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Interviewer: Treating chronic thromboembolic pulmonary hypertension, also known as CTEPH. We're going to find out more about that surgery next on The Scope.

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Interviewer: You've been diagnosed with CTEPH and we've already learned about the condition, its symptoms, and stuff like that in our earlier podcast. But today, we want to talk about the surgery that treats it. Dr. Craig Selzman is a heart surgeon and one of the directors of the Chronic Thromboembolic Pulmonary Hypertension Program at University of Utah Health Care. So in case somebody didn't hear the first podcast, just briefly sum up what's going in the heart that causes this disease?

Dr. Selzman: It's actually, believe it or not, it's in the lungs and so the lungs are sandwiched between the right side of the heart and the left side of the heart. And so what CTEPH is, we'll just call it CTEPH, it is a form of what we call pulmonary artery hypertension. So pulmonary artery hypertension is high blood pressure in the pulmonary circulation. And there are a number of causes for it for which CTEPH is one of them.

This particular situation is a form of PAH, pulmonary artery hypertension, that is related to having blood clots that come from your legs or the lower extremities usually and get lodged into the lungs. We call that a pulmonary embolism. It's very highly morbid, causes a lot of problems, and it's lethal. It's one of several leading causes of cardiopulmonary death that can happen acutely.

And so what happens also inside the lungs is that the lungs actually start to remodel. You could almost imagine like you have some stuff in your sink and it gets into drains and it's kind of there, but you could imagine that a year or two later, it kind of forms and becomes part of the wall of the pipes. And that's what's happening inside your lungs and it becomes very hard for them to do their job, which is to take in oxygen and get rid of carbon dioxide.

Interviewer: So then over time, is it the stiffness that's causing that problem, that build-up of stuff in there that's causing the stiffness?

Dr. Selzman: That's right. We want our lungs, you know, you want your lungs to look like the sponge that you just bought out of the store. You open it up, it's this very light, airy, and it's just . . . you could kick it and it would maybe go up in the air, but it's not this heavy wet sponge that you've just finished cleaning the dishes with. That's what you want your lungs to look like. And you want all those little holes because all those little holes allow oxygen to go back and forth. But if you don't have good blood vessels that go to all those little holes, all of the ability of your lungs to do that work become compromised.

Interviewer: So during this surgery, what do you do to fix it when you go in there?

Dr. Selzman: So believe it or not, this is a roto-rooter operation. What we do is we have to do this, it's a major heart operation in the sense that we have to open up your breastbone. We do have to open up the pulmonary arteries. And then what we do is we open up the pulmonary artery and we actually peel out the inner layer of the blood vessel wall.

And so, sometimes, there's actual blood clot that you remove, but it's not really just the blood clot. It's you have this really thick rind that's layering out along the blood vessel wall and you have to remove this whole rind in order to allow the blood flow to get out to the periphery of the lung where it does all of its work. In order to do this, it's a major operation, you have to go on the heart-lung machine.

We actually have to take the body temperature very low because there's a lot of blood that gets in the way when you're doing this and so you need to be able to see. And so, sometimes, we actually have to even turn the circulation of the patient off and the only way to do that is to take the blood temperature very, very low to protect the brain and other organs. So it's not something that we take lightly. It's a very relatively conceptually, straightforward operation but has some pitfalls if you don't do a lot.

Interviewer: How long does it take for an average for you to do the procedure?

Dr. Selzman: Probably three to six hours.

Interviewer: Okay.

Dr. Selzman: And a lot of that time, the nitty-gritty work is actually only maybe less than an hour, but the prep time to get ready and to take the temperature down and then to bring it back up, it does take some time.

Interviewer: And you're able to go in and get most of that, if not all of that, usually cleared out?

Dr. Selzman: We are fortunate because we have really good preoperative testing so it might be that all of the lung is affected, but sometimes, it'll be just half the lung or, you know, three-quarters of the right lung and two-thirds of the left lung. And so we can kind of target that.

