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Dr. Miriam Jacob discusses pulmonary hypertension with cardiac surgeon Dr. Michael Tong and pulmonologist Dr. Gustavo Heresi.  Topics covered include what is pulmonary hypertension and when should you consider this as a diagnosis, diagnostic testing, how treatment is decided, types of treatment such as medical management, lifestyle changes, surgery for those who have chronic thromboembolic pulmonary hypertension (CTEPH) and when lung transplant is considered.  The pulmonary hypertension team approach at Cleveland Clinic is described as well as how the team works with your local doctors to provide the best care possible – the goal is the right diagnosis – the right treatment – and knowing when to escalate therapy when needed.

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Pulmonary Hypertension – what you should know

Podcast Transcript

Announcer: Welcome to Love Your Heart, brought to you by Cleveland Clinic's Sydell and Arnold Miller Family Heart and Vascular Institute. These podcasts will help you learn more about your heart, thoracic and vascular systems, ways to stay healthy, and information about diseases and treatment options. Enjoy.

Dr. Jacob: I'm Dr. Miriam Jacob. I'm in the Advanced Heart Failure and Transplant section here at Cleveland Clinic. This is Dr. Gustavo Heresi who's our Director of Pulmonary Vascular Diseases in the Respiratory Institute and Dr. Mike Tong who is our Director of Mechanical Circulatory Support and Heart Transplant here at the Cleveland Clinic.

So we're talking about pulmonary hyperension and to start, what is pulmonary hyperension? It really just means high blood pressure of the lungs and there are different flavors and varieties of this and the typical patient comes in and they're short of breath, they might be fatigued, they might be swelling. A lot of the symptoms that a lot of our patients with heart and lung disease have.

Dr. Heresi, can you talk a little bit more about who is the kind of person that we see and we are suspicious of this diagnosis of pulmonary hyperension?

Dr. Heresi: All right, so, so pulmonary hyperension is really the oil high blood pressure, right, so people think about well, high blood pressure in the arms where they're going to have high lung pressures and the symptom is shortness of breath. Not all our patients come with shortness of breath for weeks, months, years sometimes. Other symptoms sometimes include just feeling tired, and then when the disease is more severe, chest discomfort, lightheadedness and passing out.

So, what we ... I always try to tell people, of course, is if you're having some of these symptoms, particularly again shortness of breath, and your doctors have done some of the testing that we typically do here. You know, some chest x-rays, some breathing tests, maybe some heart tests and there is no clear explanation for the symptoms and typically what we'll see, also, is patients get some water pills, some inhalers, and if there is no significant improvement in the symptoms, you need to start thinking about something like pulmonary hyperension and talk to your doctors about that and the first screening test of choice for, to start looking into this is the echo cardio, you know, the heart ultrasound that you guys CARDIOLOGY do frequently.

Dr. Jacob: Mm-hmm (affirmative), and it's a really easy test. You know, there's no pain. There's some cold jelly that puts on your chest so it's actually a really noninvasive way to screen and figure out if somebody has high pressures of the lungs so what's the next step? So say we are suspicious on the echo, so what do we do next?

Dr. Heresi: Right. And that's a very important point so we, we would never arrive at a final diagnosis and certainly we would never treat based on an echo alone. To confirm the diagnosis, assess the severity, understand where it may be coming from, we do need a procedure called Right Heart Catheterization which you do frequently for us and I'll let you explain how it goes but basically that test is the one that tell us accurately how high the blood pressure in the lungs is and also starts to tell us where it may be coming from.

About that, I can comment on with a Right Heart Catheterization, if we see pulmonary hyperension, there's a battery of tests that we need to do that include imaging of the chest, breathing tests, some blood work, tests to look for blood clots in the lungs, so a variety of testing to understand where the reasons is for the Pulmonary Pressure Elevation.

Dr. Jacob: So, a Right Heart Catheterization is an out-patient procedure. It's different than Left Heart Catheterization that most people know, and what we're literally doing is just going into the right side of the heart and the lung and measuring the blood pressure and how well the heart's pumping the blood out. We do it here everyday. We probably do 10 a day in our lab. The Pulmonologists do it as well. People come in, they're not given any sedation and we clean the next, because that's usually the vein that we go through. We numb it with a small numbing needle and put an IV there.

And then after that, we put a long plastic tube into the heart and the lungs and measure the pressures and with that procedure we're going to know is there pulmonary hyperension there or not so we will know, at least, does it exist, a little bit about it's flavor and then from there, we can say if we see that it exists, like Dr. Heresi says, we go on to define it more because that effects how we treat people.

Depending on the category we would put people in, we have medical therapies that possibly help them. We have surgical therapies that may help them. So it's really important to get the correct diagnosis first and then defining it a little bit better.

So, we have about 12 different medications out there, some are IV, some are subcutaneous, some are inhaled, some are oral. You know, Dr. Heresi, do you want to talk a little bit about how we start therapies or how we decide?

