Resources for PAH Support Groups

Hello, everyone. I'm Dr. Vijay Balasubramanian. I'm one of the pulmonologists, the medical director for Valley Advanced Lung Diseases Institute here in Fresno, California. I'm also a specialist in pulmonary hypertension and advanced lung disease. It's great to meet all of you, and here today we are going to talk about understanding your pulmonary arterial hypertension symptoms.

This presentation is sponsored and made on behalf of United Therapeutics. Speakers are compensated by UT. The information presented here is for educational purposes only and is not intended as professional medical advice. Always discuss questions you may have with your healthcare provider. Not all medications are appropriate for all patients. Please speak with your healthcare professional to determine which treatment plan is right for you.

Today's overview of the presentation is basically in three steps. Pulmonary arterial hypertension, how does it affect your body? And PAH medications, how do they improve symptoms? And what can you do to help in this process?

How does pulmonary arterial hypertension affect your body? Pulmonary arterial hypertension is a rare and a serious lung disease. The blood vessels within the lungs tend to thicken and narrow over a period of time. This makes it hard for the right side of the heart to squeeze the blood into the lungs, and therefore, it has to generate higher pressure in the blood vessels, and therefore, lesser blood flow within the lungs.

The narrowed vessels and less blood flow affects different parts of your body. Obviously the more common symptoms being shortness of breath and fatigue. And eventually, as the right side of the heart starts becoming more enlarged and strains and becomes weaker, then what we call the failing right side of the heart, then the swelling in the abdomen happens. Swelling in the ankles can happen due to more fluid and water retention as a result of this high pressure that's generated in the lung circulation. And shortness of breath and chest pain may occur due to the body not getting enough oxygen.

The blood vessels in your lungs are like highways. Here is a nice cartoon that illustrates how the blood travels within the blood vessels. When the highways work best with fully open lanes, you can see all the cars are running smoothly in their respective lanes. But when you see that there is a lane closure, you can see things backing up and the pressure builds up. And that's typically what happens within the blood vessels of the lungs. The narrowed blood vessels cause more resistance, and that is what we call or refer to as pulmonary arterial hypertension, which leads to less blood flow, and therefore, less oxygen transfer. The higher resistance in the lungs causes higher pressures in the heart, and the right side of the heart swells, enlarges, and therefore, it makes it harder for the right side of the heart to push blood through these narrow vessels. The less blood flow through the lungs lead to less oxygen reaching the parts of your body where it's needed. So adequate blood flow and oxygen are important factors for normal bodily functions to carry out day-to-day activities. That is how a patient might end up feeling short of breath or fatigued when even doing daily tasks.

The heart becomes strained over time. To compensate the strained heart pumps more forcefully trying to push blood through these narrowed vessels and this can result in fatigue, lightheadedness. And the type of lightheadedness we see in pulmonary arterial hypertension is when patients engage in activity, meaning to say, the more oxygen a patient needs, that is when the strain is felt more and they feel more lightheaded because the body is just not able to compensate for the increased need.

The strain on the heart can get worse over time, and this can lead to what we call heart failure. As the heart enlarges and strains, it won't be able to keep up and will begin to fail at some point. Patients can start feeling dizzy and can even faint with even some exertion.

Now, that is why diagnosis of pulmonary arterial hypertension can really help because once we make the diagnosis and get the patients on appropriate PAH medications that can improve the blood flow in the lungs and that reduces the strain on the heart.

How do PAH medications help improve symptoms? Healthy blood vessels require a balancing act. Basically, you have certain chemicals that help to keep the blood vessels open, and there are certain chemicals that keep the tone of the blood vessels, meaning to say it has to remain in a good tone. A healthy balance between the two helps for adequate blood flow through the blood vessels. But when you have certain substances, when this balance gets tilted one way, there might be too much of some substances and too little of others. Then you might see progressive narrowing of the blood vessels, and therefore, increases the blood pressure in the lung circulation.

Most people have more than one substance out of balance. PAH medications can help restore that balance, so can slow down the production of substances that you have too much of or increase the production of substances that you don't have enough of. Please talk to your doctor about your treatment plan if you have stopped improving or still experiencing PAH symptoms despite some treatment changes because this is a dynamic thing. It'll constantly keep moving, depending on each and every patient, depending on their status.