There are some disease processes with these pulmonary embolisms and the CTEPH that is stuff that we cannot fix. And that is the stuff that gets way, way deep out into the periphery of the lung and we just physically can't do it. When people have pulmonary hypertension related to that kind of disease, there' really only one out outside of medical therapy and that would the lung transplantation.

Interviewer: Got you. So I think you just answered who makes a good surgery candidate. If it's affecting the outside part of your lungs not so much, but if it's more in the main part.

Dr. Selzman: Yeah, I think that's a good way of thinking about it, you know, from a technical aspect. The more proximal or the less further out into the periphery defines some of the patients that we would just not even think about doing.

Interviewer: Got you. What kind of preparation does the patient go through leading up to it? Is it just typical surgery preparation, you just want to be healthy?

Dr. Selzman: Yeah. Unfortunately, you know, some of these patients aren't so healthy.

Interviewer: Because they have a hard time breathing, right? Which makes it hard to exercise and move.

Dr. Selzman: Exactly. And, you know, and also risk factors, which led them to have developed blood clots in their legs. You know, it's sometimes the blood clot is the classic traveler across country on an airplane and they're not moving their legs and they get the blood clot in the leg and then they later that day they walk around and then they get acutely short of breath and they might not even know that they had something happen.

As a matter of fact, about 40% of patients that have pulmonary embolism don't even know that they have it and a lot of CTEPH comes without an antecedent diagnosis of pulmonary embolism. And it's just something that happened that nobody . . . you just didn't know you had it. It's kind of like the silent heart attack. "Oh, I didn't know I had a heart attack, doc." It's the same kind of thing. "I didn't know I had a pulmonary embolism, doc." And so that can happen and it can be kind of very sublime, if you will, the development of the disease.

Interviewer: Got you. And then what's the recovery time look like after the procedure normally?

Dr. Selzman: Usually, in the intensive care unit for two or three days and then up on the floor for several days. Everybody is a little bit different how they do this. In the more severe cases, you can actually be in the hospital for several weeks because there's part of the lungs that aren't used to having seen blood flow and we acutely remove this stuff and then we get what's called reperfusion injury, which means of the lungs which hadn't had a lot of blood flow all of a sudden gets this rush of blood that comes to it. And that's called reperfusion and sometimes that can be very troublesome to deal with.

And so, yeah, this is not an easy physiologic process for the patient. I mean, the lungs are going through a lot of stuff. Obviously, it's very central and core, you know, heart and lungs. And so, you know, if the lungs are working good, then the heart works good. But if the lungs aren't working good, then it affects the heart and it can be a problem.

Interviewer: So you're taking a little bit of time off from work?

Dr. Selzman: Oh, yeah.

Interviewer: Probably.

Dr. Selzman: This is a major heart operation, but the cool thing about this as, you know, as big as of a procedure as it is, we just have some great stories that come back. I mean, you see these people that are on oxygen at home and then two weeks after surgery, they come and see you in clinic and they're off of oxygen and they say that, "Wow. I haven't been able to take a deep breath like this in years." And then you see them a year later and they're just so thankful because they can breathe because we see this in lung transplant.

You know, at the University of Utah, we're kind of lucky because we're one of the, we're really the only lung transplant center in the entire region. You know, you have to go to Denver or Phoenix or in California. And so we see great stories and when you see people that can't breathe, you know, just imagine you're down swimming and you're underwater. I mean, that's what these people are. And so when you allow the folks to actually take a deep breath and also the plastic hose of the oxygen just gets really old. And so it can be an incredibly gratifying thing to do for a patient.

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Dr. Miller: You have advanced heart failure. Would you ever need an artificial heart? This is Dr. Tom Miller on The Scope Radio. We will talk about that next.

Announcer: Medical news and research from University Utah physicians and specialists you can use for a happier and healthier life. You are listening to The Scope.

Dr. Miller: I am here with Dr. Craig Selzman. He is an Associate Professor of Cardiothoracic Surgery in the department of surgery. He is also an expert in heart surgery and implantation of artificial hearts.
Craig, what types of patients that have heart failure might merit a discussion about having an artificial heart implanted?

