Chronic thromboembolic pulmonary hypertension (CTEPH): What Patients Should Know

Podcast Transcript

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Dr. Nick Smedira: Hello, and welcome to the Cleveland Clinic. I'm Nick Smedira, one of the cardiac surgeons here at the clinic and today I have a distinguished panel that we're going to talk about the diagnosis and management of chronic thromboembolic pulmonary hypertension and with me, my distinguished colleagues include Dr. Gustavo Heresi, who oversees our chronic Thromboembolic Pulmonary Hypertension Clinic.  Dr. Michael Tong, a colleague of mine in cardiothoracic surgery, who performs the operation to remove the clots and the scar. Dr. Ihab Haddadin, who is an interventional radiologist, who treats patients with both acute and chronic pulmonary emboli and Dr. Jerry Bartholomew, one of our vascular medicine specialists, who is an expert in the medications needed to treat these patients. Gustavo, starting with you, what is chronic thromboembolic disease or pulmonary hypertension?

Dr. Gustavo Heresi: It's a mouthful, that's why people call it CTEPH, for short. The PH part stands for pulmonary hypertension, which simply is high lung pressures, high blood pressure inside the lungs, just like people have high blood pressure in the arms, for some you can also have high blood pressure in the lungs. Thromboembolic is just a fancy medical term for clot and chronic means it's been there for a long time, so chronic thromboembolic pulmonary hypertension is high lung pressures, due to blood clots that happened sometime in the past, never went away, and that typically leads to scar tissue inside the lungs and the common symptoms of that would be mostly shortness of breath.

Dr. Nick Smedira: Can you get it from smoking?

Dr. Gustavo Heresi: Well, smoking, as Jerry would tell us is a mild to moderate risk factor for blood clots, but in terms of risk factors specifically for CTEPH or chronic clots, not really. But, certainly, smoking is a risk factor for blood clots. In an indirect way, it could.

Dr. Nick Smedira: So when a patient has pulmonary hypertension, are there medications you can use to treat them and are those medications appropriate if they're clot-based or do you treat those differently?

Dr. Gustavo Heresi: Yeah, that's a great question. Pulmonary hypertension, in and of itself, is not a disease. It's just a term that says high lung pressure and there are many reasons behind that. One of them is blood clots. And if you find blood clots at the reason for it, then we have these very interesting interventions such as an operation to remove the clots, balloon procedures I'm sure we'll talk about, that are very effective in treating pulmonary hypertension due to blood clots. So if you are a patient and you have pulmonary hypertension, you have to make sure that your doctors have screened you for chronic blood clots. And, again, the way to do that is with these tests called the ventilation perfusion lung scan and then putting that together with CAT scans or imaging studies.

There are other reasons for pulmonary hypertension such as heart problems, lung problems and there are also a group of people who have lung pressure elevation without an obvious reason, or perhaps related to some condition such as holes in the heart or connective tissue diseases and others. Only for that latter group, these medications that we have currently available are quite effective in treating pulmonary hypertension. But the key really is, for prescribing a medication, the key is to understand why you have lung pressure elevation, because the treatment will be very different.

Dr. Nick Smedira: So, for the most part, this disease is a mechanical problem that surgery or something will help, more so than medicine?

Dr. Gustavo Heresi: Absolutely. The way you're putting it is perfect. This is a mechanical problem that requires a mechanical solution.

Dr. Nick Smedira: Solution. Jerry, Dr. Bartholomew, the patients are often on blood thinners when we see them. What's the current state of blood thinners that you would expect to see a patient on or what would you ... Most patients with these clots would be on some form of blood ... And do they need it for their entire life, or can they come off of it at some time?

Dr. Jerry Bartholomew: Sure, good question. Fortunately, we have multiple options now, which we didn't have before. Someone who was on Coumadin for life or if they were, they weren't happy because of complications from it. Diet effects it. Their INR had to be monitored regularly and their INRs were often all over the place. In fact, most people were only therapeutic within 60-70% of the time, so the DOACs have come on. They've also been called direct oral anticoagulants, or new oral anticoagulants or novel oral anticoagulants.

Dr. Nick Smedira: Is this the ones I see on TV all the time?

Dr. Jerry Bartholomew: Yeah, sure.

Dr. Nick Smedira: Ask your doctor about Xarelto or what's the other one?

Dr. Jerry Bartholomew: If you're a world-renowned golfer, you might be advertising one of these drugs, I guess, but that's the one you would see and the advantage is they don't have to be monitored. Now, that's not totally true. They should be monitored every six months or at least once a year if you have normal renal functions with a blood count and renal and liver function tests, but that's a great advantage. You can eat whatever you want. You can do whatever you want, although you still are on a blood thinner and we urge patients to avoid contact sports. The length of therapy is still always a difficult question and the American College of Chest Physician Guidelines which say if you've had an unprovoked event, a venous thromboembolic event, meaning a DVT and a pulmonary embolism and we don't know what caused it, then you should be considered for lifelong anticoagulation. And if you're a man, the risk of a recurrent clot is much higher than a woman. So men often need lifelong anticoagulation.

