in group one and are the groups where we see increased resistance with a normal volume status and increased pressure. These are, these are the more rare birds who probably deserve treatment with our, with our kind of different Pharmacopeia. So again, this is another way of looking at it. This is just a a cartoon that makes it easier to kind of understand where the process is. So again, this area here, precapillary, pulmonary vasculature, this is where pulmonary arterial hypertension is occurring. This is at the gas exchange space. So this is where interstitial lung disease and pulmonary fibrosis are occurring. So this would be kind of group three's area. Group three is diseases of, let me just go back. So group three here associated with lung disease and that's not just lung disease but also diseases of impaired gas exchange. Uh So, obstructive sleep apnea or severe sleep related uh or sleep related breathing disorders would also go here. Um And then this most important group, which is group two. Again, this is all this is over here in terms of increased pressure is simply just a lot of fluid sloshing around, not a lot of resistance. Um pressure is increased volume status may be increased. And so, you know, when we get referrals to our PH program for, you know, 787 year old gentleman with aortic stenosis and a valve vary of 0.5 centimeters square, who also has a three plus Mr you know, it's not that, that pathology is not intensely bad and concerning for him. But in terms of whether or not he should be given anything that facilitates flow to the left side of the heart, uh given the way we know that that left heart is going to react to more fluid burden, that's just that, that is not something that we need to be doing for him and that is a missed opportunity to referral that he really needs, which is to a structural heart group. So if this is the way you feel, um if it's, if this is the way you feel right now, that is perfectly reasonable, you went through a bunch of physiologic concepts. But what I hope is is that the physiology actually helped you to understand kind of what we talk about and how we classify it. Here's why you should care. Um This is data from 2012 and 2018. So it's a little older and I'm gonna show you some very recent data on the next slide. But if you look at it. It's seven years, the survival of all comers with PAH. And there are a number of different entities within this group and there are some, with better prognoses, some with worse prognoses. Um, overall with PAH group one, if you are idiopathic or uh gene in heritable, um, or genetically, uh, or you have a genetic uh error, they tend to do slightly better than the connective tissue disease population. In particular, the scleroderma population who does not do quite as well, but summed together, the survival is a little less than 50% at seven years. Uh which really isn't very good and certainly is comparable to a large number of neoplasms. You know, I think it's not unreasonable for an oncologist to look at that and say, yeah, seven years survival for the particular cancer that I treat is what, what is expected and clinical progression occurs despite improvements at one year in, in one year survival. Um I think that this, although this next piece of data from the um far registry, um which is a registry run out of the Pulmonary Hypertension Association is a little more heartening. It was published in 2022 and the Pulmonary hypertension or uh through the PH A registry, which is the Idiopathic inheritable PAH at three years do have a survival probability of about 80%. And so that begins to kind of give us hope that we are hopefully modulating some real disease drivers although I think that Dr Wang hopefully will have a word or two about some exciting new information about really modulating disease drivers rather than modulating his processes associated with Pah. So for that person who just came in with or the person who just came in who's 28 years old and looking forward to raising her daughter and um only was supposed to be have or was only supposed to have hypertension, maybe, maybe psoriatic arthritis, but certainly a long life ahead of her. Um if she looked at these curves, she might be upset. Um And in fact, why her road is still long and winding, um the fact that this still goes on um was really distressing um and tells me that despite the numbers of iterations of guidelines that we are still doing poorly, you know, we are on the sixth World sym symposium with multiple edits and we are still not get good quality right sided echocardiography. What is wrong with this picture? Why can we not get this right? You know, is it the audience? Is it the message? Is it the messengers, you know, is it us, are we not communicating well? Is there some bargain or some uh some link uh or agreement that we're not making either with the patient or the provider that is not getting them that is not bringing them across the finish line of understanding? Unclear. But America was hospitalized in 2023 