Dr. Selzman: I t's actually lower than you think. The reality of it, there is probably about five to six million folks out there, many of those that might be listening that have the diagnosis attached to them of heart failure. But of that group, the folks that have really advanced disease, it's much smaller. Not that much smaller but probably anywhere between two and 800,000 people.

Dr. Miller: Nationally, that's a big number.

Dr. Selzman: It's a big number. It's a big number. And then the folks that really have really bad stuff, it's probably about 80,000 a year. But the important thing to know about that is that everybody thinks, "Well, if my heart goes bad, I'll just get a heart transplant. That's what they do, right?" The only problem with that is there is only about 2000 to 2500 hearts a year. So you can see the disparity right off the bat that if there are only 2500 hearts that are available for transplant for the entire country, and there are 80,000 to 150,000 patients that could potentially benefit, we got a real problem there.

Dr. Miller: Well, we see that with all type of organ transplants now. Whether it's liver or kidney or pancreas or lung, there are not enough donors. But it sounds like the artificial heart is a way for people to have advanced therapy who might not be able to get to a heart transplant right away.

Dr. Selzman: Yeah, that's true and that's how we use not just the artificial heart. Artificial heart is often used to symbolize a lot of things. There are heart pumps or internal or external pumps that support your circulation is used. The artificial heart, the original one, the ones that we associate with Barney Clark and others, actually you remove your existing heart and you put in two ventricles, two pumping chambers that are combined to make a total artificial heart.
The way the majority of patients use . . . about 95% of the folks that need assistance, we use devices that support a single ventricle. And the most damaged ventricle, typically, is the left ventricle. So that's why we developed these devices called LVADs or left ventricular assist devices. That's what we most commonly use.

Dr. Miller: How does a patient with advanced heart failure and the need for surgical treatment make their way to you or a specialist?

Dr. Selzman: There are fantastic doctors in the community that can manage the early stages of heart failure. Early stages are folks that might just be on some blood pressure medicines and they get some shortness of breath, maybe they're put on a water pill that help them deal with that. But what we find is that when patients get a little bit sicker and they're going up on some of these medicines and then even worse, when they are unable to tolerate some of these medicines because their blood pressure is a little bit low or they're still fatigued, these are kinds of patients that we would recommend to be seen by someone with some experience at advanced levels of heart failures.

Dr. Miller: I'm assuming that would include a team perhaps, maybe a cardiologist who specializes in heart failure as well as a surgeon and some others. Is that right?

Dr. Selzman: Absolutely, and the University Utah, we have a team of almost 70 people that are involved in the care of various patients with heart failures, from the not so sick to the very, very sick. But it does require a team to care of all this and dedicated to understanding the particular needs of this group of patients

Dr. Miller: So how would a patient advocate to see someone or a group that specializes in this type of advanced care? They are under the care of their internist or family practitioner. At some point, they may need this specialized care.

Dr. Selzman: Yeah, it's a good question because most people that have various mid-level staging of heart failures, they are not the ones that aren't that sick or the very, very sick-they are kind of that gray zone in the middle-don't really appreciate how bad their heart failure is. And maybe their primary care provider is not maybe fully appreciating the scope of how much disease they have. As such, you are potentially missing some therapies that might otherwise not be offered to you. So, we need to have people to recognize that this disease is rampant and recognize that there are more than one or two medicines that can be used for it.

Dr. Miller: Well, it also seems to me that the earlier the patient would get into a specialist group, the better chance they would have of designing the optimal therapy over time for that particular patient. So it would be better to get them mid-way through the process then later on when things are really going south.

Dr. Selzman: Yeah, I think that's a great point because the way that our group looks at it is we are partners to the providers and the community and the patient, most importantly the patient. So we try to team up with the patient and what the best thing is. At the University of Utah in particular, our scope is 14, 15 states of people coming in. So we have some unique challenges. We cannot be everyone's heart failure doctor. So we work with the group, wherever they are coming from, to try to help that patient to find what the best pathway is.
But I totally agree with you that we'd love to see these patients early on, and we've followed the patients for decades before their heart has actually progressed to the point where it needed something in an advanced therapy fashion.