PAH medications can help traffic flow more smoothly. They can help widen the blood vessels in the lungs or open more lanes, so to speak. Now, they can flow more effectively and it reduces the pressure in the process. The resistance to flow gets lower and the blood begins to flow more normally. The treatment guidelines recommend treatment with two or more types of PAH medications for most patients. Again, just as we spoke about a couple of slides ago, most of the time, patients can have more than one chemical imbalance, and therefore, targeting at least two mechanisms can help improve outcomes. And what do we mean by outcomes? The improved functional class improved ability to do more activity and feel less short of breath, and that is what is measured in the six-minute walk distance as well. These type of improvements can be achieved with the help of this type of approach. So taking a combination of medications may improve how well you can do your day-to-day activities with minimal or less symptoms.

The right combination is the one that helps you meet your treatment goals, and the treatment goal is something that you have to set along with your provider. And what do we mean by that? What we mean by a treatment goal is improving lung function, improving heart function, and improving symptoms. The treatment goals are very much set by yourself in partnership with your provider, so you and your healthcare provider will choose the type of medications that can best treat your unique PAH.

What can you do? Take an active role by tracking your symptoms. Please be observant. Keep track of your symptoms in a way that works for you, such as a symptom journal is just an example, including activities that you can do without symptoms, activities that trigger symptoms. That way, when your healthcare provider asks you the next time, "What are the challenges that you're experiencing?" you can be very succinct in telling what your challenges are. Please share your symptoms with your healthcare provider at each visit. And the dynamic nature of improvement or worsening can be assessed by yourself in this process.

Not all cardiologists or pulmonologists have expertise in treating PAH. It's important to find a provider with specialized training in pulmonary arterial hypertension, who has experience in using all classes of PAH medications. PAH treatment guidelines recommend working with a PAH specialist. To help you find a list of PAH specialists, you can refer to the Pulmonary Hypertension Association website or you can scan the QR code. Visit findapahdoctor.com. That might give you that information.

Discuss your options with a PAH specialist such as adjusting your medication plan, adding or changing your medication, adjusting the dose of your medication. Please start making lifestyle changes, like starting an exercise program, changing your diet such as limiting salt intake, and getting rest and emotional support is very important as well. There may be more you can do to help reduce your PAH symptoms and do more daily activities.

In summary, in PAH naturally occurring substances in your lung blood vessels, can become imbalanced. The imbalances in the blood vessels prevent your lungs and heart from working properly. Treatment with a combination of medications may help restore this balance and improve PAH symptoms. Talk to your doctor about your treatment plan. They may be able to do more for you, and obviously you yourself have a great part in this.

Thank you. I hope this information was very helpful. Consider joining the PAH initiative at jointhepahinitiative.com, and you can also follow us on social media. Thank you.

Hello everyone. It is a pleasure to meet all of you.

Let me introduce myself. I'm Dr. Vijay Balasubramanian. I'm a pulmonologist who specializes in advanced lung disease, pulmonary hypertension, part of the Valley Advanced Lung Diseases Institute, Fresno, California.

Today, we are going to talk about the role of echocardiography in pulmonary arterial hypertension care. I'd like to thank United Therapeutics for sponsoring this particular segment, and I'm making this presentation on behalf of United Therapeutics. Speakers are compensated by UT. The information presented here is for educational purposes and is not intended as professional medical advice. Always discuss your individual test results and any questions you may have with your healthcare provider.

Here is an overview of our presentation today. We'll take a look into what the heart actually does in pulmonary arterial hypertension, and what your doctor is looking for on an echocardiogram. We'll also take a deeper dive a little later during the presentation into some of these aspects.

What happens to the heart in pulmonary arterial hypertension? The heart has two sides to it: the left side and the right side. The main aspect of the left side is to pump the blood from head to toe, and the main purpose of the right side of the heart is to receive the blood from the rest of the body. When the blood comes to the right side of the heart, the right side of the heart then has to pump this blood into the lungs. This is done normally effortlessly as the lungs are conveniently situated right next to the heart.

But in pulmonary arterial hypertension, the blood vessels within the lungs get progressively narrower. That means that the right side of the heart now has to pump harder—it has to work harder—to pump the blood into the lungs, which results in strain of the right side of the heart. It starts becoming larger and also becoming weaker with time.

The changes in your right side of the heart as seen on an echocardiography can also be indicated indirectly by changes in some blood values like BNP, which is a blood test that you may be familiar with, your doctor might be requesting on a regular basis. BNP is a protein within the blood; basically, it indicates that if the heart is working extra hard, then the BNP is secreted more. So, if the BNP is lower, that means the heart is squeezing effortlessly. The higher the BNP, that means the heart is straining more.

