Practical PH - Foundations of PH - Diagnosing PH

Nov 21, 2025
Tune into Practical PH’s inaugural episode of the Foundations of PH series with PH expert Dr. Namita Sood who takes us through a systematic approach of accurately diagnosing pulmonary hypertension.    

00:00 — Podcast Welcome & Series Overview
01:05 – Guest Introduction & Journey Into PH
03:23 – Patient Case Introduction & Key History
06:20 – Physical Exam Findings & Early Risk Assessment
09:04 – Essential Diagnostic Approach
11:40 – Workup Results & ILD Interpretation
15:01 – Next Steps: ILD Care & Right-Heart Cath
17:01 – Group 3 PH Evaluation & Management
20:08 – Treatment Options & Inhaled Treprostinil
22:46 – Key Takeaways & Closing

Creators and Guests

Host

Kathryn del Valle, MD

Assistant Professor of Medicine Mayo Clinic, Rochester

Host

Khushboo Goel, MD

Assistant Professor of Medicine Mayo Clinic, Rochester

Guest

Namita Sood, MD

Director, Advanced Lung Disease Program, Co-Director, Pulmonary Hypertension, Program Professor of Medicine University of California Davis

What is ATS Assemblies & Sections?

Discussions about pulmonary, critical care, and sleep medicine from our Assemblies & Sections.

You're listening to the assembly on pulmonary circulation podcast, brought to you by the American Thoracic

Society. Welcome back everyone

to Practical PH, where we invite experts in the field to talk about a wide range of topics within pulmonary

hypertension, with a particular focus on providing practical pearls. My name is Katherine Del Valle,

a PhD specialist at Mayo Clinic in Rochester. And I'm Khushboo Goel, a

PhD specialist at Cedar Sinai and LA. So we know it's been a while since our last episode, previewing

all the pulmonary circulation sessions at ATF. But we're back today with the new series called Foundations of Yeesh. We're lucky to be

joined by Dr. • Namita Sood, Professor of Medicine at the University of California, Davis. She's the co-director

of the pulmonary hypertension program and the director of the advanced lung disease program at UC Davis.

And today we'll be talking about how to evaluate and diagnose pulmonary hypertension and how to connect it to

experts each other. So talk to us

who do we like to start practical pH by asking our guests about their journey into the world of pH.

So how did you become interested in pulmonary vascular disease and what excites you the most about the field?

So for me, it wasn't like an active choice. It just happened. I was the first to you fellow at the

University of North Carolina. My focus was microbial epitilial interactions and ventilators

associated pneumonia. And kind of planning to be an ICU dog when I diagnosed a 60-some-year-old

lady with idiopathic traumatic hypertension. I was like of course it was a fellow's clinic but I was

like the third or fourth pulmonologist she was seeing. And at that time, I sort of suggested

to her recenter to one of the centers, Dr. Lurubin actually in Maryland and she said she'd seen too many doctors and she'd

rather stay here. So that was my first homely archival hypertension patient that I treated with epoprost mal.

And from there, by the end of my first year at five patients and by the end of my fellowship, we established

a PH clinic at UNC. So, and then I kind of got hired to the Ohio State

University to set up the polyvasculacy. So that's what I focused on for the next 17 years of my life. So that's

how it all started and developed. That's awesome. Thank you for sharing.

So we'll get into today's episode and topic by starting off with a patient case as we always do.

So Dr. Sue, you're seeing a new patient in your clinic who's been referred to you for pH. She's a 45 year

old lady with hypothyroidism and a history of unprovoked DVT on a do-ac with a chief complaint

of progressive shortness of breath, legs, swelling, and a chronic dry cough. When you look at her outside

records, you see that the reason for referral is an echocardiogram which report a VA systolic pressure of 50

and says, "sivier pulmonary hypertension in the interpretation." But before we get to ahead

of ourselves, we know that the journey to any diagnosis always start the good at HRI. So, what questions

do you ask patients who are referred to you for possible pulmonary hypertension? So, I'm going to start by saying

that, you know, even after years of doing this, the hardest thing in pulmonary until hypertension is to

appropriately phenotype the patient. That really is, it's all about the workup, right? It's

all about getting a good history and the workup. So, I take a few shortcuts because I give my patients

a detailed questionnaire. It, you know, even before they see me and clinic, everybody gets this questionnaire

that they have to fill out. I'll which kind of is a spread kind of structured initially starting

with their symptom, which then when I'm sort of translating that to my note or sort of

assessing the patient, it gives me an idea as to what their function class or their risk of eradication is going to be should they have here. And then

followed by the past medical history with sort of specific questions regarding any autoimmune disease and

so forth. There, it was your history tobacco, previous chemotherapy, drug,

it's recreational drug use. So it just kind of goes into this whole thing because I think poultry hypertension is not a disease that

is sort of in isolation, right? It is a manifestation of a disease in the lung that could be related to many

systemic diseases. So knowing if they have systemic hypertension, have they had cancer before, have

they had, I do they have renal disease. All that is so important to us. So I kind of take that shortcut

and I just get that captured, proactively from the patient. So when I'm doing my note, all that information isn't front

of me. In case my visit was hurried or I forgot to ask the question, it is there for me to review when

I'm actually putting it in my note. So I think a good detailed history. I feel like

rheumatologist, nephrologist, all this take better histories than us. And I think we just need to be in that club and get a very

detailed history. Um, a studying out.

