Building The GW Pulmonary Hypertension Program

Dr. Mardi Gomberg-Maitland discusses pulmonary hypertension and the GW Pulmonary Hypertension Program.
Building The GW Pulmonary Hypertension Program
Mardi Gomberg-Maitland,MD
Mardi Gomberg-Maitland, MD, MSc is a cardiologist and Professor of Medicine at the GW School of Medicine & Health Sciences and is affiliated with The George Washington University Hospital. She also serves as the Medical Director of the MFA/GWUH Pulmonary Hypertension Program and will build a comprehensive management program to include outpatient and inpatient diagnosis and treatment, noninvasive and invasive testing, and state of the art Pulmonary Hypertension medication options.

Learn more about Mardi Gomberg-Maitland,MD

Dr. Mike Smith (Host):  Pulmonary hypertension is high blood pressure in the arteries to your lungs. It’s a serious condition that requires specialty care. Welcome to The GW HealthCast. I’m Dr. Mike Smith and today’s topic: Building the GW Pulmonary Hypertension Program. My guest is Dr. Mardi Gomberg. Dr. Gomberg is Medical Director of the GW Medical Faculty Associates and the George Washington University Hospital Pulmonary Hypertension Program and Professor of Medicine at the George Washington University School of Medicine and Health Sciences and is affiliated with The George Washington University Hospital. Dr. Gomberg, welcome to the show.

Mardi Gomberg-Maitland, MD, MSc (Guest):  Thanks for having me.

Host:  So, I know most of my  audience has heard of regular hypertension, right, I don’t think a lot of them have heard of pulmonary hypertension. Can you tell us what that is and how common it is?

Dr. Gomberg:  Sure. So, like you said, most people think about what we call systemic or blood pressure that we measure with the cuff in the office. That’s pretty common and folks know a lot about it because it can cause strokes and cardiovascular illnesses that are pretty common within pretty much everywhere in the world.

However, pulmonary hypertension which is a high pressure in the lungs; is not as common but as dangerous to have. Because when your pressure gets high in the lungs; it backs up to the rest of the body. So, let me explain that a little bit more clearly.

So, really the heart and lungs is just a plumbing system with the right side of the heart in the middle of the lungs and then the left side of the heart. And it just pumps basically, you are pumping blood into the lungs, your getting oxygen and then you are pumping it into the heart and then it goes from the heart all the way to the body all around and it just keeps circling back all around in a nice system.

But what happens is, if the pressure gets high anywhere in the circuit; but especially in the lungs; then the heart has to work that much harder to pump through the circuit. So, if you think about your sink getting clogged up; what happens when it gets narrowing and you can’t get through; the water just starts to overflow and that’s when the heart starts to fail when it’s in people, and patients develop what we call heart failure.

So, actually, although it’s pulmonary meaning lungs high pressure, it actually is a disease that affects the heart and the lungs together. And that’s why as a cardiologist, I’m doing pulmonary hypertension as my specialty but my colleague here Dr. Ahari is a pulmonologist, and this is why we have a really nice team because we have both a cardiologist and a pulmonologist to care for these patients.

Host: Right. I definitely want to get into the uniqueness of the program that you direct. But before we go there, what causes this in most people?

Dr. Gomberg:  Yup. So, there are five different classification groups of high pressure in the lungs because there are a multitude of things that can affect the lungs and the heart to cause the pressure to go high. So, the most common is actually any type of lung disease, anything that affects the lung tissues or affects the ability of the lungs to work and utilize oxygen.

That’s over, within the US and Europe, over seven million people as a prevalence. Compared to left heart disease which is when there’s something wrong with the actual heart whether it’s the valves or the doors to the heart chambers or the heart itself which can cause a backup into the lungs and the pressure to elevate. That’s about five million in prevalence.

But then it becomes a little bit less clear what the actual numbers of patients that have the other two types because they are not as common. You have pulmonary arterial hypertension which is what – one of the things that we are providing care for that’s not that common. It’s actually considered an orphan disease meaning that it’s relatively rare in the spectrum of things when it’s only isolation of the narrowing of the blood vessels in the lungs. And so that’s really not very common.

