#294 Pulmonary Hypertension Update

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Well, Paul, we've done it again. We sure have, Matt. I feel like we start every episode in this exact same way. And you know what? I'm here for it. I think that's comforting to the audience. Uh they'll they'll also be comforted to know that we're talking about pulmonary hypertension tonight with a focus on CTEF, which I don't think I fully understood before really going through things to prepare for this episode. Our guest is Dr. Estefania Oliveros.

And a reminder to the audience that this episode and most of our episodes are available for free CME credit through VCU Health Continuing Education for free at curbsiders.vcuhealth.org. And Paul, I know you're gonna tell them about our wonderful guest and our super producer co-host, but first can you remind them, in general, what do we do on this show?

In general, Matt, we are the Internal Medicine Podcast. We use expert interviews to bring you clinical pearls and practice changing knowledge, and as you mentioned, we have Uh super producer and newly minted physician, uh Dr. Deb Gorth, who has produced this episode and put it together with our guest. Doctor Estephania Oliveros and we're gonna hear her, as you say, all about pulmonary hypertension with a little bit of a focus on CTEF, which I

like you found a little bit bewildering prior to hearing all about it tonight. So for you all to know at home, Dr. El Oliveros is an advanced heart failure cardiologist and a clinician researcher specializing in right-sided heart failure, pulmonary hypertension, and CTEF. She practices in Philadelphia.

doctor Oliveros earned her medical degree in Venezuela. After graduating, she completed a research fellowship at the Mayo Clinic before residency at Temple University Hospital, and moved to Rush University Medical Center for her cardiology fellowship. Finally, she completed her heart failure fellowship at Mount Sinai Hospital in New York. And so, without further ado, let's hear from Dr. Oliveros.

Well, Stephania, thank you so much for joining us. We're excited to talk some pulmonary hypertension, but first

Tell the audience a little bit about yourself and any hobby or interest you wanted them to know about, something you do outside of medicine, to remind them that you're a person that does other things. Um, hi, thank you so much for having me. I'm very happy to be here. Um, I'm one of the heart failure cardiologists at Temple University. I love to paint. And that's what I do with my free time. A lot of watercolor.

That sounds wonderful. I Paul, you do do you paint? I I could see you being like a a a secret painter that you've just never mentioned on air before. No, no, I have no creative um impulses whatsoever. So an appreciator, but I can't actually come up with anything on my own. Okay. So along those lines, Paul It was a it was a weird it was a weird guess, um which it was incorrect.

Um but along those lines, as someone who can't create things but likes um enjoying ev somebody else's creative output, uh smooth transition, five years we've been doing this. Can you give me a book recommendation? What what book Um I I've actually managed to carve my way through a lot of the recommendations on the show, so now give me something new that I should read. I just read uh The Daughter of Fortune by Isabel Allende. Um she's an author from Chile. It's a beautiful written uh novel.

if you are into that type of stuff. I I like beautiful novels. That sounds that's right by Alex. It's a beautiful novel. Yeah, no, it really is. Um I had stopped reading in Spanish for like the past like three years or so and I did that just to test myself and it was wonderful. I felt so good. And you said Daughters of Fortune? Yep. Mm-hmm. Excellent. The Daughter of Fortune. Oh Daughter of Fortune.

So you've obviously succeeded a lot to get where you are, but one of our favorite questions is what is your favorite failure during your career and then what did you learn from it? Um I guess uh one of the things at the beginning when I I I started was that I didn't get it together to get all my like the steps in. Um I was very young when I finished medical school, so it took me a while to decide that I didn't want to do full on research.

uh but actually clinical stuff. So I spent like two, three years trying to find my way. But in that I also learned a lot of how to do research. So it was like a a plus, but it took me a while. And then I'm gonna just jump into this next one. You know, as a learner and a new intern, I'd like to know what is the best advice that you have ever received as a learner or a teacher or during your career. I could use some advice.

Um, I think internear is super hard. So kudos to you for like, you know, enduring all of that. Um, sure you're gonna be great. I think one of the things that someone told me when I was an intern was like learn to identify the sick people. And I think people take that for granted, but that's a skill that you develop. And then um as you

continue to advance you have to I guess like focus on like how you learn because it will modify how you learn as an adult learner. If you're a visual or like you like to learn by teaching others. So you can keep growing and honing on those skills. So when you are like ready to go and become a like a full-on internist, you can learn how to teach and how to continue learning because it never stops. Fantastic.

You've given us a book recommendation. I wanted to see, Deb, did you want to share a book recommendation as well with the audience? Yeah. And so I one of my goals is to not give up reading during intern year. And so I'm a month in and I haven't done that yet. Um so my first book that I finished was Say Nothing by Patrick Radden Keefe. Um, it's kind of about the sectarian violence in Northern Ireland.

And as someone who kind of came up post nine eleven, I think we have a very specific view of what what terrorism is in the the global geopolitical situation and I I just hadn't been exposed to sort of the the conflict in Ireland as thoroughly because it just was it wasn't on the news when I was realising what the news was. Sure. Paul Williams, do you have a pick of the week?