for shortness of breath, body aches, sore throat, and lower extremity swelling. She was diagnosed with UR I and ACH F exacerbation. Given her history of hypertension and her mild obesity, she was given diuretics which did give her significant symptomatic improvement and she was discharged on goal directed medical therapy or I apologize. Guideline directed medical therapy after her first echocardiogram revealed an LVEF of 45%. Now, her echo was limited and her RV was not well seen. Um which I thought was curious. Now for her guideline directed medical therapy consisted of an ace inhibitor, a beta blocker and some diuretic. She returned a month later to the ed with syncope where her blood pressure was about 70 systolic. Her ace was held and her BP improved. Imagine that um her new echo revealed a large dilated IVC estimated pressure in uh of in the right atrium from an I BC Respi AAS variation exam was 15 millimeters of mercury with severe tricusp agitation consistent with A P A pressure of 100 and five, an RVN diastolic diameter of five centimeters with poor qualitative R function. A small pericardial effusion which didn't appear to be causing evidence of tampon. No, but rest assured her LVEF was fine. So she was sent home with an outpatient right Heart Cath plant. Really, that's the best we can do after years and years and years of, you know, symposiums and guidelines and consensus statements and recommendations and education. This is still the best we can do. So, something, something, you know, something's not right in, in our world of education. But let's talk about the how of right heart failure. In other words, how does the right heart fail? In other words, we've talked a little bit about so we know that obviously America's right heart is failing. So how is her right heart failing? What is it about the right heart? That is not that under pressure doesn't seem to hold together? Well, to understand that first, you've got to, to understand that first, you hold one second here. OK. To understand that first, both ventricles are not the same. Um The right side is smaller. Uh It is not consent, it does not concentrically contract an RV and LV myocyte are clearly different. Um They respond differently to norepinephrine and the right side of the heart again is not programmed to respond to increased resistance by hypertrophy. The majority of the right side's pressure. Uh The adaptation is by dilation, not by hypertrophy. And you see the images of an echo uh a normal echo image apical four chamber right there. And you'll see that. Pardon me? And you'll see that the right side is small compared to the left, the right frame is slightly large there, but that's a pretty reasonable representation of a normal heart. So this is Arthur Guen's experiment in 1954 with clamping off the pulmonary arteries of dogs. Apologies to pet owners everywhere. Uh But what I wanted you to see here was that under acute load, the right heart doesn't do very well. And as you continually compress the pulmonary arteries of the dogs to a point, to a point to a point, all of a sudden the, the right atrial pressure goes up and then all of a sudden the right ventricle pressure, the right ventricular pressure, P A pressures go up and all of a sudden all the pressure is equalized and they do that at around 40 millimeters of mercury, such that the right heart cannot handle large acute increases in its workload. Chronically, the right heart responds to stress with, with dilation of the chamber, uh which is shown nicely on this echo image um as well as significant tricusp regurgitation leading to right atrial dilation can also lead to intra atrial septal shifts. So the left atrium looks effaced nice picture of the pulmonary vein right there. And the LV becomes a small, nearly a faced chamber. Um higher and the higher wall stress on the right side causes increased 0202 demand and consumption and basically sets up a situation of chronic RV ischemia in terms of let's now, let's magnify again. So you've seen what happens at the level of the echocardiogram, what's happening at the level of the cells at, at the level of the vessels in the pulmonary arteries themselves. There are only really three key factors in generating pulmonary vascular resistance, um at least in so far as we know right now, one is abnormal cellular proliferation at the level of the pulmonary artery, smooth muscles, disregulation of vaso motor tone and more vasoconstriction and non laminar flow. And you got a really pretty picture of rivers and rapids where you, where you will see that that is not smooth flow. And this can occur in the small pulmonary arteries and arterioles. And you'll see that this is, this is non laminar. It's turbulent, turbulent flow causes areas of rushing in the flow and causes areas of eddies which can also form areas of clot. This is as, as far down into, I'm gonna skip that one. This is as far down into the chemistry as I'm going to take you. But these are the biologic pathways for management of PAH as they