And so I think, it's best if we're going to be asking about equations, drug use, or other associated illnesses, especially hypertension, sleep apnea. We know

disease, blood issues, feverish chemotherapy or immune disease. I think that's

all very important. So I think we just need to get a very detailed history like a good internal in every patient. Absolutely.

I think that's so, so important. And I'm so glad you underscored the importance of the history. Because

I think a lot of times our patients will tell us where we should go. Where we should go if we listen carefully. That

gives you some more information about this particular patient. She says that her shortness of breath has gradually worsened

over the past few years. Coupled with some issues with bilateral leg swelling for what she's needed some lacex. She also

notices that her hands and fingers are very sensitive to cold weather and tend to turn red or white. And that she has some

difficulty swallowing solid food. She has a 15-pack year smoking history and quit about 10

years ago. She denies any recreational drug use. On exam she

has some fine and literary crackles along bass. I'm trace-pitting a demo along her shins.

And some tightening of her skin when she tries to make a fist. Based on her history and physical

exam, what do you think her biggest risk factors are P.H.R. and what other work-up should we do? So I think

you're presenting to me a case with symptoms of escalarderma, and it could be any other sort

of overlap what I mean to these with that. So the work-up again, it doesn't matter where

the patient starts, right? So this one sort of seems like a very easy patient that, you know, the patient has clerdermine, now she had an increased

RBSP. And of course she's at high risk of developing primary hypertension, so bingo, we have the diagnosis, but unfortunately, even

in the patients where things seem this obvious, it is not that easy, right?

So there is no shortcut to a proper workout, right? Everybody needs the

complete work-up. It doesn't matter how simple things appear. So the echo, which

is a usual screening test, to consider if the patient might have primary hypertension, hypertension is not a diagnostic test.

It is a screening test. And we want to use that echo to look at several things here. Number

one, I start actually at tell my fellows in all of them. Like you look at the left side, right? You know, what is the ear on

that reported on that? What is the left atrium looking like? Is the mitral valve healthy, right?

And then you look at the right side of chambers. Is the right atrium enlarged? Is the right ventricle enlarged? Is the septon?

Is there a percode refusion? And then train your eyes to that TRJ philosophy that everybody gets focused on. Right?

And then you have basically looked at the echo. You want to include left hot disease. You want to see if you want to worry about palm rehabitention.

And even if the RBSP is not that increased, but if my right side of chambers are enlarged, I am worried.

Or if my septum is buoying into the LV during sisterly, I am very, very worried. So you got to look at the whole

echo. And we use it to say, am I worried about palm rehabitention? But also we use it

to say, what is the state of my left hot? Because that is the commonest reason for elevated a peer

pressure. So starting with the echo, then I hope in our history, we got symptoms of

it. That might suggest airway or pernkimal lung disease, right, does the patient have a chronic heart?

They, you know, copying a serum. They had a history of tobacco use. But after that, you need full set of primary

function tests on this patient, a physical exam. If there is any risk factors, any suggestion that the patient might have

pernkimal lung disease, or any risk factors that they might have pernkimal lung disease, these patients need or high-resolution

CT scan to look at the pernkimal, right? Traumatic disease is never going to announce itself till we look for it.

So everybody gets a ventilation profusion scan. And then our routine labs, we want to look at our electees, we want to

look for HIV, it's a treatable disease. So no matter how you cut it, how many years you do it, that work up has to

be complete. And the way we do this again, many years ago, I told you,

I started as a fellow, so I was very afraid. So we created this little cheat where, you know, we would complete the whole

thing, you know, the patients information, their exposure, and then their echocardiogram,

the VQ scan, and then fill it up before the patient underwent to write-out care. And that bits

of that have now been embedded into my epic note. So when we get patients from

outside, we incorporate the data we have, the missing data we ordered before we kind of arrived at

a diagnosis and treatment for the patient. And you just have to set up some process because otherwise no matter how good you are and

how long you've been doing it, it is hard to keep track of if the work up has been complete or no.

And the problem is that if you phenotype the patient incorrectly, that patient is not going to get better.

