Dr. Michael Cuttica, MD, pulmonologist at Northwestern Medicine, talks about a new technique for treating a disease called CTEPH.
Interview conducted by Ivanhoe Broadcast News in June 2022.
What is CTEPH?
CUTTICA: CTEPH is a disease. It stands for chronic thromboembolic pulmonary hypertension. What that means is in patients, typically who either have a pulmonary embolism or sometimes people who don’t recognize they have pulmonary embolism or a clot in the lung. CTEPH develops when the clot doesn’t fully resolve itself and the clot over time turns into scar tissue inside the blood vessels in the lungs, which then affects the way blood can flow through the lung. You develop a back pressure that puts strain on the heart and leaves the patients feeling short of breath and limited.
Is it life-threatening?
CUTTICA: Untreated CTEPH can be life threatening. The great thing about this disease, although it’s a terrible disease and leads to lots of symptoms and life-limiting type of issues for patients, it is a curable disease. So, as long as we identify it, or physicians identify the disease, get people appropriately worked up and treated, we have options that can cure the disease and get people back to normal life.
Do you think it can be or people don’t take care of it as quickly as you would if you had a heart attack or something because you’re not feeling that?
CUTTICA: This is, I think, a very under-recognized disease. There’s a lot of data out there around the diagnosis of pulmonary embolism or acute blood clot in the lung. People have heard of that. People have seen it and know people that have it. We have a lot of data about what to do as far as how long to treat people with blood thinners after an acute blood clot. But there’s a real lack of knowledge around what happens to people after they’ve been treated with a blood thinning medication. How many people go back to normal? How many people develop these long-term complications like chronic thromboembolic disease? So, it is often missed. A lot of symptoms can be very limited. And these symptoms are oftentimes attributed to getting older, or out of shape, or gaining weight, or having asthma, etc. And always in the background is this diagnosis of, well, I had a clot a couple of years ago and life never really got back to normal. And that can be missed sometimes. And eventually, the workup will progress, and they don’t respond to asthma treatments. Or you say, well, wait a minute, I’m not that old. I shouldn’t be that short of breath. And someone gets the diagnostic test and says, wait a minute, that clot looks like it’s not gone. And what do we do about it now?
So, how do you treat it?
CUTTICA: So, when someone finally presents and has the screening test that picks up the presence of a chronic clot, there are basically three treatment pathways that we have that patients could take.
Can a PET scan see it?
CUTTICA: So, eventually, the way we diagnosis disease, oftentimes it’s progressive shortness of breath, limitations in their ability to exercise. And then often, the recognition of the diagnosis comes from an echocardiogram of the heart that shows that the right side of the heart is struggling to push the blood through the lung. Once you identify that and you start thinking around, well, what could be obstructing blood flow through the lung? Oftentimes, the next step is patients undergo a CAT scan of the lung or a CT scan that’s designed to look for clot inside the lung. And if you’re looking for chronic clot, you can pick it up and see there’s evidence that the clot didn’t resolve itself, the previous pulmonary embolism didn’t resolve itself. Once that diagnosis is made, then we have three, basically three treatment pathways the patients can go down. The best treatment pathway, the pathway that offers the potential for a cure of the disease is the surgical pathway, or a pulmonary thromboendarterectomy, which is surgical removal of this chronic clot tissue from the lung. If people are not surgical candidates or if they’ve had the surgery and end up having residual clot inside the lung, then they have nonsurgical interventions like a balloon pulmonary angioplasty that’s done in the cardiac catheterization lab where we use wires and balloons to push the clots out of the way. And then in patients who aren’t candidates for either of those surgeries, we have medicines that are designed to try and make the blood vessels in the lungs open up to allow more normal blood flow around the chronic clots that are in there.
Why wouldn’t you be a candidate? Is it age-related, health-related?
CUTTICA: No. The candidacy for any of these interventions comes down to many factors. There is no age limit or weight limit or any of those types of things for being a candidate for the surgery. Candidacy for the surgery revolves around where the clots are in the lung. Are they small clots far out in the small blood vessels so that the surgeon can’t get to it to surgically remove them? Or does the patient have a lot of other medical problems that would make them not a good candidate to undergo? It is a major surgery. So, once we have a team of doctors that includes surgeons, cardiothoracic surgeons, cardiologists, pulmonologists, hematologists, an entire team that gets together and reviews these cases once a week to decide, do we think this patient is a good candidate for surgery, for balloon pulmonary angioplasty, or for medical management?
Tell me about Em.
CUTTICA: I met Em a little over a year ago. I grew up in the 1980s, so I was a Celtics fan in the ’80s, which was the era of Larry Bird and Robert Parish. Em was a little bit before that, but I certainly knew who he was, and I was excited when he was referred to see me. So, after I finished fanboying around the fact that he was an NBA champion, we got down to talking about his clotting events. What he and his wife told me was, several years ago he was very active, even though he was in his late 70s, early 80s, swimming regularly, playing basketball, being very active. And then, he had a series of clotting events, or pulmonary emboli in the lung, and never fully recovered from it. He was treated appropriately. He was put on blood thinning medications. And over the preceding next couple of years, he had to stop swimming. He couldn’t exercise anymore. And when I met them, he got to the point where he couldn’t even go for walks with his wife and keep up with her anymore because he was so short of breath and so limited. With his history of pulmonary embolism, we saw his echocardiogram showed the right side of the heart was under quite a bit of strain. He ended up getting a CT scan that confirmed that the clots were still there, that they had not resolved themselves even though he had been on blood thinning medications. And then we finished the work up and he was given the diagnosis of chronic thromboembolic pulmonary hypertension.
Were there a couple of clots in there or a lot?
CUTTICA: This is a very common question that people ask, how many clots do I have in my lung? And that can be a very difficult question to answer because oftentimes, these clots sort of shower into the lung and then over time, they change from being what we think of as an acute blood clot, like when you cut yourself and you start bleeding, and you form a clot to stop the bleeding. These clots evolve and become scar tissue. They become part of the wall of the blood vessel as scar tissue in there. So, maybe clotting is a little bit of a misnomer because they’re no longer true clots as we think about clots. They’re scar tissue that are part of the blood vessel wall. So, when we look at the CT scan, we see oftentimes, diffuse changes in the blood vessel wall that goes throughout both lungs, which was the case in Em. He had multiple clots or lots of changes in the blood vessels throughout both of his lungs.
How’s he doing today since you treated him?
CUTTICA: Once we finished the work up and presented him at our group meeting, everybody agreed, even though he was on the older side, that he was a great surgical candidate. So, he underwent his pulmonary thromboendoarterectomy surgery and did amazing. He came out, tolerated the surgery well. I think he was in the hospital for about a week afterwards, which is average length of stay following the surgery. And he’s done great since then. He’s become more active. He’s going through a rehab program right now, getting stronger and starting to start to exercise and lead an active life again. I look forward to the day where I get to go and shoot some hoops with him.
Do you think Em being an elite athlete helped?
CUTTICA: I do think that plays a huge role, especially for him. It played into our decision. I mean, he was in his 80s. So, that did play into our decision around the risk of surgery and trying to figure out if he was a good candidate. The fact that he is an elite athlete did figure into our decision about how well he would tolerate the surgery. Despite the fact that he was in his 80s, he tolerated the surgery very well, came through it and made a solid recovery.
END OF INTERVIEW
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