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Treating CTEPH Without an Organ Transplant

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Treating CTEPH Without an Organ Transplant

Apr 19, 2017

Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious lung condition that can arise after an individual suffers a pulmonary embolism. The condition can lead to a buildup of tissue in the lungs that often leads to high blood pressure, heart strain and eventual heart failure. Luckily for patients, there is a surgery available that can treat CTEPH without a full lung transplant. Heart surgeon Dr. Craig Selzman explains the steps involved in the six-hour “roto-rooter operation.”

Episode Transcript

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

Announcer: Health tips, medical news, research and more for a happier, healthier life. From University of Utah Health Sciences, this is The Scope.

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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