And this can parallel along with changes in functional class, which is a subjective aspect of how well a patient is doing, or how much it takes for somebody to become symptomatic. As a PAH condition progresses, it can eventually lead to a hospitalization. The inverse is also true: as the pulmonary artery hypertension improves, so does the changes in the right side of your heart, and obviously changes in your BNP and NT-proBNP, as well as changes in functional class in the favorable way—from functional class four, to class three, to class two—and improvements in six minute walk distance as well.

How Do We Monitor the Changes in the Heart?

Two important tests are right heart catheterization (a direct measurement of blood pressure in the right side of the heart) and then the echocardiogram.

As you may recollect, when you go to the doctor's office, they slap a cuff around your arm and this measures the pressure at which the blood is circulating through your body. The right heart catheterization measures the pressures in the heart directly, in the heart as well as the blood vessels of the lungs. It also indicates how well the heart is pumping blood. Now, these measurements are used to not only diagnose and confirm pulmonary arterial hypertension, it can also help to determine disease severity, and monitor changes in pulmonary artery hypertension when compared to prior results.

Information gathered from the right heart catheterization can help provide various important details.

One of the important things is pulmonary vascular resistance, which is basically a measurement of how the blood is flowing through the blood vessels, an indication of how difficult it is for the blood to flow or how much resistance it experiences.

Pulmonary capillary wedge pressure is basically a measurement that's indicative of the filling pressure of the left side of the heart. In other words, all of us need a certain amount of volume that needs to be circulating through our body. And the left side of the heart is the part of the heart, as you know, that squeezes the blood from head to toe. So we need to get an idea as to what is the total output of the heart. And the pulmonary capillary wedge pressure is that particular measure that gives us an idea about this filling pressure on the left side of the heart.

This is measured when they do the catheterization: the tip of the catheter has a tiny little balloon. As they advance the catheter into the blood circulation within the lung blood vessels, at a certain point the balloon completely occludes the smaller pulmonary blood vessel, at which point it becomes like a manometer. So the pressure at the tip of the balloon is basically the same pressure on the left side of the heart. This is such a crucial measurement that gives us very important information about what we refer to as the filling pressure of the left side of the heart.

The pulmonary arterial pressure on the other hand is just a direct measurement of the pressure at which the blood is circulating within the blood vessels of the lungs.

Here, the right atrial pressure is basically the smaller chamber on top of the right ventricle, and this is the chamber that receives the blood from the rest of the body. And obviously, it gives us an idea of what the pressure is on the right side of the heart when it first receives the blood.

And then the other important test is the echocardiography, which is basically an ultrasound of the heart. You may recollect this test where they put a gel around your heart and they put a tight pressure on the probe and they go around the heart to see the images of the heart.

Obviously, when there is pulmonary arterial hypertension, the blood vessels within the lungs are narrowing, and this is behind your chest and there is no direct way of measuring that apart from doing a right heart catheterization, which will give us that information very accurately.

Today, experts use echocardiograms to monitor pulmonary arterial hypertension severity and progression. An echocardiogram is a non-invasive way to monitor changes in the heart. It uses sound waves or ultrasound to create pictures of the heart. It is the most widely used tool for assessing the size, structure, and function of the right side of the heart.

The echocardiogram can show a healthcare provider how PAH has affected the patient's heart, say how well a current treatment plan is working, and when a patient's treatment plan needs to be adjusted. Seeing one's heart size and function helps the doctor or the healthcare provider understand where your PAH may be headed.

So what does your doctor look for on an echocardiogram? We are looking for evidence of size and shape of the right-sided heart chambers, and how well it's functioning.

The most common condition that most of them in the community are used to are left-sided problems, such as having a heart attack. How well is the heart squeezing? And most of the echocardiograms are going to emphasize on the left side of the heart. But when a pulmonary hypertension provider is requesting a right heart focused echocardiogram, that means that we are looking for certain other features of the right side of the heart and not only the usual left-sided heart indicators. That's how the right-sided heart echo is different compared to your generic left-sided heart echo, which is a lot more common.

Did you know that the heart appears upside down on an echocardiogram, as illustrated on this particular cartoon? We will see some real images shortly. You can see that the two ventricles, the right ventricle and the left ventricle as depicted there are on the top, and then the right atrium and the left atrium in the bottom.

In the echo of a person who does not have PAH, here is a nice illustration of what we call a four-chamber view. You can see that the right ventricle is here, the left ventricle is bigger, the right atrium, and the left atrium. Generally in a normal echocardiogram, the left side of the heart is much larger because the function of the left ventricle is to squeeze the blood from head to toe, but the right ventricle just needs to squeeze the blood to the adjacent lungs. Therefore, the right ventricle is smaller than the left ventricle. And the right ventricle has a thin wall and a narrow apex, as illustrated in the cartoon here. The right and left-sided atria are similar in size.

