Recognizing Pulmonary Hypertension

[00:00:00] Welcome to Heart to Heart Nurses brought to you by the Preventive Cardiovascular Nurses Association. PCNA's mission is to promote nurses as leaders in cardiovascular disease prevention and management.  

Geralyn Warfield (host): Welcome to today's episode, the first in a three-part episode mini-series on pulmonary hypertension. Today I'm joined by Traci Housten and Lisa Maher to discuss recognizing pulmonary hypertension, and I'd like our audience to know a little bit more about who we're speaking with today. So, Traci, could you please introduce yourself?  

Traci Housten (guest): Thank you, Geralyn and, thank you to PCNA for inviting me to participate in this great session on pulmonary hypertension. 

I am a nurse with the pulmonary hypertension program at Johns Hopkins in the great city of Baltimore, Maryland. At Johns Hopkins, our pulmonary hypertension program is based in the Department of Pulmonary and [00:01:00] Critical Care, but we have close collaborations and relationships with people from multiple disciplines to care for patients with pulmonary hypertension and their families. 

I started working in pulmonary hypertension all the way back in 1995 and got my job completely by accident. Probably like some of the listeners, I had no idea at the time what pulmonary hypertension was, and back then there were only two types of pulmonary hypertension and one medication. And, we have certainly come a long way. 

Since that time, I've been fortunate to work with lots of brilliant and dedicated colleagues all over the country to expand our understanding of this disease and increase medical treatment options for patients, and to the point that we currently have many medications given by multiple delivery systems, in addition to clinical trials of additional medications as [00:02:00] well. 

In addition to my clinical and research work at Johns Hopkins, I've also been fortunate to have leadership roles with the Pulmonary Hypertension Association, and work to develop specialty organizations for allied health professionals working in this field and identify centers of excellence in PH care in the United States. 

But honestly the people who have taught me the most in this job every day are the patients, and the grace and courage that they display, taking care of themselves in this, in the face of really uncertain futures with a serious diagnosis. And unfortunately for many of them still, even with all our advances, many of our medications are really just palliative, and only work for short periods of time. 

And so, there's really a lot more work to do in recognizing these patients and getting better treatments for them in the future.  

Geralyn Warfield (host): Thank you so very much for that great introduction. Lisa, could you tell us about [00:03:00] yourself?  

Lisa Maher (guest): Yeah. Thanks Geralyn, and thanks also to PCNA for allowing me to participate on this podcast. 

I'm a nurse practitioner with Unity Point Health in Waterloo, Iowa, and I work at the Unity Point Health Allen Hospital Cardiovascular Clinic. I'm currently the president-elect of PCNA, and also the chapter leader of PCNA Cedar Valley, Iowa Chapter. I work in a general cardiology setting and I've been in practice approximately 15 years, and oftentimes, I find myself on the first line of defense in finding patients with pulmonary hypertension. 

I've found that it's a condition that a lot of times is easily missed or easily ignored. I found that a lot because I also manage, along with general cardiology, I also manage a sleep clinic, which is in the setting of our general cardiology clinic. And with these sleep patients, a lot of times I, when I'm reviewing their charts, I find the condition of [00:04:00] pulmonary hypertension. Along with these types of patients I also enjoyed preventative care and I have previously worked in our lifestyle medicine clinic.  

Geralyn Warfield (host): Well, I'm sure our audience recognizes the great set of knowledge and expertise that today's guests bring to us. So, let's go ahead and get started with our content and let's make sure that we all are on the same page when it comes to understanding the terminology we'll be using. 

In general, what are we talking about when we use the phrase pulmonary hypertension, also known as PH? 

Traci Housten (guest): Great question, Geralyn, and it's really important to understand that the words “pulmonary hypertension” are not a specific diagnosis, although we often use it that way, and I've probably already used it that way about three times since we started this podcast. 

The words “pulmonary hypertension” are really just a hemodynamic observation that the pulmonary artery pressure or blood pressure in the lungs is higher than what's considered normal. [00:05:00] And we also have a more recently added understanding that the pulmonary vascular resistance may also, must also be elevated for a diagnosis of pulmonary hypertension. 

Those hemodynamic observations are important for a couple of reasons. One is, this information is only available through a right heart catheterization. You can only get data about the pulmonary artery pressure from a heart catheterization definitively. While some of the other testing we do may give the suggestion of pulmonary hypertension or elevated blood pressure in the lungs, the right heart catheterization is the only way to get that data accurately. 

Even with that data, the next question before a patient would get diagnosed or treated is why the blood pressure is elevated or what other medical condition or type of pulmonary hypertension might the patient have. The specific type of pulmonary hypertension is really what should guide treatment and [00:06:00] discussions about disease state and disease prognosis. 