Dr. Miller: And so these patients, when they do end up with a left ventricular assist device, tend to do quite well over time and the technology now allows these patients to do better, be healthier and people shouldn't really think about the old days. The technology is very different now than it used to be.

Dr. Selzman: We'd love to put on our little quarterly newsletter showing all the pictures of people doing their fly fishing, they're playing golfs, some of these got some moose hanging over them. You name it, you can do it. You can live life and mind you, that by the time we are seeing these patients, most of the people are not living life. They are basically struggling to go from the couch to get to the restroom or couch to get to the refrigerator, and these kinds of therapies give back life.

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Dr. Miller: About 32 years ago, Barney Clark had the first artificial heart implanted here at the University of Utah. We're going to talk about how things have changed since then on The Scope Radio. This is Dr. Tom Miller.

Announcer: Medical news and research from the University of Utah physicians and specialists you can use for a happier and healthier life. You're listening to The Scope.

Dr. Miller: I'm here with Dr. Craig Selzman. He's an Associate Professor of Cardiothoracic Surgery in the Department of Surgery here at the University of Utah, and Craig is an expert in heart transplantation as well as ventricular assist devices, which are the new artificial hearts.
About 32 years ago, Barney Clark was the first man to undergo an artificial heart implantation, and that was here at the University of Utah. Now a lot has changed since then, I think. Dr. Selzman, can you talk to us about what's new with artificial hearts?

Dr. Selzman: Since that time, so much has happened. It's been in two or three different areas, and the first area is just the technology.

Dr. Miller: That's got to have changed tremendously.

Dr. Selzman: It's huge.

Dr. Miller: I remember the pictures of Barney Clark. The pumps were huge. They had to wheel it around with Barney Clark. It was noisy. It was loud. It was terrible.

Dr. Selzman: It's a Zamboni machine. We still have one here in the hospital, if necessary, to clean the ice outside. But the deal now is that we have these pumps so small and they're very patient-friendly so that the patients can actually go home. They can do everything. They can play 18 holes of golf, walking. You name it; they can do it.
The one thing that is a little bit difficult is there's electricity going through this; they're not going for a swim any time soon, but that's even changing. We have pumps now that allow people to have artificial heart support that can actually go swimming with internal batteries and stuff. So it's been huge.

Dr. Miller: So are the pumps still external to the body, or are they within the body now?

Dr. Selzman: They're all within the body. We still have some that are used externally. Those are used for really the sickest of patients that we're really just trying to do whatever we can to save their lives. But in terms of folks that you want to actually go home and regain life, the thing about a lot of this field is that the chances of you having a long life with advanced heart failure is low. Probably the patients we're talking about, less than 10% are alive at two years.

Dr. Miller: I mean, that's as bad as many cancers.

Dr. Selzman: It's metastatic cancer. It's basically the same thing. I use an analogy a lot, and the one thing that the VADs have done in this artificial technology is that it's the therapy that is so much better than chemotherapy, meaning that you can put somebody on with an advanced cancer, and you might buy another two, three year, four months. We're talking about buying five to 10 years. And not only just years, but also quality years. These patients are back with their families. They're going back to work. They're living life. It's amazing.

Dr. Miller: Craig, does this mean that actually having a heart transplant may become obsolete?

Dr. Selzman: Ah, that's a great question, and it's one of the current debates within our community because the life expectancy, the one year survival rate for patients that are getting these machines is almost as good as getting a heart transplant. The problem with heart transplant: great therapy. There are just not enough of them.
For the last 25 years, we've done about 2,000-2,500 hearts a year in the United States, but there are probably 80- to 100,000 folks that need it today. So these pumps have really filled that gap. Early on, because the pump technology wasn't quite so good, you'd have complications. But now the complication rates are actually very manageable and the survival is quite good. One year survival is over 90% right now.

Dr. Miller: I remember in the Barney Clark days that that clots arising from the use of that type of pump were a real problem. That's pretty much eliminated now, is that right?