Dr. Heresi: Yeah, so, the first step is to understand where the pulmonary hyperension is coming from, right? So if it's due to heart disease, you would work on the heart and try to fix that. If it's due to lung disease, like emphysema, Pulmonary Fibrosis, then we in Pulmonary medicine try to manage that.

If it's due to blood clots, we talk about surgical therapies or oral intervention procedures that can treat that and if it is due to connect deficiencies like Lupus, or others, then we have these medical therapies that we use and basically, we now start very aggressively. We typically start a couple of medications from the get go but what we do is, we do a careful evaluation to assess how severe this is. If the pulmonary hyperension elevation is quite severe and the right side of the heart is pretty weak, we typically would start our most aggressive therapies which include the pump therapy with an infused medical and if not, then we typically start with pills and then we add medications as we go along.

Dr. Jacob: So speaking of one of the flavors of pulmonary hyperension, there's one called Chronic Thrombo-Ambala Disease so it's when people get blood clots or they get scarring, actually, of the lung arteries and it actually puts back pressure on the right side of the heart and causes, you know, this pulmonary hyperension disease, so, Dr. Tong, can you talk a little bit about the surgical treatments that we have for some of our patients?

Dr. Tong: Yes. So, the condition that you just described, it's C-TEF for short. It stands for Chronic Thrombo-Embolic pulmonary hyperension and essentially, what it is, is in patients who have developed pulmonary embolus, in most patients with just blood thinners, the pulmonary embolus will be dissolved and it'll go away. But in a small group of those patients, they will often either get recurrent disease, recurrent pulmonary embolus, or the pulmonary embolus doesn't go away, it doesn't dissolve and over time that clot becomes scarred and that scar becomes stuck onto the wall of the blood vessels in the artery and when the scar builds up, the right heart has nowhere to pump the blood to and the pressure will just build up.

And in those cases, if we see this on imaging such as a CT scan, or a pulmonary angiogram, we can then take these patients to surgery and it'll often be a curative procedure. What we do during surfer is we'll put the patient on a heart/lung machine and then we'll open up the arteries that go to the lung and I will find where these scar is, and we'll peel the scar off the blood vessels and do a complete extraction of all the scar.

This is predicated on the scar being present in the large vessels and in the medium sized vessels and we can continue to peel the scar as it enters into the smaller vessels and once it gets to the vessels that are too small for my instruments, we start pulling on the scar like a piece of spaghetti and get a complete extraction.

Sometimes you do have patients where the scar starts very far down into the lungs, in the blood vessels that are just too small for my instruments to get to and in those type of patients, surgery will not be successful if I can't get to the scar with the instruments. In those cases, luckily, we have a new modalities of treatment with a balloon and catheters. Our intervention radiology colleagues can go in and using a ballon, get to those smaller vessels and push those scar tissue aside and to allow the blood flow to get back into the segments of the lungs that were occluded before.

So now a days, we do have multiple modalities. We have surgery which is an excellent option for many of these patients and patients who are either too high risk for surgery or who have disease that are in the too small of a vessel for surgery, and the balloon angioplasty is also very successful.

And occasionally, you have patients who have disease in the tiniest of the vessels call the capillaries, and in those patients, neither surgery or the balloons will be successful. In those patients, we rely on medications and then the medications do fail, then we still have the option of doing lung transplantation.

Dr. Jacob: So I think, you know, our expectation and our hope is to improve quality of life, to reduce symptoms, sometimes get people off oxygen, get people having, you know, functioning better. For those patients where we've been aggressive with surgery or medications and they're getting worse and worse and we don't know, we don't have anything else to do ... what's the next step?

Dr. Heresi: Right, so the next step for those patients is to basically replace the lungs with new lungs, right? Lung transplantation and I think it's important to consider that. Luckily, with all these new medications, the need for that has come down dramatically over the last couple of decades. We used to do way more transplantation or some of us even heart/lung transplantation for our pulmonary hyperension patients. But now a days, it's really a minority. However, we do do a few every year and I think that's important to recognize that lung transplantation remains a treatment of choice for people who are on maximum medical therapy which typically would include a couple of pill, and infused pump medication. If those patients, and people who are still quite limited; very short breath and a lot of oxygen, very high lung pressures, lung transplantation is the way to go.

There's also certain, particular, conditions that would make us think immediately about that and that's just to stress how importance of the evaluation. There are some things in the lungs, relatively rare conditions, that we call pulmonary renoclusive disease, for example, but there are certain conditions in the lungs that we know from the get go they are not too likely to respond to these medications and so we immediately think about lung transplantation and the start of that process.

Dr. Jacob: So it sounds like, you know, for us, we really think it's important for patients to get to the right place to get the correct diagnosis, to get hooked in for the right treatments, and also, escalate therapy, you know, to surgery or transplant when it's appropriate. But I agree, things have changed so much even in the last 10 years, 15 years, that most of our patients, actually, are living their lives on complex medical regiments, but doing really well with that.