Dr. Nick Smedira: Oh, that's a great answer. Mike, this is an operation we do here at the Cleveland Clinic. We have quite a long history with it. Tell the audience what this operation is like and how you do the operation?

Dr. Michael Tong: Yeah, this is a big operation. It's one of the bigger operations that we do in cardiac surgery. The operation typically takes about four to five hours. The operation requires that we put patients on the heart-lung machine and then once on the heart-lung machine, we will cool the patient's body from 98 degrees, down to about 65 degrees and once we're at 65 degrees, we would shut off the circulation completely, so blood will no longer be moving in the patient. And then, by having the patient cooled, it gives us the safety that we need. It takes us about 20 minutes on each side to extract all the clots and we need to be able to get a good extraction and get rid of as much clot as we possibly can to get a good outcome for the patient and we can only do that safely by cooling the patient. So once we have the clots extracted, we will warm the patient back up and then take them off the heart-lung machine.
 
Now, I know that sounds scary, but we do this every day and this is something that we do every day with not just operations with clots in the lungs, but also with the aortic operations as well, so this is something that we have particular expertise in and we can do very effectively with very low risk of complications and mortality.

Dr. Nick Smedira: That's fantastic. Ihab, I may have jumped the gun. We need a roadmap before we get in there to do the surgery or decide exactly what we're going to do. We start with maybe a VQ scan. When we talk to a patient, what are the tests that we would get that you think is necessary to really understand what we need to do?

Dr. Ihab Haddadin: The VQ scan is a good screening tool. One of the other things is we need to get some anatomical and physiological characterization of the disease. A CT scan is incredibly important because that allows us to figure out the extent of the disease and whether it is surgically accessible or not. An echo that's done by the Cardiology Department here just allows us to get an idea of how much strain the heart is under and then in certain select patients, not in all patients, where the CT scan may not necessarily give us all the information, we can perform a pulmonary angiogram, which is a catheter that goes into the pulmonary artery to inject some dye so you can get much more selective, much detailed anatomical pictures than with a CT scan that might help make the decision of what is the extent of the disease and what the best management strategy is.

Dr. Nick Smedira: That's great. And there's also, now, this new idea of blowing up a balloon in the pulmonary artery?

Dr. Ihab Haddadin: For certain patients we discuss this as a team to try to decide on the best approach for patients who may not be a surgical candidate and the balloon procedure would be an alternative. It's not quite as definitive. It's still in its infancy and it's not a one-stop shop where you can do the whole thing in one session, unfortunately. It does require multiple procedures that are about a month apart and for some patients, depending on the extent of the disease, it might be nine or 10 sessions, so this is a treatment with a balloon that can take over the course of close to a year. It is minimally invasive. It does require a small incision. Most of the time we're able to do it with minimal sedatives with the patient being awake, but we don't get rid of the scar. We just break it apart to help decrease the stress that the scar causes.

Dr. Nick Smedira: Fantastic. So, for a high-risk patient, this may be an option. So, Gus, if a patient is trying to figure out where to go to have this type of therapy, they've been told they might have this chronic thromboembolic disease, what should they look for to make a decision of where they have their care?

Dr. Gustavo Heresi: Yeah, this is a complex disease, as we are discussing and pulmonary hypertension, itself, it's a somewhat obscure condition that many physicians out there don't necessarily know a lot about and so this is why across the country there are many centers with certain expertise and experience in pulmonary hypertension, so certainly going to a specialty tertiary referral center is a good idea. And within pulmonary hypertension, really the field of CTEPH, of chronic thromboembolic disease is even more obscure, so there are really a handful of centers across the country that have really considerable experience and expertise with this. Of course, the Cleveland Clinic is one of them. And so we always tell people, talk to your doctors, make sure that your scans and your records are reviewed by a member of our team or your closest CTEPH specialty center and we're always happy to pick up the phone and receive CDs with pictures and review them and then talk to the patients or talk to their referring doctors.

Dr. Nick Smedira: Yeah, I think that's fantastic. You know, from my experience, it's such a complex disease and patients often have other things going on with their lungs, so to identify a center that has expertise and focuses on it, studies it, does research on it, and has integrated a multidisciplinary team to manage it, both in the diagnostic phase, the surgical intervention phase, and the post-phase is really critical to getting outstanding outcomes. So, I want to thank the panelists for their time and expertise and I really enjoyed the conversation.

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