are. Currently, this is an old slide. I look forward to seeing some new slides come out through studies that include stellar and pulsar. Um and start and to finally start talking about 1/4 pathway uh where we really affect uh or we really have disease modifying therapy. But we've all heard of the Prostacyclin pathway, the Nric oxide pathway and the endothelin pathway. So I'm not going to spend a lot of time on that. But I wanted you to kind of see the progression of how we started out with concept with kind of macro concepts like pressure, volume and resistance and then we got down to, you know, the heart itself and we got through how it responded to the right heart itself, how it responded to stress in terms of dilation and the hypertrophy. And then we saw how the pulmonary vasculature, you know, induced dilation hypertrophy. And then this is the biochemistry of how the vessels change to cause increased resistance in that system. So I'm concerned, could it be pah um And that is often me, although not with such buck teeth. Um So bottom line is this is a non-specific illness when it first shows up the NIH registry from way back in the eighties and nineties compared to reveal in the 20 tens, the most common thing remaining is still dyspnea. Um It is still the way they show up dyspnea or dyspnea on exertion. Some of these other things, fatigue and chest pain, fatigue, yes, but chest pain is a later presentation. Certainly edema is, is a consistent presentation with most of them. But near syncope and chest pain, I think of as a later presentation and that is consistent with the NIH registry being older and people showing up later at that point. Uh As we learned more about pulmonary hypertension, this is another slide from the, the European Heart Journal with the most recent set of guidelines. And I'd like to point out for your for your interest again, Disney on exertion, I think is an earlier sign with fatigue and exhaustion, bending over which I did. I had not heard of Ben AEA, which I thought was an interesting coinage. Um I had really, that was not my thing but ok, um, hemoptysis and you know, an abdominal distension from ascites and nausea, presumably from a chronic RB ischemia. I think those are later signs, but all of these are symptoms of pat symptoms of patients who are progressively doing more poorly and also hoarseness, interestingly related to compression of the left laryngeal nerve by a pulmonary artery dilation actually has a name called Ortner syndrome. Um I thought that was of interest for the fellows, but every once in a while you hear your patients and they sound breathless, not only do they sound breathless, but they sound, you know, they sound um they, they sound raspy and that can be a sign of markedly increased P A pressure signs of RV, backward failure here in terms of physical physical exam findings. Um I don't like RV, backward failure. I'd like you to think of that as RV, congestion such that you've had pato jugular reflux because of congestive hepatopathy. Um and asides from congestive hepatopathy, signs of RB forward failure, just simply, you know, evolving cardiogenic shock with peripheral cino dizziness, power and cooler extremities. And then there are some signs pointing toward the underlying causes of PH at the lower left there. So echo is the first easiest assessment. You see the prior picture on the left where we pointed out some pointed out some nice normal things on the right, we obviously have a grossly abnormal right atrium. Um And interestingly, we have this right here, which is fellows say it per right, pericardial effusion, sad. Um And obviously inter atrial atrial septal Boeing and obviously a flattened disk, interventricular septum. So this that heart is obviously in a lot of trouble. Um So there, so what the new guidelines encourage you to do is look at the tricuspid jet regurg at velocity represented on the left and use the PH signs on the right by two D echo and use those things together. Uh And there's a not a diagram in the guidelines to use the PH signs on two D echo, which include the increased RB to lb ratio where the right side of the heart is, in fact larger than the left side of the heart, the intra atrial septal Boeing, an assessment of the right atrium and the inferior vena cava mapped to the uh tricusp Agger at jet velocity to kind of give you a pre-test probability of PAH. I think a lot of us would look at that and say if you had that kind of echocardiography, um you know, we would expect that your tricuspid jet regurg velocity would be greater than three or 3.5 anyway. And that question would answer itself. But nevertheless, that is the direction of the guidelines. Um And that's a really pretty picture of what you don't want to see. Um And for the, for those, for those of you not to steal thunder from Dr Solway talk, but that is one heck of a lot of tricuspid regurgitation in a very dilated right ventricle with a septum with a septum that BS and RV, that is nearly kissing because it is so under filled