Yeah, su I mean always. I'm just I'm just waiting for you to ask me, buddy. Um I'm gonna recommend the the twenty twenty one movie Nobody, starring Bob Odenkirk. It is it is far from a masterpiece, but I enjoyed it very much. It hit me at the right time, so I've been a huge fan of Odin Kirk since um Mr. Show with Bob and David back in the nineties and he's always been this avatar

of like just barely simmering rage, which actually works well in this particular movie as this sort of everyday guy who lives through a home invasion and then kind of gets set off and then as things unspol and unfold, he ends up sort of involved

in a fight with the Russian mafia and just sort of he turns out in this everyday schmuck to someone who um listen, I'm trying not to ruin it for you, but there's there's a lot of shooting and fighting and and broken bones and stuff, which if that's up your alley, it's spectacular watching Bob Odenkirk just go around and and beat people.

Um so it invites a lot of comparisons to John Wick and is actually produced by one of the guys who's involved with John Wick, but I I feel like he actually has more of a character art and it's a little bit funnier and later at times. So if you're in the mood for like big dumb violent fun, I would highly recommend the movie Nobody.

Okay. I just uh to give a book recommendation which will be quick, I Paul, a book you recommended to me probably ten years ago now, American Gods, I just reread that book. recently. I think the T V show may or may not have been cancelled, but the book is definitely worth a read if you're a fan of Neil Gaiman. It's uh I would say it's like a f fantasy, but set in like a modern world. It's a it's a good read.

So definitely check that out. So lots of picks of the week this this this time, Paul. It's been a while since we've done that. Yeah, it feels good. Um but we should probably yeah, I th I think it feels good. Maybe we'll bring it back. We'll see. People will start to complain. Too many picks of the week.

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Let's get on to the main topic, which is pulmonary hypertension. And Deb, we've done this topic in the past before. So how do you want to start this? How how are we going to handle this? And do you want to set up the show a little bit for the audience? Yeah, I'd love to. So this is a pulmonary hypertension update. So we actually covered pulmonary hypertension in twenty eighteen. And a few things have changed since then about how we categorize pulmonary hypertension and how we define it.

So just to start us off, can you give us a basic what is pulmonary hypertension and then maybe the changes in the definition since since our last episode? Sounds great. Um so pulmonary vascular like disease. Like pulmonary hypertension is when you have a person that has a mean uh PA above twenty millimeters of mercury or a PVR of more than three wood units.

That's the definition. In the past it used to be a mean PA of more than twenty-five, and that's how it has changed in between. They just changed the standard deviations of the deficient. Is it important for us to really understand what a wood unit is or the the the P V what the P V R is? Because I I mean I read the definition. I'm just

I'm not quite sure that it it means that much to me. How d how do you think about it? Is it is that something or should we just go I usually go by the mean pulmonary artery pressure, but What what's this P V R wood unit stuff? The way I think about it is um When you're transitioning the thought process is when you're talking about blood pressure, just systemic blood pressure, you talk about a map, right?

So it's kind of the same thing. Instead of a map of like sixty, which is what your organs need to perfuse, you're gonna talk about a mean PA of like twenty, which is that mean pulmonary pressure that needs to be across the whole tube, right? Which starts by the pulmonary artery, goes into the pulmonary capillary bed, and ends up in the left atrium. So if you see it as a whole circuit, so that's your mean pH. So then you have the resistance which is just pressure and that pressure is

How much tightness at the end of that system you're seeing, how much resistance you you're seeing. So we use three uh with units just as a as a number and a car. I don't know if that helps. Yeah. Like a hose. That's how I explained that to my patients.

Okay. Uh so can you talk me through because I I feel like a lot of the the classes of pulmonary hypertension are are often discussed in terms of whether or not they're precapillary or postcapillary.

Do you mind just sort of talking me through what that means? I feel like conceptually I I've I've struggled with that a little bit. So like how does that framework help and what does that mean exactly? So when you think about pulmonary hypertension, you have if you imagine a whole circuit, you would have The pulmonary arteries are step one, where your right ventricle is pushing blood.

And that goes into the capillary bed. And on the end of that, you have the venial component and that drains into the left atrium. So if you had to divide the circuit in different things, you will say anything that Before the capillaries, this is the precapillary bed. You're usually talking about like the pulmonary arteries itself. So that pressure that's idiopathic.

prior known patient as idiopathic, the pure pulmonary arterial hypertension. If you have to cut that in another percent, is like all the postcapillary, which is the venules that are draining into the left atrium. So all that resistance that can come resistance that can come from the left ventricle is pushing that resistance like backwards. So it's all like passive congestion.

So that's why when people are doing this definition of pre or postcapillary or combined is because you can have a little bit of like everything if you have combined or just a piece if it's post-capillary coming from the left side or if it from the your right ventricle or pulmonary arterial side, it's precapular. And the watch, which should be like a surrogate for what's happening on the left side. Across the septum, it's what's going to be elevated or not. So that's how you define each group.

And depending on the theology that we can go over it a little bit, those definitions uh vary because it's sometimes a little bit difficult to kind of give just one group to one patient. So that's why these broad definitions come in.

I think it's always hard to think about the the five groups. To recap for the audience from last episode when we talked with doctor Ryan about this He he thinks of it. He kind of simplified it that about 60% of pulmonary hypertension that you're gonna see is gonna be caused by left-sided heart disease of some sort. He talked about the company it keeps.