Dr. Selzman: Whenever you have blood that goes through a washing machine or a Kenmore or a Maytag, it's just not normal. And so we still are faced with this complex problem of hemostasis, thrombosis, and anti-coagulations. For the most part for these pumps, you will be taking some blood thinner medicines.

Dr. Miller: But the risk of having a clot is lower?

Dr. Selzman: It's much lower. I mean, it hearkens back to the old heart valve days where we used to have that had a ball that would go up and down, the old Bjork-Shiley valves and stuff. We don't use those any more, and so the whole field is new.

Dr. Miller: The whole technology.

Dr. Selzman: Yeah.

Dr. Miller: What does the future look like in terms of these devices? I mean, do you see these devices becoming smaller, like calculators becoming smaller and smaller?

Dr. Selzman: Yeah. I think there are two or three things that are going to really make a difference here, and you could actually trace back. If you look at the pacemaker defibrillator field, defibrillators used to be these big giant things that you used to put in the abdominal cavity, and now they're these little things below your collarbone.
So the same things happened in the VAD world. They're getting smaller and smaller, and because they're getting smaller and smaller, the complications are less and less, you're actually going to start putting these things in patients that are less sick to try to get them off this spiral of decline that happens with heart failure. So size definitely better, complication rate less. And then the main thing that's coming from an industrial standpoint is power. So just like you're trying to get batteries in that 747, or what was it, the 757, the lithium battery was the only bad thing.

Dr. Miller: Exactly.

Dr. Selzman: So they're starting to do the same thing with these VADs. And so a lot of the original DeBakey pump, believe it or not, was a collaboration with N.A.S.A. to help do these things.

Dr. Miller: Spinoff technology.

Dr. Selzman: Yeah, so there's a lot of cross collaboration there. The interesting thing I think really where the field is going, it's not so much about the pump because the pumps are getting better and better. But it's about the patient and the biology of the patient. And our goal is not to actually put the pump in to have them have it for the rest of their life or for them to get a transplant, but actually to get better so that we can remove the pump.
So how do we do that? Well, we're going to recondition the heart, probably use other biologic therapies to help us. So the main thing that I see the field going in the next five to 10 years is not so much about a pump, but about a patient that has a bad heart. How do you make that heart better with the assistance of a heart pump so that you can actually remove it so that patients can live long lives that way?

Announcer: We're your daily dose of science, conversation and medicine. This is The Scope. This is the University of Utah Health Sciences Radio.

Craig Selzman: This is Craig Selzman. I am an Associate Professor of Surgery in the Division of Cardiothoracic Surgery here at the University of Utah, and I am the surgical director of our Heart Transplant Program and our Mechanical Circulatory Support and Artificial Heart Program.

Host: In the news, we've heard Randy Travis had something called cardiomyopathy. Am I pronouncing that right?

Craig Selzman: Correct.

Host: What exactly is that?

Craig Selzman: Cardiomyopathy is a generalized word. Cardio, heart. Myo, muscle. Opathy, not working so good. So basically it means that the heart muscle itself is not working so well.

Host: And there's a virus that causes this. Is that correct?

Craig Selzman: There are a lot of different things that can cause a cardiomyopathy. So the word that leads before cardiomyopathy describes why it had happened. It could be because of bad blood flow. We would call that ischemic cardiomyopathy. It could be because of something that we have no idea, which we would call it idiopathic cardiomyopathy. But if we think it's a virus, then we'll call it a viral cardiomyopathy.

Host: Yeah. And in the case of Randy Travis, it indeed is.

Craig Selzman: Well, that is the presumption.

Host: Okay.

Craig Selzman: To the best of my knowledge, it hasn't been confirmed. I think that's what everybody thinks is going on.

Host: All right. And this condition causes the heart to weaken, if I'm correct?

Craig Selzman: That's correct.

Host: And then what's the cure for that? What did they actually do in his case?