a good sized pericardial effusion that is not hurting the right atrium yet. But uh this patient probably could use any one of the therapies that Doctor El Wing is gonna talk about shortly. Um So this is the PAH diagnostic algorithm. Um And what I've shown you is basically the history symptoms. We've talked a lot about history and symptoms. We've talked about echocardiographic probability. Now, a lot of people would rather fast track selected patients if you've got a scleroderma patient who has an echo like that with a history like this. Often, those could come to a Ph expert center. I think that as long as the prescribed work up gets done and there is collaboration about what the right heart catheter data needs to show, then it might be acceptable to do that outside of a center. But we do generally like to do our own right heart Caths. So diagnosis, this is the North Star. This is not a historical artifact. It is something that is, that is used every day in practice. Um We um um it is not something that um you know, IIII I remember a whole session at AC CP where when we saw these on a chest radiograph, they were referred to as simply a historical artifact. Um There is class one recommendation for right heart cath to perform, to guide diagnosis and treatment decisions. There are measurements obtainable through right heart catheterization that are protocols on the right. You can look at those in the guidelines. I don't want to spend too much time kind of going over those. But a and often a shunt run is advised to capture less common uh teos of pulmonary hypertension to include um to include congenital anomalies, which may include, you know, partial anomalous, pulmonary venous return or other vascular malformations. Um There are complications associated with right heart cath. This is an old slide and so the incidence of serious complication is certainly less than 1% of fellows you will be asked about pulmonary arterial aneurysm and rupture and how you manage that on your critical care boards. So I wanted to put that in there for you. Make sure you get the most out of your right heart cath, test your balloon. If you're the one placing it, it's really bad to go in with a balloon that's uh nonfunctional. And if you're not doing your own right heart Cath, please check your tracings before they take the device out. There is no such thing as a negative seven millimeter uh right ventricular diastolic pressure. It does not exist no such animal. And you'd be amazed at how many negatives I get when they oh the pressure is 64 over minus seven. No, a vacuum does not exist in nature. Make sure the wedge always is estimated at end expiration and consider comparison to capnograph to verify that. Also, if you need to, if you're not certain about the wedge, don't hesitate to ask your cardiologist or whoever is capping for an lbedp and check your wedge saturation. So, America's story, there's her c data kind of as expected. Um She is in trouble. Her cardiac index is less than 1.7 L per minute per meter square below two is often an indication for uh advanced parenteral continuous invasive therapy. Um Doctor Elwin is gonna tell us in just a second what she would do, but we completed her sleep evaluation, stopped her beta blockers and anything other than a little bit of Aldactone started her for an oxygen initiated triple therapy. Um And uh she is improving. Um She was very hard to negotiate with upon what she would do as a young mom and a nurse who couldn't stop working. So I think that that's definitely something to talk about, you know, did I get her on everything I wanted? No. Did I get her on most of what I wanted? Yes, because it was actually PAH therapy versus uh GDMT. And I said, what is that triple therapy that you're asking about. So anyway, um the only and the only rather only diagnostic human dynamics and proper clinical context taken together are going to get you a pah patient who is likely to benefit from Pah directed therapy. And I can't say that enough if you've matched the clinical context and you found the right hens, you found a pah patient, the other things you may need a little help with and we're happy to help. Um This is the summit of chart from this. It's gonna take some time to go through, but the reference is at the bottom is European Heart Journal in October of last year. As you go through it, it makes more sense. Again, emphasizing the fact that groups two and three are common. Everything else is uncommon or rare. And so therefore the prescription of Pah directed therapy should be uncommon, although certainly in a specialized clinic, somewhat different. Um The ph A re remain remains an incredible organization and they have an incredible online website to learn more about the disease process and I apologize for going over. Thank you for listening. Published November 28, 2023 Created by Related Presenters Michael S. Eggert, M.D. Sentara Pulmonary Critical Care and Sleep Specialists View full profile
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