Those are often your patients with obesity and high blood pressure and CKD and diabetes. A lot of the times, you know, you'll you'll see it in those patients. Another 20% might have the chronic hypoxic lung disease. And then

About ten percent might have a combination of chronic hypoxic lung disease and left sided heart disease. And he said that group there, the lung the chronic hypoxic lung disease and the people with left sided heart disease, that makes up like ninety percent of the pulmonary hypertension. But this episode is gonna focus more on the other ten percent that

We understand much less well. So, Stephania, can you tell us about the groups? Can you remind the audience about the groups and and then we'll start to get in eventually to the specifics of some of these groups that we wanted to pull out for tonight? Perfect. So group one is the pulmonary arterial hypertension. Group two is uh the left-sided heart disease.

Group three is those people that have like the lung diseases and hypoxia that you mentioned. Then group four are those that have occlusions in the pulmonary artery, and group five are like everyone. I I don't know that audience, I can't promise you that we're all gonna understand what group five is after tonight, but we'll understand the other groups a bit better, so

Uh Deb, what's what's next on the agenda? I think we've kind of like we talked about the new definitions, we talked a little bit about the precapillary, postcapillary, and the classifications. So where do you want to start tonight? Oh we could jump right into the case from CashLack. Does that sound good? I I thought you might take us there. Let's do a case. So this is a case that came into cash flak. Mr. Paul M. Balai. He is a twenty one year old college football player.

So three years ago after a long flight home from football camp, he was diagnosed and treated with DBT. He's had no other intervening medical problems. However, over the past six months he's experienced increasing fatigue shortness of breath, and occasional syncope. These symptoms have required him to stop playing football,

before the end of his season and he is coming into office today because his football coach made him, because his football coach wants him to be ready to play the next fall. Uh in terms of his family history, his father and uncle both had ischemic heart events in their sixties. And then he has no other medical problems and is on no other medication.

So I I think it's a very cool case because it's something that you are really gonna see uh when you're training these patients. So When you're having people that have pulmonary hypertension, what you mentioned are usually the most common things. The shortness of breath. It's like eighty percent of the people have that. Fatigue you'll see like in a third of them. And then the syncope part is what makes it very scary. And that's when whenever you hear that story, you have to pause and try to

figure out if this young kid is having something that could like really affect his viability. He can die. So the majority of these kids, uh especially like at that young age, they'll tell you, Oh feel good, there's nothing else bothering me and you're like what could a twenty one year old uh have wrong? And that's where your like little alarm has to set up and kind of like try to look for clues for these types of disease, like pulmonary hypertension.

Yeah, I feel like there would probably be delayed diagnosis. People would assume this guy has asthma or something like that and probably not that he's out of shape because he's on a sports team, but it seems like a diagnosis of pulmonary hypertension could be delayed in such a young person. I think for sure this patient would probably get an echo. I think just based on the syncope alone.

Um, I'm not sure that I w well well, I can just put myself in here and say I might not be astute enough to have gotten the past history of D V T or necessarily connect the pieces. I think just sort of based on this person with this sort of what sounds like exertional dystany in the young age, I would probably get the an echocardiogram. So I guess I'm wondering

Is this typically how pulmonary hypertension presents? So w I guess what like what are the typical presenting symptoms? Is it is it often these younger patients or like what what do you ordinarily see? Is this a prototypic case or is there a more typical presentation?

So this is actually like a common presentation. You see a young kid. that's having shortness of breath and syncope and that's where you start thinking about these things once you rolled out the asthma, the obesity, the uh history of like maybe substance use, uh dehydration.

those are like the first things that you think of. And then you keep it in the back of your head, like why this young kid is having is passing out while exercising. So that should trigger a thought process leaning towards like cardiac. I would say. And from there I think is where you stamp your

Yeah. And I would classically think of like a hypertrophic cardiomyopathy in a young person exercising who passes out and sports physicals, the a lot of the times they're trying to get that sort of history from people to work them up further.

Right, I guess that I guess that's my and maybe I'm not asking the question right. Like I feel like syncope as an initial presentation I I would think that there would be other symptoms leading up to the syncope first, I guess. And is that am I misunderstanding some of the pathophysiology behind that? Like I feel like the syncope seems like it would be sort of a later finding, or is that really just

Isolated as things could be and pre-aggressive discount exertion are typically how these present. So it would be thieves near first.

And then when the patient has severe pulmonary hypertension is when you would see syncope. You wouldn't see syncope in a mild case of pulmonary hypertension. It has to be someone that has restrictive flow from the pulmonary arteries to like your coronaries and brain in because of the resistance in the lungs and then that's why you see or because you have an arrhythmic episode related to that hypoxia that leads to the symptoms.