Unfortunately, many patients get the words pulmonary hypertension placed on their chart, but they've never had a right heart catheterization, and many patients can get started on treatment for pulmonary hypertension even when they haven't had this test and a full diagnosis. Your listeners are going to hear more about the information we get from right heart catheterization and the classification system for pulmonary hypertension in one of your next sessions in this series. 

Lisa Maher (guest): Thanks, Traci. I think you did a great job explaining that, and I always love too that when I'm on these types of things that I'm always learning as we're talking and, I always think that's great, especially with nurses working together and collaborating. So, I just want to add a couple of things to that. 

So, as I said before, I work in general cardiology. So oftentimes I get patients referred to me for shortness of breath, [00:07:00] which we know can be a lot of different conditions. But a lot of times what that leaves me to order is further testing. One of these things is an echo, and we know that as Traci has just said, echo is not a gold standard for diagnosis of pulmonary hypertension. 

But when we see those elevation of pressures on an echo, it can clue us that, hey, something's going on, or something may be going on and we need to take a look at what else we can do. So, when I talk about pulmonary hypertension with a patient, I tell them that there's an increased pressure in the lung, which leads us to see some abnormal findings or some elevated numbers on their echo. 

And I let them know that this can affect the heart, so we want to go through and take a look at things. But I also agree that patients do get this pulmonary hypertension diagnosis placed on their chart, and these pressures can [00:08:00] come and go. And so, I do think that's important to understand as well. 

Geralyn Warfield (host): That is all excellent information and I'm hoping that our listeners will be able to learn a little bit more from you about the typical symptoms that we'd see in clinic in a patient that has PH.  

Traci Housten (guest): Well, by far the most common symptom of pulmonary hypertension is dyspnea on exertion, so not at rest, but whenever the patient would start to do any type of exertion, they can get quite short of breath. And unfortunately, that's the most common symptom for many, many other much more common medical conditions. 

So, it's not unusual for patients to spend quite a bit of time complaining of shortness of breath, being short of breath, having worsening shortness of breath before a diagnosis of pulmonary hypertension is made. That shortness of breath with pulmonary hypertension can start out mildly and progress slowly over time. 

That's most common. Or for some patients, it can [00:09:00] actually start fairly suddenly and be severe. Some of the other symptoms, which again can be associated with many other medical conditions, include dizziness along with the dyspnea exertion, dizziness when bending over, lower extremity edema, tachycardia or low oxygen levels, hypoxia when patients are exerting themselves as well. 

Very late symptoms of the disease when the right side of the heart is under a lot of stress, and failure can be ascitesand syncope with exertion. 

Lisa Maher (guest):  I agree, that's typically what I see too as far as symptom, is that they present with shortness of breath and clearly that dyspnea on exertion. Again, I also treat patients with underlying sleep apnea who can also present with a multitude of symptoms including fatigue. And as we have both alluded to sometimes patients when they have that multitude of symptoms, it can lead us in so [00:10:00] many different directions in regards to testing. 

It's important to listen to the patient's main complaint and if they keep having that main complaint, help to try to identify or distinguish those symptoms a little more clearly. 

Geralyn Warfield (host): So, is PH a genetic disease? Is one of the key things that you would do in clinic is to make sure that you have an accurate family history? 

Lisa Maher (guest):   Yeah, family history taking is very important. So, with pulmonary hypertension, inherited gene mutations can cause about 20% of the cases that have no other cause. 

So, if there is an underlying family history or concern for a genetic mutation, referral onto a genetic counselor would prove beneficial. That also helps put some of the family dynamics at ease because if there are other siblings involved or children involved, I think it's also important to referred that genetic [00:11:00] counselor for further information and for further cascade screening.  

Individuals with pulmonary hypertension can meet classification for heritable pulmonary hypertension, or what was previously known as familial pulmonary hypertension. If they meet any of the following criteria. Either one, if they belong to a family who has known, documented pulmonary hypertension, in any two or more individuals or two, if they possess a rare variant, or a mutation in a gene known to address, known to strongly associate with pulmonary hypertension. So, the most common gene cause is present in approximately 75% of inherited cases. 

And this is a gene mutation called bone morphogenetic protein receptor two or BMPR2. [00:12:00] This gene helps regulate cell growth in the walls of the lungs’ small arteries. Inheriting a BMPR2 gene does not mean or guarantee that you'll develop pulmonary hypertension, but again, gene variants do not work on their own, and so everyone with this gene mutation will not necessarily have the condition, but it will be important again to refer onto that genetic counselor.  