But what we have found over time is that there’s an association between disease states that can affect the lung’s blood vessels as well. For example, connective tissue disease or scleroderma or lupus which can be quite common; there’s a certain percentage whether it’s 30-40% of these patients that can develop high pressure in the lungs.

So, when they estimate the prevalence, it’s really not completely accurate because we’re just going by records of recordings at offices but we’re in a much lower number where it’s about a quarter of a million. And then finally, the other clear cause of high pressure in the lungs would be just blood clots in the lungs. Which we have been hearing a lot about lately because we actually have therapies for that which we didn’t have in the past. And that’s another type of patient that we see here at the Pulmonary Hypertension Program.

Host:  So, just to kind of summarize all that on one hand, you can have disease in the lung itself that can cause that increase in pressure, back up to the heart or you can have disease on the left side of the heart which then backs up also into the lungs. And then there are certain conditions like you mentioned connective tissue diseases that can increase the risk for pulmonary hypertension. So, what’s the work up then with all this. And I think what a great opportunity Dr. Gomberg, to really focus on what the GW Hospital and the GW Faculty Associates Pulmonary Hypertension Program is all about. I know this is a very great clinic that you direct so, why don’t you walk us through how you guys manage this, how do we diagnose pulmonary hypertension? What’s the treatment and what are the outcomes that you see?

Dr. Gomberg:  So, what the most common symptom that the patients present with is shortness of breath when they are walking. This is because the heart is trying to work harder, just the heart rate should go up, your blood pressure goes up in anyone who does any exercise. But in a normal person, you are able to sort of compensate so that you don’t get super high blood pressure in the body or in the lungs.

However, when you have narrowing in the lungs, you can’t do that. And so, the patients now their oxygen levels go down and they get trouble breathing. So, that’s the most common presentation. And the issue with that, is that shortness of breath is a pretty common complaint and so most folks are not going to think about pulmonary hypertension. Because common things are common and this one isn’t.

And so, what we find, and what we find across the country is that patients present to pulmonary hypertension specialists late in their disease process. Because they are often diagnosed with more common conditions such as asthma or obstructive lung disease or even just anxiety. And that hasn’t changed over time. Really for the last 15 years, we still see the same referral patterns.

And so, one of the things that’s really important is that you get the standard testing for patients when they don’t get better especially when you treat them for things that are common. So, you give them an inhaler and they are not getting better, you don’t say oh well, I guess it’s anxiety. You say okay, what else could this be? You should have an electrocardiogram. You should have a chest x-ray. You should have a full exam. We should look and make sure that the person doesn’t have what we call a screening blood test for connective tissue disease or an ANA, or thyroid disease. I mean you basically want to start thinking about it sooner.

But the best screening test is really an ultrasound or an echocardiography of the heart and that’s because it’s our first way to estimate blood pressure in the lungs. Because unlike systemic blood pressure where you can put the blood pressure cuff on your arm; you can’t do that in the lungs. And the echo allows us to estimate the pressure by looking at the blood flow across two of the chambers of the heart so across the tricuspid valve.

And what that means from a nontechnical standpoint is that if it’s high; then there’s a possibility that the pressure is high in the lungs. It’s not a perfect test and it’s not as exact as the cuff; but it’s a really good screening. It also allows us to look at the different chambers of the heart, so both sides, the right side and the left side to see how they are functioning. It allows us to look at the heart valves which could be one of the causes of high pressure in the lungs and it actually gives us a sense of how large the blood vessel or the tube, the pulmonary artery is because we can see a part of that even on the ultrasound screen.

So, I think that it’s getting the appropriate work up and not just thinking that it’s all shortness of breath that’s only lung related but also thinking about the heart in itself. Even in young folks.