One of the reasons I was so excited to pick this as a topic is it there was a study that was looking at the mean time to diagnosis. of this type of pulmonary hypertension. It seems like from presentation to diagnosis the the average was around 14 months. So for a condition that's completely treatable, it's definitely worth having somewhere on our

Deb, which type of pulmonary hypertension was that study quoting? Was it type was it group one? Yeah, that that was looking specifically at C T E P H. Okay. So that is group four. CTEF, is that how the cool kids say it? Mm-hmm. I think so. Yeah, so because I I think one of the valid things in this case though is that you do have someone that's young that has the history of DVT with the shortness of breath and that's how you put it together. But most commonly what you have is

Someone in their forties that has been fine until one year, two years ago. They start having progressive shortness of breath. and they come to your clinic to you try to pull data, pull data, do a good story of what their symptoms were. And then you find out that now they can breathe. Now get they get uh shorter breath from room to room. They're now syncopising. It's kind of like more progressive over time. That's one of the things that you see. And then when you start

looking and peeking into their risk factors you find that they had a PE a year ago and they were briefly on blood thieners. So those are the people that you wait like fourteen months like that said. And you say, hey, these clot may not resolve, and then you think of pulmonary hypertension at CTAF.

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Can you talk a little bit about the uh pathophysiology of CTEF because When I was reading about this I was a little surprised to see that they think maybe it's not that the clot is always coming from

a distal extremity and then embolizing that maybe there's some disease within the pulmonary vascular bed itself where the clot is forming de novo in there. Am I misunderstanding that or is that part of what people think happens from this?'Cause there's sometimes there's not a history of DVTPE and these pages. That's correct. Like the majority of them do not have the history, but the problem is that no one really knows why it is.

Yeah. Like we assume that it comes from there, but there's like some post mortem uh studies that have looked at biopsies and some of them think that there's like in situ changes. And what happens on these lungs is that They kind of create the same look that a pulmonary arterial hypertension patient would like. You would see the same cut. So that's why you're talking about incetotrombosis and like these other pulmonary vascular changes at the micro level.

So I'd like to talk about kind of what are some of the other things in the patient history that we should investigate. So I gave you kind of a bare bones, but what are the other questions I should be asking? So when you get these patients with shortness of breath, I think it's important to just get a sense of when did it start. if it's getting worse over time, is it uh at rest or is it with exertion? You try to take out pieces like are you wheezing? Are you having dry coughs?

'Cause you wanna make sure that you're not missing other common causes of shortness of breath. And then you start to think about heart failure kind of symptoms. Are they having orthopnea, bendopnia? Swelling in the legs, what is it that's making them feel so short of breath?

Those are like the basic things when they come to plan aid that I try to think about in terms of the shortness of breath etc. just to get a very good history to cr try to associate it with either cardiac, pulmonary, sometimes GI if they're having like GERD.

Can you remind the audience? what sort of things in the history, because I I think for this patient specifically, maybe we should still be thinking about could they have like a group one pulmonary hypertension. Can you talk about what things are associated with that that you would think of or ask about? So when I meet these patients, I usually ask about have you had any rashes, any joint pain? Does like your fingers g get blue, like trying to figure out if they have like any ring-off?

Are you getting like footstuck? Like thinking of like crest kind of scene? Do you have any chance of having like HIV, hepatitis C or any other type of hepatitis? So I'm always going through these like mental lists of anything that could be kind of group one. And then one of the important hints is that you wanna ask about that family history. Like does anyone at home have any autoimmune disorder?

any history of like lung transplant, any history of heart failure, something like that. Because those are usually the clues. It's all about in these cases of getting like a good history of the Mm-hmm. Paul, should we ask about the physical exam? Uh is that

Yeah, I I would love to hear about the physical examination. Is there is there anything particularly localizing or I actually I guess a better question to ask is when you're approaching a patient like this where you suspect pulmonary hypertension, what what things are you looking for on physical examination? What would really point you hard in that direction?

I love pizza example in them. It's so cool. So the first thing is usually uh if they're decompensated they tend to be a little bit tacky card. They would have an S1 that's normal, but the S2 is usually like more accentuated, like a little bit more intense. So that speaks to that P two, the pulmonic valve component that needs the resistance. Then, if they have some pressure building, you can hear like the tricuspid regurgitation murmur, that systolic murmur in the left.

personal border and the my favorite is just because I did heart failure is the JVP. So I'll spend hours in that J V P. uh turtlenecks are the worst thing that could happen. Uh but um in in all honesty is basically measuring that Jugular uh Venus pulse pressure, trying to see if there's a large V wave. Sometimes you have deep breath and if it like drops down that JVP you think of like Cosmo sign which would hint like to RV failure and and so on like S3s or extremity dema. It's very very nice

And the rashes and the fingers. Those are your biggest keys for like what you were asking before. Because sometimes the physical exam hints to other etiologies of pulmonary hypertension. This one time I had this patient that had tel injectaso and that was the hint for portal pulmonary hypertension. That is very cool. That's a very cool diagnosis.

In the first office visit with you, I imagine probably these patients will come with a chest x-ray, an EKG, and an echocardiogram. What other testing do you think someone should send before they get to you or and and if it hasn't been done, what would you send on that first visit? For any like pH screening, those are basically like the three things that we order.

Then if I'm talking about blood work, just come on CBC because I'm looking and thinking about hemoglobin that can go down, like anemia that could cause like shortness of breath. I also look at the platelets because they have hypercoagulable states. The majority of them? or a percentage of them, I should say. And then also LFTs.

I usually send for TSH. It's very common to see people that have pulmonary hypertension associated with hyperthyroidism or hypothyroidism. It's like twenty percent actually of your patients. And you just treat that and you can actually reverse the pulmonary hypertension. And it's amazing. Then other things are like the hepatitis panel. ANA is like a blanket.