Geralyn Warfield (host): So, in addition to making sure that we have an accurate and complete symptom history and an accurate and complete family history within the constraints of the clinical setting and people's memory and information about their family, what kind of physical exam findings in a patient would you see if they had pulmonary hypertension? 

Traci Housten (guest): I'm happy to talk about that. I think physical exam is important in multiple aspects of pulmonary hypertension. When trying to determine if a patient may have elevated blood pressure in the lungs on physical exam, the most [00:13:00] common physical finding is going to be a loud second heart sound pr P2 and potentially a right-sided fourth heart sound with an RV lift or heave. 

However, these things may not even be present in very early or very mild disease. Jugular venous pressure assessment could be elevated, again, maybe not in early disease, but most, even more important are physical findings that might help classify the type of pulmonary hypertension patient, that type of pulmonary hypertension that the patient may have.  

For example, patients may have physical findings of a rheumatologic disease, most commonly in pulmonary hypertension that would be scleroderma. So, findings of Raynauds, telangiectasias, sclerodactyly in patients with chronic thromboembolic disease, you may hear pulmonary flow murmurs or bruits[00:14:00] in the area of the pulmonary arteries. 

Clubbing is an important finding that may direct you to a patient who could have group three interstitial lung disease or obstructive lung disease related pulmonary hypertension or congenital heart disease.  

Geralyn Warfield (host): So, in addition to those physical findings, what type of testing should be done to evaluate pulmonary hypertension? 

Lisa Maher (guest):  I think I'll address this first from a general cardiology standpoint, and then I'll hand off to Traci to go into more guideline measures. But, when we think of general cardiology, again, a lot of times, we're doing a lot of testing for a lot of different symptoms. So, when I have concerns of a shortness of breath or any of these, those other physical exam findings that Traci mentioned earlier, I usually start with an echocardiogram, or an echocardiogram with bubble. 

If I'm worried [00:15:00] about underlying shortness of breath, I will either do a VQ scan or a CTA of the chest. I look at underlying sleep testing, pulmonary function tests, and even a pulmonology referral. Again, a lot of it is cascade where I order one test and something else clues me in. So, then I continue to order on and on. 

Again, typically, because I am seeing a lot of patients with underlying concern for sleep apnea, a lot of times I will order an overnight oximetry and, or a sleep study. And then further testing, as far as a general cardiology, we do right heart caths at the same time typically as we do a left heart cath. At that point, because I work in general cardiology, if I have underlying concern, they get handed off to specialized pulmonary hypertension clinic. 

Traci Housten (guest): So as a specialty program for patients with pulmonary hypertension, we get referred patients where [00:16:00] the provider thinks that there is evidence of significant pulmonary hypertension, after some of this initial testing. As Lisa mentioned, there has been a standard evaluation for pulmonary hypertension that's guideline driven and well-published for many years. 

But unfortunately, most of the time, even once they're referred to us, most patients have not had every component of that evaluation. And so, part of our job is to make sure that we have ordered and investigated every test in an effort, not just to determine whether the patient has pulmonary hypertension, but to also give us the information we need to be able to classify that patient and their type of pulmonary hypertension. 

So, in addition to the testing that Lisa mentioned, we are going to make sure that the patient has had a chest x-ray. It's sometimes surprising to me people [00:17:00] have been dyspneic for quite some time, but have never had a simple chest x-ray.  

We will usually want an echocardiogram with a bubble study looking not just front congenital heart disease, but also information about whether the patient may have a patent foramen ovale. VQ scan is the recommended test to evaluate for chronic thromboembolic disease and is favored over CTA scan.  

Full pulmonary function testing is essential to evaluate for interstitial lung disease or other pulmonary parenchymal problems. We will screen patients with ANA, an ANA anti-nuclear antibody test and HIV test. 

Hepatitis testing and thyroid function testing all would hint to conditions that may be associated with the development of pulmonary hypertension. And more recently we [00:18:00] would add a test for Pro Brain Natriuretic peptide, which is a marker of cardiac function in trying to determine if a patient, you know, has enough evidence to proceed of right heart dysfunction, to proceed with an evaluation and a right heart catheterization.  

We also like to get an objective measurement of a patient's functional capacity with a six-minute walk test, done for both distance, oxygenation, heart rate. And a subjective symptom assessment of patients with the functional class assessment.  

And depending on these results, other testing may be indicated, which as I mentioned, includes perhaps a CTA if a VQ scan is abnormal and, high resolution CT testing if the PFT are abnormal, or cardiac MRI if congenital heart disease might be an option.  