And, you know, some other things that we've ... we're studying right now are, kind of, lifestyle things that we can change as well. So, Dr. Heresi, can you talk about one of the studies that we have here, in particular, for our patients with pulmonary hyperension?

Dr. Heresi: Yes, so we ... as you pointed out, we have made a lot of progress. We are luckily being able to help a lot of people with these serious and deadly disease, however, we're not doing great. We're not curing the disease except when Michael removes these lungs from people but for the most part pulmonary hyperension remains and incurable disease and people frequently remain limited so we're looking at new things. We're looking at new medical therapies, looking at new mechanisms offcation, trying to move beyond just opening up vessels and perhaps targeting the basis of the disease and one of the things that actually makes intuitive sense although we've never really proven is, is just healthy diet and exercise, right? And, of course, it helps, in general for your well-being but we actually think, and there's some preliminary data to support this, that diet and exercise will actually help intrinsically, pulmonary vascular disease, pulmonary hyperension.

So we have a study looking at that. Basically, you know, improving your diet, having some, more vegetables, olive oil, fruits, et cetera and also getting a personal trainer to sort of train every day for three months and we think that that's going to lead to improvements in pulmonary hyperension. But, we need to do the study to prove it, of course.

Dr. Jacob: Right. And it's a great study because I think patients feel very motivated and are excited to do it and it benefits them. They recondition them, but we're also trying to learn a lot-

Dr. Heresi: Correct.

Dr. Jacob: ... to say what is the proper way of treating our patients. With regards to, you know, how our teams work, you know, Dr. Tong, can you talk a little bit about how when a patients case comes to us, or we see a patient, kind of how this, the chronic thrombo-embolic pulmonary hyperension group looks at things?

Dr. Tong: Yeah, the real benefit of ... for patients that come to a place like Cleveland Clinic is that you get the expertise of an entire team. Every individual on the team will have a very serious expertise and skill sets and you'll really be able to leverage the experience of the team. From when it comes to the C-TEF program, Dr. Heresi and myself, along with our vascular medicine colleagues, and our interventional radiology colleagues among many others, we meet together to evaluate each patients and to come up with a specific treatment targeted for them. We'll look at all their investigations, look at all their imaging, look at ... and see these patients in person and then we can come up with a treatment plan that's specific for them and they will have the most likelihood, the chance of success.

Likewise, a lung transplant program, there is multiple member of the team; pulmonologists, surgeons, social workers ... and also other individuals, dieticians and it's really so that we can treat every patient as an individual. It's not uncommon when we see patients that they've been symptomatic for a long time. They've bene having symptoms for six months, a year, two years, three years and they really haven't had a good understanding of why their symptoms are they way they are. They may have been started on various treatments, they've been on ... may have been misdiagnosed as a pneumonia or et cetera and the treatment really hasn't been working and it's not until they come and see us where we can then try to figure out exactly what's effecting them.

And once we have the right diagnosis, that's ... only then can we really start the right treatment for them. So, as part of the C-TEF team, since we've been working together as a team over the last ... over the last five to 10 years, our outcomes have improved tremendously. Finding the right patients to take to surgery, finding the right patients to treat up with medications, finding the right patients for ballon angioplasty, we've gotten our mortality in C-TEF down to about 2 - 3% and this is an operation that, historically, in most places will have mortality of about 10% or so.

So, and likewise with the lung transplant team, they've had incredible outcomes and been able to bring patients from all over the United States and they've bene all over the world, too, so that's the real benefit for patients to come to the Cleveland Clinic. We're able to ... we will meet you and evaluate you as a team. We'll help you find the underlying cause of your disease. We are able to give you every treatment modality and get you involved in multiple clinical trials so that you have access to the most advanced therapies and we can do that with excellent outcomes.

Dr. Jacob: I think one important thing to know about pulmonary hyperension, although hit's called pulmonary hyperension, it actually requires pulmonologists, cardiologists, cardiac surgeons, thoracic surgeons, all kinds of different specialties to treat it and we feel like patients where there's a suspicion for this disease, should come to a center that specializes in this so that they can have the proper diagnosis and then start on the proper treatment depending on what that diagnosis is and how they're defined.

And our team here, as you see, is made up of different individuals with different training backgrounds and I think that gives us a great advantage to identify the right diseases and treat patients properly and individualize their treatment plan.

Dr. Heresi: Absolutely and let me just add to that, so that people know so, that is absolute true. We have an excellent expert, multi-disciplinary team but we also will partner with your local docs, right-

Dr. Jacob: That's right.

Dr. Heresi: ... for sure it's a relationship that we build because obviously, you know, we realize this ... that sometimes difficult to come down here. It's a big place, and so we would always partner and build that relationship with your doctors so that we, you know, they are part of the team. They are really part of our team when we, when we take care of you over that ... over the long term.

Dr. Jacob: That great. All right, thank you so much.

Announcer: Thank you for listening. We hope you enjoyed the podcast. We welcome your comments and feedback. Please contact us at heartatccf.org. Like what you heard. Please subscribe and share the link on iTunes.

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