What about the heart of a patient with pulmonary arterial hypertension? Here, we have two pictures: we have the advanced PAH on the left side with a red label, and then a normal four-chamber view on a normal patient. So again, you can see that the right-sided chambers are significantly enlarged in patients with PAH, both the right ventricle as well as the right atrium. In this echo, the right ventricle is much larger. The muscle of the right ventricle thickens, as you

Therefore, monitoring the right side of the heart with echocardiograms help healthcare providers to be proactive. Changes in the right side of the heart in parallel with changes in the BNP or NT-proBNP, along with functional class and six minute walk distances, helps to make a very good objective assessment of the right side of the heart.

Echocardiograms are recommended for patients every three to six months. Catching disease progression early helps the doctor make adjustments to try to prevent symptoms before they happen.

Let's take a little bit of a deeper dive. In PAH, we do something called risk assessment. As you all know, PAH is a serious disease and has a fatal outcome if we don't pick it up and treat it well. So what we mean by risk is the risk of death. Most patients are diagnosed at the intermediate or high-risk status.

We make that determination based on several indicators on the left side of the table here, without going into the details. These include subjective details and objective details. The clinician has to put all these parameters together to make a very good judgment as to whether the patient belongs to a low, intermediate, or high-risk category.

And echocardiogram and right heart catheterization play a crucial role in making this determination. Our job as a healthcare provider is to make sure that we get the patient from the high-risk, towards the intermediate risk, towards the low-risk. The more proactively we make an effort to get the patient to the low-risk, the better the outcomes are.

PAH is a rare disease, so not all cardiologists and pulmonologists have expertise treating it. It is one of those disease states where both cardiologists and pulmonologists do treat this condition depending on your respective local resource. PAH experts have experience understanding echocardiograms from patients with pulmonary arterial hypertension. The specialists can use echocardiograms to interpret, say, how to make a treatment plan, and how the treatment plan is impacting the right side of the heart.

In summary, monitoring the right side of the heart is very crucial in the management of pulmonary arterial hypertension. Changes in the heart are one of the first signs that PAH is getting better or worse depending on how we look at it.

Echocardiogram is a non-invasive way to look at the size, structure, and function of the heart. Today, echo is an important part of risk assessment as we spoke about a couple of slides ago. Monitoring the right heart allows healthcare providers to set and proactively adjust treatment plans that may help slow disease progression and prevent symptoms before they happen.

Please ask your healthcare provider about how your heart is changing over time. Thank you for this opportunity. And if this information was helpful, please consider joining the PAH Initiative at jointhepahinitiative.com. You can also follow us on social media. Thank you.

Advocate for Your Well-Being—Be Prepared for Each Appointment

Brought to you by the PAH I Initiative and sponsored by United Therapeutics, committed to improving the lives of patients.

Pulmonary arterial hypertension (or PAH) is a difficult disease to live with. There’s a lot to keep up with day-to-day and only a limited amount of time to talk with your healthcare provider during each visit.

We’ve put together a few simple steps that you can take to ensure you get the most out of every visit. We’ve divided the steps by things you can do before, during, and after your visit.

So, first things first—What can you do before your appointment?

First and foremost, learn everything you can about PAH.

The PAH Initiative and patient organizations have informative websites and accessible tools that can help you learn the latest about PAH.

“Risk status” is an example of important new information that is covered on PAHInitiative.com. If you haven’t already done so, browse the website and learn everything you can about how your risk status plays a role in your future health. [On-screen text: disease information, risk status, and treatment options]

Learning as much as you can will allow you to walk into your appointment with confidence, better able to understand what your healthcare provider is telling you, and be fully prepared to ask any questions you may still have.

Let’s hear from a patient with PAH:

“Learn all you can about PAH because an educated patient is the best advocate for themselves.”

Now that you’ve learned all you can about PAH, it’s time to think about your own journey. What are your personal goals and visions for your future?

Already have a goal in mind? Take a moment to write it down. You can also create a “vision board.” Have some fun with this! Go through an old magazine or search online for images that inspire you to reach your goals.

Next, spend time monitoring and tracking how you’re doing. It’s easy to forget the details of how you felt since your last visit. So, try using the simple method of calendar tracking. Try using a scale that is easy to understand, like a smiley face. Activities you might want to track include exercise, diet, weight, happiness or mood. [On-screen text: Track how you’re doing regularly so you can easily tell your healthcare provider how things are going between appointments.]