Ultimately if patients, if their workup is suggestive of [00:19:00] elevated blood pressure in the lungs, a right heart catheterization will be done. If it looks like that patient may have either CTEPF or other forms of pulmonary hypertension, there might be specific things to do during the right heart catheterization.  

For example, a pulmonary angiogram to determine operability for a CTEPF patient, or vasodilator or vasoreactivity testing in patients that have idiopathic forms of pulmonary hypertension or drug-induced forms of pulmonary hypertension. 

And so, as you can tell from this exhaustive list of testing, in addition, even when patients get to specialty centers, it can take some time to fully evaluate the condition before treatment can even begin.  

Geralyn Warfield (host): We have been speaking with Traci Housten and Lisa Maher about pulmonary hypertension. We're going to take a quick break and we will be right back. 

 

Geralyn Warfield (host): We'd like to welcome our audience back for our discussion about pulmonary hypertension, and [00:20:00] I'm sure our audience is wondering how often they might see this in clinical practice. What really is the prevalence of pulmonary hypertension?  

Lisa Maher (guest):  Geralyn, I'll be happy to talk a little bit more about that. So, there is an estimated 1% of the global population with an increased prevalence of 10% in those over age 65. 

When we look at sources, the Lung Association estimates, pulmonary hypertension anywhere from approximately 500 to 1000 cases diagnosed in the United States each year. So even though that maybe doesn't seem like a lot, if it is you or your family member, you definitely want that diagnosed. 

And I would guess too that, that probably underestimates the actual number of pulmonary hypertension patients out there purely because of the range of symptoms. And again, how late we may [00:21:00] find those.  

 

Geralyn Warfield (host): So, while this particular disease state, while pulmonary [00:22:00] hypertension is not the most common thing seen in clinical practice, it obviously has a significant impact on the individual who has this, plus their family.  

And I'm wondering, we talked earlier about how the symptoms of PH mimic so many other things and how the diagnostics can take quite a bit of time, it's a multi-step process, there are a lot of providers, a lot of specialties perhaps, that are involved in this process. And I suspect as a result that many individuals with PH don't always receive a diagnosis as early as they could.  

And I'm hoping we could talk a little bit more about what are some of the ramifications of a delayed diagnosis. 

Lisa Maher (guest):  I'm going to let Traci speak more to this, but I can tell you from a general cardiology standpoint, I know there are a lot of times we personally will delay the diagnosis and unfortunately that can mean the difference between life and death.  

Traci Housten (guest): Yes, pulmonary hypertension is a very [00:23:00] complicated disease with very complicated treatments, and so there are multiple ways along the patient's journey that things can get delayed. 

First, they can have a delay to an accurate diagnosis. Again, not all pulmonary hypertension is the same. Not all pulmonary hypertension should be treated the same way, and so patients can spend a lot of time not having any diagnosis or having an incorrect diagnosis.  

Even once the diagnosis is made, there can be a delay in appropriate treatment. 

Those delays can occur because complex treatments are expensive and often there are delays with insurance authorizations or the patient's ability to pay for these complicated, expensive medications. And potentially sort of an inexperience with prescribers about how to mitigate some of those issues. [00:24:00]  

There can also be a delay in getting patients to specialty referral centers that are experienced in the care of pulmonary hypertension. And those centers also can provide access for patients to research and clinical trials of new medications and new therapies and new ways to think about the disease. 

There have been multiple studies that have shown that there continues to be, despite all of our efforts, a long delay in patients getting an accurate diagnosis and accurate treatment. During this time, while patients are waiting and continuing to feel unwell, the pulmonary hypertension is most likely progressing, as is the stress on the right side of the heart as the blood pressure in the lungs rises. 

And there have been some studies that show that even a delay of initiation of medications of a few months [00:25:00] can cause those patients to never really catch up or improve as much as the patients who got treatment earlier. That was data that followed patients in a randomized control trial who initially got placebo and then got actual medication at the end of the trial. 

And so, it's very important that patients get referred to experienced programs that can help move things along for these patients and follow them carefully over time.  

Geralyn Warfield (host): I hope our audience recognizes that call to action to be aware of pulmonary hypertension and the fact that it may be hiding in plain sight in our clinical practice and just to be aware of what it looks like, what the symptomology is, and I think we have a much better understanding of that after spending time with our guests. 

Traci Housten and Lisa Maher, we're very grateful to you for your time today. We're also very grateful to Merck Sharp and Dohme Corporation for their unrestricted grant funding for this [00:26:00] podcast episode. This is your host, Geralyn Warfield, and we will see you next time. 

 

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