Test. I know it's very difficult and rheumatologists do not like it when you order it without knowing. But it's something that we do in part as part of the screening. And the DQ scan. what it's recommended when you're trying to rule out causes of CTAF. And that's how we use it like as a map for every else that comes from there. Do you order the overnight oxymetry and pulmonary function tests as well on all your patients? Is that more of like a case by case?

So if they're telling me that they have Disney and the the the presentation is Disney, I will usually take uh the PFTs. Mm. Because the majority of these patients all have like a little bit of like restrictive lung disease, uh especially our pulmonary hypertension group one. And some of them have obstructive lung disease. So you want to take that out of the question when you're trying to get to the thoral like answer. what is it that's causing you to be blue or not breathe?

For this guy here, let's let's take it back to the case. So we've we've talked a bunch. So we have Mr. Paul Embeleye. He's a twenty one year old guy. He's a football player. He's been having shortness of breath and syncope. He's got a little bit of what sounds like a suspicious family history. And we've ordered the labs that you talked about there. Like we've taken a history. We don't really think he has a rheumatologic disease.

Um, he he doesn't have HIV and doesn't seem like based on his imaging he has like interstitial lung disease and he's not taking like any amphetamines or diet pills, so to speak.

So we're not really finding anything else other than this history of prior pulmonary embolism. Can you talk a little bit about the VQ scan? Because I think probably Most of the audience would think of VQ scans as like not as helpful as a C T pulmonary angiogram, uh, in their day to day practice. But why why do we like it so much for someone like this guy?

So if um that is true and I think yeah the beauty of the VQ scan is that it's your gold standard when you're trying to rule out causes of like C When you're trying to understand a little bit more the anatomic part of the lungs or trying to see if there's an acute P is usually something that you you find it more useful to be completely honest.

When we are evaluating patients for CTAP though, we do use the CTPA, but I think as a blanket test, VQ scan will have a very strong possibility, like uh sensitivity and specificity for like CTAF, if that's what you're thinking. Yeah, from what I was reading, the negative predictive value approaches a hundred percent. Is that right? So if it's a normal perfusion on the VQ scan, then CTEF is very, very unlikely. That's correct.

Okay. Yeah. The last time we talked about this with Dr. Ryan, I'm not sure if you remember this, Paul, but I think he told us that. Not every center is as as useful reading them and they might even repeat them when he when they get to the center. Is that does that sound right? That sounds familiar. Yeah.

I so at your institution, it do you have specific people that read these? I I m I know you're at a lung center, so there's a lot of probably probably used to this diagnosis, but it it sounded like if you got it somewhere where they're not used to looking at VQ scans. then it might not be as reliable and maybe I'm I'm not sure if you repeat it uh when patients come to you. So out of

Consciousness for health care cost and because of the patient is receiving radiation. Sure. We try to obtain the raw images no matter what the reason. you will see it very frequently called like low probability because it's low or high probability for P and sometimes it gets missed, unfortunately. Um, I'd love to hear what are some of the so I guess what are some of the expected findings on VQ scan? Like what would it look like?

So when you're having like a positive VQ scan, what you see is a ventilation part. Which assesses how the lung is like the lung parenchyma is working. And then you have a perfusion part where the pulmonary vasculature is having. feeling like perfusion defects if anything. What you would see is wedge like defects. And you will see it by regions wherever the clot is. And usually, if you don't have parenchymal lung disease, but only pulmonary vascular obstruction, you'll see an unmatched disease.

So that's how you see it called. That's basically like the fine. It's interesting because with COVID, we don't receive any ventilation scans anymore. So sometimes you can have like a very elevated hemidiaphragm in that. you like a perfusion defect, but it's not, it's just an elevated em hemidiaphragm that's causing a perfusion defect, quote unquote. So you have to eventually like see the images and compare with your chest x-ray C T.

So tell us how might it go for a patient like this if he had a VQ scan and what what would be the next steps for him in terms of diagnosis and and then uh treatment if we're if we're at that point. So if we had a BQ scan that was positive we'll probably get a C T with contrast.

and that to characterize what we're seeing, just to make sure that we're having matching data. And then from there we'll probably obtain, I assume you already have an echocardiogram, so we'll get a write hard cap to actually get the measurements in the heart. to see there's any degree of pulmonary hypertension

I'd love to back up and kind of define we. So we try to target kind of general internists. So I think a really good question would be, at what point would you try to refer this patient to a specialized center? And who would get this? So usually uh when you have someone that has uh shortness of breath, you have a positive VQ scan. Ideally you should refer them at that point.

for a cardiology or pulmonology evaluation, depending on the center where you are, the pulmonary hypertension expertise center may vary. between both specialties. And then from there, they should refer the patient for the additional testing that I mentioned, such as like ride her calf, MVQ scan and And I may and I'm sorry to go out of order and I may have missed a step here, um just due to Yeah.

But can I ask you to talk about the role of the echocardiogram? The way I would emer imagine this sort of going down is you see this patient would probably lead with the echo see probably suggest an opponent of hypertension. on that and then make the referral and then all this advanced imaging would kind of happen through opponent or hypertension specialists would is how I would picture this kind of going down, but maybe I'm thinking about it wrong and maybe

a boulder primary care doctor would even get the VQ scan in advance of that referral. But I guess to go back to make it an easy question to answer, can you just talk about the role of echocardiography in diagnosing pulmonary hypertension while we're talking about sort of diagnostic modalities?