So it's a lot of wasted effort and time and expense at every end. Yeah,

I think you make two really good points about how systematic we have to be with pH, which is that don't make any assumptions

and really go through each of those WHO group classifications. And all the subclassic and

really just we have to evaluate for everything. And then the second important point was that the Echo

Cardiogram is not a formal diagnosis, but it really does help you restratify how worried you are

about pH. So Catherine, I guess you want to give us some more information on this case.

Okay, absolutely, I think we got most of the information that Dr. Sudrecomended. So she,

this patient has an estimated culinary arterial systolic pressure on Echo of 50. The

Echo also shows a moderately dilated right ventricle with mildly reduced function and RV pressure

and overload suggested. She has a normal left ventricular ejection fraction. So overall,

this is pretty concerning for pulmonary hypertension, especially when coupled with her history and physical exam. So you proceed

with all of the workup we've talked about and that reveals a positive A&A green, a positive

SCL70 antibody, a pulmonary function testing that demonstrates moderate restrictive defect and reduced

diffusion capacity. And she has a high resolution CT chest which shows a UIP pattern with basilar

predominant reticulations, honeycombing, and traction bronchiexticit. You also go ahead and prescribe

deadual glycec, which she's used in her many in the past. So you do all of those

things and fast forwarding to a follow-up appointment. At that point, she's dramatically

about the same, but the adema has resolved a bit with the glycec. So at this point, Dr. Seud, what do you counsel

this patient about and what are your next steps to diagnose her? So I think this is looking

like a patient who has clad or map based on the autoimmune

profile and the physical findings you suggested with the interstitial lung disease. And she may or may

not have pulmonary hypertension associated with that. So I think several things need

to happen at this time number one. I want to be sure that she's established with the rheumatologist and we're addressing the interstitial

lung disease. And I want to be sure that we have assessed her for at needs for

supplement loxogen, you know, with her walkest and so forth and optimize that. I want to be sure that she doesn't

have any symptoms or, you know, to suggest that she might have sleep apnea if she has then

I would address that as well. And then she needs a right-hat catheterization

to assess her hemodynamic to arrive at a diagnosis of, you know,

probably have tension if she has it or no. It is a pre-capillary or pre-imposed capillary and

so on. And how does your pre-test probability for

what type of pulmonary hypertension or clinical classification affect how that right-hat cath is done? I think sometimes

we don't, we forget that it's not just getting resting quite right-hat right-hat cath numbers. We might do other maneuvers

whether it's a reactivity touching or a fluid challenge or a shunt run. So do you mind just

commenting a little bit about that and what some of those tests are and in which scenarios are those actually

relevant? So maybe in this case maybe not in the case. Yeah, so at the Razodality Challenge which is the

the communist one we use. So the question that test as is, is my patient going to be a

candidate for calcium channel blockers? Right? So if you have a patient that you think has idiopathic, permeatial

hypertension based on your initial workup, the patient still has good RV function. And in that

case the Razodality Challenge within Hail Nitric Oxide is indicated to

see if the peer pressures drop down and without dropping the cardiac output. In that

case that patient could benefit from calcium tonne block. So that's the Razodality Challenge. It's only

been validated and studied in patients with idiopathic permeatial hypertension, not in any other type of our PH.

Now the other one that is frequently being used, because remember the commonest cause for

permeate hypertension is left part disease. And where we get very very confused as PH physicians

is when we have elevated peer pressures, but the left side of the heart doesn't look

normal and the wedge is right at that cause. So the normal LVEDP is actually less than 10

and most people, right your wedge is supposed to be 10 or less, but then we give it 2 standard deviations away and we say 15 is good.

So when it's sitting right at that 15 or 14, my LV on the echocardiogram looks a little bit different.

The patient has a longstanding history of systemic hypertension. The left syndrome looks mildly dilated. And my peer

pressures, my mean peer pressure is 35 or 40. So then I'm confused. You know, it's the patient. Pomeratial hypertension

or my brain is screaming that there has to be an element of left heart disease, but I'm thinking

that numbers are looking like recapillary. So in that case, you could challenge the left heart a little bit.

Right? And the way you can do it is by doing a leg race. Basically, when you do a leg

race, you immediately improve, increase the return to the, to the venous system.