When tracking how you’re doing, try asking yourself questions that are specific, detailed, and that relate to activities you do often. Questions like “How do I feel today?” can be troublesome because they are general and can be difficult to answer. Try asking yourself a more specific question like “Was I able to go on my walk today without having to rest?” [On-screen text: Was I winded after my walking routine today? How did I feel after my walk to the mailbox? Was I able to get in and out of the car with ease? Was I able to climb the stairs with ease? How did I feel after doing a load of laundry?]

Remember, you should also ask yourself questions that tie into your personal goals, like “Am I making the kind of progress I envisioned when I set my goals?”

It’s also important to ask questions about how you’re managing your lifestyle. Asking yourself questions like “Am I monitoring my fluid intake?” and “Have I maintained a low-sodium diet?” before your visit will help you be prepared to discuss your health and wellness during your visit. [On-screen text: Am I maintaining a low-sodium diet? Am I monitoring my fluid intake? Am I tracking and recording my weight?]

OK, so what’s next?

Choose a 3-ring binder and make it your dedicated PAH binder. Not only should you use it to organize all your research and tracking, but you should also use it to store your medical information and contact numbers.

Your binder is your tool, so you can use it for both education and inspiration throughout your treatment journey. [On-screen text: Health insurance information, records of your medical history, current medications, current risk status, personal goals, healthcare team’s phone numbers. Questions for your healthcare provider? Put them in your binder, too!]

So far, we’ve covered all of the important steps that you can take. But whether you realize it or not, you’ll need some help along the way.

The next tip we have for you is finding a great support system, made up of one or more “advocates.” Having an advocate can help strengthen your voice in times of uncertainty and also give you friendly reminders to help keep you on track. [On-screen text: Make sure your advocate understands your personal goals. Remember, your advocate walks with you, not for you.]

Let’s hear from a patient with PAH:

“Do what you can; let others help you with what you can’t. Most of all, keep up your spirit.”

It’s now the big day! You’re prepared. What’s next?

On the day of your appointment, remember to dress for success! The 6-minute walk test will be easier to do if you’re wearing proper walking shoes and clothing that is comfortable. [On-screen text: Dress for success(full tests)]

The next thing to remember on the day of your appointment is to bring your binder! To avoid forgetting your binder, get in the habit of leaving it near your keys, jacket, purse, or other “going out” items. You can also set an alarm on your smart phone to remind you to grab your binder before appointments. [On-screen text: Your caregiver can help you remember your PAH binder.]

Visiting your healthcare provider can feel stressful, or even uncomfortable. After all, you’re discussing details about yourself and your health. Just remember that your healthcare provider’s number 1 goal is to help you, so he or she needs to know all of your concerns and questions. And the only way they can do that is if you speak up. [On-screen text: Ask questions! How am I doing on my tests? What else can I do to improve my risk status? What is cardiopulmonary rehabilitation, and should I be doing it? Which pathway(s) am I treating? How do I improve my risk status? Would I benefit from additional medication? What exercises can/should I do?]

Let’s hear from a patient with PAH:

“I cannot stress the importance enough of having a trusting relationship with your doctor… Make sure your voice is heard.”

We know you may be overwhelmed by the end of your appointment and excited to get out of there; however, one last thing before you go—ask your healthcare provider, “What are my next steps?” [On-screen text: What can I do to help lower my risk status? What should I be monitoring between now and my next appointment?]

Don’t leave without knowing exactly what steps you need to take to continue on the best PAH treatment path for you.

Your PAH binder is a great place to write this information down so you can refer back to it in a couple weeks and make sure you’ve followed through with any important steps you need to take.

We realize that visiting your healthcare provider can be stressful. So, to help make it a little easier, just remember “BAND”: Bring your binder, Ask questions, What are your next steps? And Dress comfortably.

Congratulations! You’ve made it through a successful visit with your healthcare provider. You walk out of your appointment feeling great, and you should! So now, what?

Most importantly after your visit, take your medications as directed, especially if you are starting a new medication. Keep your healthcare provider informed about any side effects you experience. Refer to your PAH binder notes from your last appointment if you need a refresher on something your healthcare provider told you during the visit. [On-screen text: Follow through on treatment plans made by you and your healthcare provider.]

It’s also important to manage your sodium intake and fluid levels, schedule follow-up appointments, and track and record your weight and walking distance between your appointments.

Don’t be afraid to call your healthcare provider between visits. You don’t have to wait until your next appointment to ask questions or clarify something.

Let’s hear from a patient with PAH:

“Don’t feel that you are alone in this battle. You’re not alone.”

Thank you for taking the time to learn more about what you can do today to help improve your future. Visit PAHInitiative.com for more helpful resources, information, and inspiration as you continue on your PAH journey.