Yeah, with um that's a great question. So With any patient that you have pulmonary hypertension or you're suspecting pulmonary hypertension, if you send the patient then y they find that there's um an echocardiogram with suggestive of pulmonary artery pressures that are elevated. or a right ventricle that's enlarged or dysfunctional, those are keys that hint whoever placed the order for the echo to suggest that there's pulmonary arterial hypertension and then from there you can refer that

To a specialist so they can continue the workup. That question is very tricky because it depends a lot on the expertise. local expertise that you have. And I feel that the guidelines are really not very specific on when and how to refer and who to refer. They kind of like give you like an algorithm, but it doesn't stop

like tells you where do the internal medicine duck hands off the platoon. I that's how I feel. I think if if you think you have somebody, you know, that isn't in the ninety percent group where they have Chronic hypoxic lung disease or left sided heart disease, and you think someone has one of the other groups, like either one of the group one pulmonary arterial hypertension or

group four, CTEF, or group five, you don't know, you know, the one that I don't understand, then those are people that I would certainly refer. And I think we're capable of getting a lot of this history, Paul. And depending on whether or not I suspect a rheumatologic disease, I may order some of these basic labs and some of the viral screening stuff, depending on the history. But if I suspect it's not

In the normal like flavor of pulmonary hypertension that I'm used to seeing, which is group two and three, then I I would pretty much refer. Paul, do you have a different practice? No no, God, no. If I suspect actually really any form of owner of hypertension, they're off to a specialist. I'm not gonna try to manage any of that stuff on my own. I I agree with you, you know,'cause I get screening A and A's on all of my patients. Um just just to get a basic. That's just good primary care. Yeah.

Yeah, I mean and then if it lines up with the C T angiogram, like if for anyone who just coughs funny, then then I know and can send them off. But no, like I

For sure. I I guess what I'm getting at is I you know, I think my concern is I could see this being a misdiagnosis or a late diagnosis for someone who doesn't quite recognize some of the cardinal features or maybe doesn't feel comfortable with some of the the more advanced diagnostics in terms of the imaging, like, you know, it's how how often do you pull the trick on a V Q scan?

In the primary care setting is is not something that you do very often. So I'm just trying to think through for our primary outpatient friends how we think about this and how best to serve the patients without missing anything. And we should remind the audience that CTEF is is a rare diagnosis, correct? So

I I mean I can't I I don't even know that I've ever uh seen a case. I certainly haven't diagnosed a case myself. Estephania, how do you think about this? Uh I don't know if you have stats like for what percentage of population has it or something that would even be meaningful, but it seems like it's a pretty rare thing.

It is. So the way I explain it to my patients because they always when they come to clinic they're like, Why me? Why you So the way I explain it is you have an acute P at one point in your life, and about 90% of those people will resolve that P, acute P with antiquity. then you have these little tiny 10% of which some of them will have just recedal clot, but they won't progress to pulmonary hypertension itself.

And you are unfortunately on that less than three to five percent depending on the data. The problem is that because we this diagnose goes under the radar, we don't actually have actual statistics of the prevalence for the incidence of uh CTAF because we don't go around testing everyone that had a P once upon a time because it's not a routine.

W and how do you talk to them about the treatment? What might that sound like? Let's take it back to our patient, Mr. Balai here and tell'em like we've we've made the diagnosis. We're pretty sure this is CTEF. How would you talk to him about what the treatment might look like and what the what the options are? So the beautiful thing about CTAF is that Pulmonary and arteractomy, which would be the surgery, is the only form of

therapy that makes the disease curable. So you tell them that. You have the option of having surgery. It's a big undertake because we're talking about heart and lung surgery, but it's something that will cure you, right? And then after that you have other therapies which are medications, real C What. It's one of the medications that's approved at the moment for that.

And then there's other investigational things that can happen down the line, which include volume pulmonary angioplasty, or if the disease is not like you can refer actually the patient for a long transplantation if it goes that way. So that's kind of the conversation.

And this is essentially W the the surgery is is an open heart surgery. They're on cardio pulmonary bypass. There's I think cooling of like the heart and the head and And the br and the head. Yes. It's it audience, you should we could link to the paper that uh Dr. Gorth pulled out uh for our pre reading for Paul Eye, but it was very yeah, it sounds pretty crazy.

It is like a crazy I thought they just went in there and just like sucked the clot out and they're just like, Let's call it a day but they're like dissecting into like the pulmonary arteries. It's it's a it's crazy. Yeah, God bless you for saying that, because I I had the same impression, like yeah, I thought just a couple snip snip and then you get the clot and then the patient feels better. I did not realize like it was

quite so extensive as cracking over the chest and like you're on bypass and they're freezing the head and like it's a hole. And while you're in there if you want to do a cabbage while you're warming them back up, you kinda like, Oh my God Like it's just so it's it's not nothing. Like it's it's definitive treatment, but holy smokes.

It is. It is. So that's why there's not a lot of places that do it. That's why it has to be referred to an expert center for that. And um it's a big undertake and when you're trying to see who is a candidate, you may have upper policy. but you may not be a surgical candidate. If you have too many comorbidities, that may not be the right choice for you. And what about the medicine you mentioned? How how well does that work?