And with that, if the wedge goes up, that suggests that the left side, the left side,

I may not be that, that healthy. Right, or you could do a fluid challenge, where you give 500

CCs of fluid, and see what happens to your, your wedge pressure. The third set setting

could be doing exercise testing, where you exercise the patient, normally the peer pressures should

go up. The cutting code puts you in a healthy patient. The peer pressures should go up. The cutting code put should go up, and

your PBR should actually come down. In patient with EH, the peer pressures would go up. The cutting

code put would drop, and your PBR would go up, but your wedge would

say about the same. But somebody with left-hout disease, the peer pressures might go up, and the wedge kind of goes up

at the same time, and the cutting code put could drop. If you continue with the exercise, so that way you're sort of trying to separate out, where the

pathology is, and what is the pressures being, what is driving

the pressures. The challenge with these challenges is

that, the ways of daily to challenge in PH is the easiest one. Everything else is a little bit

nuanced, so you really need to sort of be mindful of getting all the numbers together, looking

at the waveforms very carefully, and sort of picking up the right patient. How the

Pomew Vascular Bed Responds inpatient who has interstitial lung disease, with these challenges, has

not been studied. So we really don't know, especially you know, patient with blood or mal, all the patients,

you would be worried that maybe there is left heart involvement, but if they have interstitial lung disease, how to use

and interpret these challenges, it's not been well studied. So I would discourage

people from doing them at this time. Absolutely. So it really seems like we have to not only obtain a history

with intention, in exam with intention, but also cast with intention to make sure we're putting things together, directly.

So from the important, I'm going to make one other point. The important thing with the cat is really to look

at the waveforms very carefully, because a lot of times they get interpreted just by the

computer reading, like drawing the line, and then we do the challenge, and then we just follow whatever is being read.

But if we pay attention to the waveforms, and what is the variation that is happening with that challenge, or the exercise

or the breathing, then I think you'll get a little bit more information out of the tracing.

Absolutely, that is such an important point. So in our patient, you know, she undergoes the right-heart cat,

and her numbers, after you've reviewed the waveforms and made sure everything is correct, her mean pay pressure 32

with a wedge pressure of 8, her thermo-dolution cardiac output is OR, which result

in a thermo-dolution pulmonary bachelor's of about 6 videos. So this is confirming

the diagnosis of a pre-capillary pulmonary brain. And then the next step will lead a term in a treatment plan for her Ph.D.

based on what we think are primary, who grew clinical classification in it. We'll talk about the treatment approach, kind of

more in a future episode, practical Ph.D. But today we wanted to focus on this process, getting the

Ph.D. So to conclude, to this patient has Ph.D.

the setting of the scleroderma, right? So here we started thinking that this would probably

be a slam dunk. Ph.D. related to scleroderma, but when we kind of looked at the patient a

little bit more detail, she does have interstitial lung disease as well, so she kind of falls into that group 3 C.D.L.D.

will pay Ph.D. - Exactly. And so for our audience listening

and all the clinicians out there, we wanted to highlight a couple take-home point for this particular

case. So one is, as we've highlighted already, it is how important it is to be systematic about

evaluating for PH risk factors through a good history, physical, and other standard diagnostic studies.

So no cutting of corners. And the second is to remember that the formal diagnosis of PH always

relies on humo dynamic data, via a right heart catheterization and coupled with a WHO group classification

based on your work. So we shouldn't be starting pulmonary visa dilator therapy based

only on an echo. And as we found in this case, the treatment approach can be very different

depending on the type of PH, which you can assess only with an echo. As doctors sued so nicely

outlined for us. And another thing I wanted to bring up in this discussion

was that your doctor sued, you have the perspective of being a PH specialist at a PH accredited, you know,

center of comprehensive care. So can you talk about when patients should be referred to expert PH centers,

and how physicians in the community can really find where to refer? So,

thank you. So, somebody did this. They asked how to manage a PH patient, and one of the top things that

came out of referred to a PH center. Which would be great, right? But it's not always practical.

The insurance companies don't sometimes, you know, approve the closest center. That is available

for that patient. Sometimes travel, expense is a problem. Sometimes patients just don't feel comfortable coming into cities and

so forth. So, we have to, you know, I think we as a PH centers, yeah,

it centers, we have to reach out and partner with the, make ourselves aware to the physicians in the community. And then

be available to them as a resource at whatever level they need us to be. Sometimes people

will do the workup and they send them to us where right-hat cat. Sometimes they will do the full workup and they just want to run the patient

by to get, like, what would, what should I start the patient on treatment? Or they will do the initial treatment and if the patient's

not improved or they just, they start the treatment and they just want to check in for us to see that if the patient is

appropriately treated. So I think we have to make ourselves available to the physicians

in, whatever capacity they would like us to consult on. And I would say to the community physicians, like,

you know, this is, it is hard, even at the best of centers, it is sometimes hard to get a comprehensive workout in

a timely manner. And we are happy to help them with that.

And they should have a lower threshold to send the patients over to us. And, you know, the PHA has a very

nice website, where they list all the centers, the reach milk centers, the centers, comprehensive centers of care,

where the information is to how to read them. And that is a resource for

all the community physicians to use. Well, Dr. Sue, thank you so so much for sharing your expertise and time

with us today. This has been a phenomenal session. I know I learned a lot and I'm sure our audience did as well. So thank

you so much. Thank you. And you are a listener. They tuned for our next episode in this

foundation, Which will be about wrist gratification of each with

another very special. Thank you for joining

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