Craig Selzman: Well, the cure for the majority of patients, 80, 85, 90% is absolutely nothing. It's just supportive care. But there is this very small group of patients that go into cardiovascular collapse. That means that the heart muscle is unable to pump enough blood around the body to get to the brain, to the kidneys, and to other organs and such that they need extra help. A lot of the times, that extra help can be managed with medicines in the vein, but if that doesn't work, sometimes you actually have to use mechanical means to do that. And that's what happened for Randy Travis.

Host: Is this an invasive surgery? Do they have to open his chest to get it in?

Craig Selzman: No. This is not really surgery. What it is is basically a catheter that's snaked up through one of the blood vessels that's easily accessible through the skin in the groin. So we call it a percutaneous heart pump. It's not really surgery in the classic sense.

Host: But you're hosing that thing up through the groin all the way to the heart. How difficult of a procedure is that?

Craig Selzman: Just like a lot of things in life, 85% is very straightforward, and 10 to 15% can be challenging.

Host: Sure. But for the most part . . .

Craig Selzman: Conceptually, it's very straightforward. Technically, it's very straightforward, for the most part.

Host: And nowadays, it's fairly common?

Craig Selzman: Fairly common. We did one on Monday.

Host: All right. The device that they placed, is he going to have to have that in him for the rest of his life?

Craig Selzman: No. This is a temporary device. It's actually meant for really short periods of support. It could be hours or days.

Host: Okay.

Craig Selzman: The hope here, with someone with his kind of situation, is using this pump to try to support his organs, giving them blood flow while the doctors down there figure out is his heart muscle going to get better or not.

Host: Gotcha.

Craig Selzman: And what this pump does or what any mechanical pump does is it allows the heart to just rest because it takes over, giving blood flow to the rest of the body. If you don't have it, his heart is having to work so hard and it's just tired. By doing this, it allows the heart to relax and just kind of get a little bit of rest, get some new energy.

Host: Kind of like any other muscle, you're just giving it a chance to recover?

Craig Selzman: That's right. So by doing that, it gives the physicians a chance to see whether or not his heart will recover or not. If the heart recovers, let's take the first situation, you can just pull this thing out, and then it carries on. The majority of patients that have this problem can go onto live normal, active lives. A subgroup of those patients that won't get better will kind of limp along a little bit and become heart failure patients. And then there's this other category of patients that maybe will not be able to survive without a pump like this. In that situation, what we would do is we would switch this percutaneous pump to something that's a little bit more durable.

Host: Okay. So is he looking at some lifestyle changes if he recovers?

Craig Selzman: I don't know enough the guy to, I can imagine.

Host: Some alcohol use has been recorded.

Craig Selzman: He's lived a hard life in many ways, and that could be contributing to this. And certainly if you live a cleaner life, sometimes you can reverse a lot of this stuff, especially with the heart.

Host: Would a lifestyle change have prevented this from happening in the first place?

Craig Selzman: Potentially. But if it's truly viral cardiomyopathy, this can attack anybody. I mean, in the pediatric community, you see this much more frequently than in the adult community. This is a very promiscuous disease. The virus doesn't care who it attacks. Is your heart maybe more susceptible to it? Is somebody's heart more susceptible to it? Possibly.

Host: What would cause that?

Craig Selzman: Let's say he had lived a hard life and had an alcoholic cardiomyopathy, which is a type of thing that happens. For other folks, after pregnancy have a thing called peripartum cardiomyopathy that makes their heart a little bit weaker, so they're more susceptible to having other insults attack their heart.

Host: What kind of recovery time would you look for after a procedure like Randy Travis had?

Craig Selzman: I would hope that in the next three to five days that they'll have a sense of whether or not the left ventricle, there are two main pumping chambers of the heart, the left ventricle and the right ventricle. This pump is supporting his left ventricle. The hope would be, then, in a three to five period, that they would have some idea of which of those tiers he's in. Is he going to be somebody that's going to get completely better and pull the pump, not really better but let's pull the pump and see how he does without the pump, and then the third one being it isn't going to work and we're going to have to do something else. But usually within three to five days, you have an idea of what's going on.