And is it is it something that provides just symptom relief for patients? Is there a mortality benefit if they if they take the medication? So there's symptom relief, there's hospitalization reduction, there's no mortality benefit. What you see is an improvement in hemodynamics The balloon uh pulmonary angioplasty that you mentioned, is that still experimental and do we have data on heart outcomes for that as far as like symptoms and hospitalizations? No, nothing yet. Yeah, you're just like,

Audience, she was she was like a screen cap of that face. Like, Oh you dear sweet man, you poor stupid child. No no No no no I didn't say that. I didn't say that. What I said was uh no it's something that we do. There's a lot of interest in the in the area. Um, it's something that people are looking into. Uh we still have to find like randomized control trials on it. It's a lot of observational data.

I'm sure it's hard to enroll if it's it's such a rare condition and to find people that are candidates and have this rare condition, I'm sure it's it's tough. But for a guy like this, I mean he's a twenty one year old college football player.

He may may be someone that would undergo a surgery or or transplant as you said. And and then from what I was reading, sometimes people, even if they get surgery but they still have symptoms, they may also get the medication or even maybe angioplasty if there's still residual stuff that they think could be intervened upon.

That that is so that's something that uh we discussed ahead of time. If the patient has a lot of disease close to the heart, which is what we call proximal, is something that the surgeon is gonna be able to snare out, basically, and like remove. When the disease is very distal and the vessels get very small, they may not be able to get to those distal planes. So they'll benefit of a combination of medotherapy or the balloon pulmonary angioplasm.

Can we talk a little bit about and I don't believe these medications are part of it and you didn't mention that, but they're they're for the pulmonary arterial hypertension, there's a bunch of different meds Are those ever used experimentally or have they been tried in CTEF or can you speak to that a little bit? And how how familiar do you think the audience needs to be with with those medications? So for CTEF specifically, right now it's uh real CWAT, the one that it's like FDA approved.

Other medications have been tried in different clinical trials but they have not met uh hard outcomes. There's a lot of ongoing enrolling trials that will try to address that in the next couple of years, three to five years, hopefully. And uh for the audience I think it's important to know like the medications itself.

more than for prescribing for like the side effects if you have these patients in your primary care, like you are the pri the TCP, just to know what is it that their the patients can complain because the side effects can be ruthless at times. Can you talk about what are some of the classes and and the side effects if you could kind of run through for each class and which side effects we should look out for? Okay, so the easiest I'm gonna go from easiest tolerable to

I cannot handle please stop. Okay. So Foss with yesterday? Type 5 inhibitors like uh sildenophyll and taldalophil are like the first group. It's like the Viagra, basically. And what patients usually complain of is headache. So you actually medicate them with Tylenol as long as It's safe and those are like the basic things uh that we do and we tell the

Some of them can have hypotension when they take the medication. So we try to uh counsel them of not taking the medications with other blood pressure medications because it really can plummet. Down. The most important thing is to tell them not to take these medications with nitrates like isotol or isosorbide, those type of things, because both of them act in the same path. Then the second medication that is kind of tolerable is the endothelium receptor and So all of them have enton in the name.

So that's how I remember it. Ambricenten Boscentin Massitentin and they sound funny, but those are those are. Um, all of them have liver toxicity, so you kind of have to monitor ASTs, ALTs, and we do it like every three months or so. They have a lot of like GI side effects like nausea, diarrhea, people swell up. So those are the things that are common that you'll see. Then last

Is it could be the process cyclin analogues and those are the ones that can be I V, PO, or inhaled, sometimes subcutaneous as well. Those are kind of like RB Gurgun. Epoprosterno is the only one of all these meds that I'm talking about that have shown mortality benefit. And those cause the same thing. that I've mentioned from all the others, but in addition to that it can cause

local erythema and in duration and sometimes if they're subcutaneous people they look like they have a cellulitis but it's just like in duration from the site where they're injecting. I'm saying all the bad things, but they're very good drugs. Um and then last but not least, we have two, the processitane IP receptor agonists, Selexipact, and The other one is Rio C Watt, which is uh the one that we talked about for uh C Tup specifically, that's the one-lade cyclist stimulator.

And that is the same as the nitrates. You cannot combine them because they act on the same pathway. A few weeks ago we had a patient that went home, took the Rio Cua. on top of her isortil and ended up in an ICU with profound hypotension because of that because you cannot use both So those are the medications. They're very good in terms of what they do for the pulmonary hypertension, but they do come with cycles. and specifically pulmonary arterial hypertension for th except for the

Rio Sigwat, which you mentioned, that one specifically is for CTEF, but the rest of these were for pulmonary arterial hypertension. Are they used just for the group one or this this group five like multifactorial, is it like case by case for people in that group? Have you ever met anyone in that group, Paul? I don't I don't think I ever have. I don't know. I well, not that has been declared that. I'm you know I'm sure they exist, but I I've not made the diagnosis.

Stephanie, so these meds uh they these are for group group one mostly? Yes, they are for approved for group one. Then for the other group That's when you try to go into the phenotypes, risk assessments, and that's where like I would suggest like referral to expert centers so they can characterize the patient and kind of like tailor therapy. But it would be um just like expert opinion. There's no like FDA approach.