Host:  Right. Well I was just going to say so when you go through that workup, as someone comes into the clinic and they go through all of this, I mean at the same time, you are also looking at if there’s signs of that high blood pressure looking at what might be that cause, right so you mentioned some of the other screens that they might do like the connective tissue screens. Do you do that in the clinic there or is that something that they would do through their own like primary care doctor?

Dr. Gomberg:  Yeah so, what we’ve developed recently in our guidelines, so the pulmonary hypertension committee met in 2018 at the World Symposium and what we changed in our guidelines actually addresses exactly what you are discussing. Which is we actually don’t want you to wait to refer it and get all the testing. We want you to refer it to a specialist if you are even thinking about it. And that’s because we find that most centers and internists, pulmonologists, cardiologists don’t get all the tests and if you don’t get the appropriate diagnoses as far as the etiology of the high pressure in the lungs; you may actually completely treat the wrong disease and you can cause more harm.

So, we did a study when I was at the University of Chicago actually looking at our referrals with the University of Michigan and Baylor and looking to see how well we do with diagnosing on the outside, getting all the appropriate testing and if the diagnosis changed when they were sent to a referral center and we found that more than half the patients were given medications for the arterial where there’s just narrowing of the blood vessels when they didn’t have it.

And so, I say that with caution because we think oh it’s just high blood pressure, we can treat it. But we really want you to refer it because this disease has no cure. It’s fatal and so you don’t want to treat inappropriately, and you want to treat exactly what’s going on with the patient because you don’t want to be causing more harm.

Host:  How many patients do you see in the Pulmonary Hypertension Program at GW Hospital?

Dr. Gomberg:  So, this is a new program. I just got here in January. So, they’ve been seeing – yeah, very new. I formerly was running one of the largest programs in the country at the University of Chicago where we had 300-400 patients. I think that right now, we are sitting between 50 and 100 but we are starting to really consolidate all the little sort of pods of people that were seeing these patients. So, now there’s direct referral from our rheumatology colleagues, as I said Dr. Ahari has patients that now are going to become part of this program and then Dr. Panjrath, who is our heart failure – advanced heart failure expert has also been seeing them.

So, now we have a consolidated program of multiple sections within medicine so that we can really grow this program.

Host:  Right. You had mentioned that – this is obviously a very serious disease. So, what are the treatment goals when you are faced with pulmonary hypertension?

Dr. Gomberg:  Yeah, so right now, our treatment goals are to make the patients feel better, to give them better quality of life, better exercise tolerance and to live longer. And that’s really always our goal in medicine. We have recently shown that our medications are able to do that. Which is something that’s taken some time to do. We haven’t been able to completely reverse the disease. But we’ve given patients that used to be given a six month prognosis, some are 15 years out from the diagnoses. And so the goals are really, they’ve actually set what we call low risk criteria that we are all aiming for.

So, one would be minimal symptoms when you are climbing up the stairs and doing extreme exertion. The second would be that these patients have as close to normal pressure in the lungs and function of the heart. So we want the right side of the heart to be back to normal size and not enlarged and not working. And then, we want to see that they can really walk at their capacity based on their age and weight. So, we have different cut offs for what we call a six minute walk test where you walk as far as you can in six minutes, and we’d like to see that patients improve such that they are not limited in their everyday activities.

Host:  Right. Dr. Gomberg, this was a lot of fascinating information. In summary, what would you like the audience to know about pulmonary hypertension?

Dr. Gomberg:  I’d like the audience to know that pulmonary hypertension is as important as systemic hypertension. It causes heart failure and it really limits the patients and their ability to function and that don’t dismiss shortness of breath and feeling run down as just getting older. Everybody should go in and see their internist and get routine checkups because I think a lot of times folks wait until it’s far late in the disease and then there aren’t as many options that I can provide.

Host:  Right. Very good summary Dr. Gomberg. I want to thank you for the work that you’re doing. And thank you for coming on the show today. You're listening to the GW Healthcast. Please visit to get connected with Dr. Gomberg or another provider, or call 1-888-4GW-DOCS to schedule an in-person or virtual appointment. . I’m Dr. Mike Smith. thanks for listening.