For group five, the the other the other category. Everything else. Yeah. Or three yeah. No, three three may change, but um the other ones. Yeah.

I see. And I guess we're in a rapid fire question round now. The the um calcium channel blockers, people shouldn't just throw those at like everybody with pulmonary hypertension, right? So there's it used to be this acute vasoreactivity testing that you have some people that respond to it, some people that don't. It doesn't mean that you're gonna respond.

It's not part of the guidelines, but people do it. They do this is a cath lab test where they test for vasoreactivity and they're giving calcium channel blockers in a cath lab. So you give like adenosine in the cath lab a very high doses and basically some people will have an improvement in the resistance and an improvement in the cardiac output.

So those are the calcium channel blocker responders. It's a very, very tiny, tiny, tiny group. So it's like an orphan be inside the orphan, inside the orphan disease. And they respond. Okay. But it's gotta be super satisfying when you finally find that one patient. Like that has to be like finding a unicorn. That's yeah. It's probably yes. Okay. So it's not that the calcium channel blockers can be are like contraindicated.

necessarily for for anybody with pulmonary hypertension. It's just that like that you're saying the vasore reactivity test is no longer really done routinely. It's not part of the guideline recommendation. No, it is done routinely, but like the calcium channel blockers is for that specific group that responds. Okay. Not for everyone that has pulmonary.

Got it. But if you need it for hypertension control in in some patients, then that's a different story. We're just talking about if you're specifically using it for the pulmonary arterial hypertension, that's a very specific Exactly. Yeah. And we have more potent vasodilators than the calcium channel blocker itself. So the practice is to include the other therapies in people that have D A If Deb is taking care of patients, she's in the ICU and someone comes in super sick.

hypotensive, they have pulmonary arterial hypertension, they're on like some prostocyclines. Should she stop them because they're hypotensive? Please don't stop them. We just had to say it again. Yeah. Cause you know, it seems uh so can you so they don't they lower blood pressure in the lungs, but you're saying they're they're not responsible for this person's hypotension?

So they could definitely because some of them you have a systemic effect, but if you are so sick that you need a prostacycline. It means that you are probably in right-sided heart failure and severe pulmonary hypertension, and stopping your medications right away can cause rebound hypertension and it can be failed.

Yeah. So like their pulmonary blood flow a c their blood flow across the pulmonary arteries could just stop. Yeah. It's not good. Yeah, it's we're not gonna do that, right? Yes, please don't do that. That's a good tip. Okay. You know, I I think the audience maybe they've heard that before, but I I feel like it is a public service announcement, uh, for you to say that on on air for everybody.

All right, well this is this has all been extraordinarily helpful. I do require closure for poor mister Valley, our twenty one year old, so we've we've talked about the diagnosis and potential management for him. But doctor Gorth, don't leave me hanging. What what happened with our patient? Please tell me that he's Playing football after his open heart surgery, question mark. His head warmed up after they um

He did not get back on the football field, but he was caught by his PCP, referred to an expert center, and did undergo surgery. So He is walking around, not passing out, but walking around not on a f not on a football field. He focused on his studies, Paul, and now he's gonna become a chemical engineer or something. Or a heart failure cardiologist. There you go. There you go. I think it's a very interesting case and I'm sorry that he's not playing any That's very sad.

But what we usually like to do with these patients is try to get them to their normal routine. I think the take-home home points are like when you're seeing patients with Disney and fatigue, uh syncope. and you are thinking that they have a prior history of thromboembolic disease or any hypercoagulable state, those are people that you wanna escalate their care.

And try to refer them to an expert center because there may be an underlying process that actually has a cure and you can do them well by escalating care. All right.

This has been another episode of the Curbsiders, bringing you a little knowledge food for your brain hole. Yummy. Missed opportunity. Get your show notes at the Curbsiders dot com and while you're there sign up for our mailing list to get our weekly show notes in your inbox. Yeah, but I thought you were just I thought you were didn't want to do it, so uh I'm anti yummy, so it's really hard to be thrust into this yummy roll. How dare you?

Shamping at the bit, but yeah, it's a shame. Well, we're committed to providing the audience with high value practice-changing knowledge, and to do that we want their feedback. So they should subscribe, rate, and review our show on Apple Podcasts. They can email us at the Curbsiders at gmail dot com. Special thanks to our producer for this episode, Deb Gorth, and to our wonderful guest, Dr. Estefania.

Oliveros, and to our social media team, Beth Garbs Garbotelli on Twitter, Maddie Mad Dog Morgan on Instagram, Tima Karganov is on the website. Chris the Chewman Chew is on Facebook. A reminder to the audience that they can get CME credit for free through Vcu Health Continuing Education at curbsiders.vcuhealth.org. And with all of that, everybody. I've been Dr. Matthew Frank Wado. And I've been Dr. Deb Gorth.

In um is the funny arms. You you said it, I don't know why we included our guest in the sign out, which we never do, but I'm glad that you're there. And We would be remiss if we did not thank the great Stuart Brigham for composing our theme music. We should also thank the amazing Claire Morgan of Notterly for editing our audio. And as always, I remain Doctor Paul Nelson Williams. Thank you and goodbye.