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Interviewer: CTEPH is a hard to diagnose disease that about one in 1,000 people will develop. And because it is relatively rare, many patients and even doctors haven't heard of it. The symptoms can easily be confused with other diseases and what makes it even harder to detect is many people don't have any symptoms or the symptoms that they do have are mistaken for other illnesses. Dr. John Ryan is a cardiologist, one of the directors of the Chronic Thromboembolic Pulmonary Hypertension Program at 麻豆学生精品版. So it's so hard to diagnose, how do people even find out they have the disease.
Dr. Ryan: No, it's really tough. So it is, as you describe, Chronic Thromboembolic Pulmonary Hypertension so it is a cause of pulmonary hypertension. Pulmonary hypertension is elevated pressures in the lungs. So, oftentimes, if people are short of breath, if they're fatigue, if they have some leg swelling, they might get an ultrasound of their heart, and then that might show that they have high pressures in their lungs. And that's where the pulmonary hypertension is.
So then, when you look at the causes of pulmonary hypertension, a lot of pulmonary hypertension is caused by problems with the heart, a lot of it is caused by problems with the airways, such as emphysema, COPD. Some of it is caused by exposures to things such as methamphetamines. And rarely, within pulmonary hypertension, you can have it caused by chronic thromboembolic disease or chronic pulmonary embolisms.
The reason this is complicated is because some people won't know that they've had a previous pulmonary embolism. So if we want to talk about pulmonary embolisms, because I think that will be the first thing to talk about and then we can go into the chronic pulmonary embolism or chronic thromboembolic pulmonary hypertension, the abbreviation for this is CTEPH. So pulmonary emboli are common and pulmonary embolism is common, you have a clot in the lungs.
A lot of folks will have family members who have this, sometimes they themselves have had it. It can happen after a hip replacement, it can happen after a long-haul flight, which is something that you've come across before, and that's where you have a clot in your lungs. In 97% of people who have clots in their lungs, that's a classical way they head back to their regular everyday life.
In 3% of people, that's not the case. In 3% of people, they end up with this chronic pulmonary embolism, which can either be recurrent pulmonary embolisms, so they have one and they have another and another. Or it can be that the clot doesn't actually go away. Whereas, 97% of the time, the clot dissolves. In 3% of people who have pulmonary embolisms, the clot may not dissolve and therefore, they're left with this blockage in their pulmonary vasculature.
The analogy I kind of give among other analogies, which at least people humor me and tell that my analogies are very good, but the analogy that I give here is that a clot in your lung is like a car crash on the freeway. All the blood flow stops. And then, eventually, the crash gets cleared. In chronic thromboembolic pulmonary hypertension, that crash does not fully get cleared, you're instead of having four lanes, you're now down to one lane. So you end up with just this one trickle amount of blood going through because the clot has never gone away. Or the vessel has changed in response to that clot and folks get short of breath.
However, what makes it hard, and the thing you've asked me in the beginning about, you know, why is this so hard to pick up, what makes this hard is, first of all, it is in . . . of all the causes of pulmonary hypertension, it's least common. Fifty percent of people who have it don't know that they had a blot clot sometime in their life. They think this was never picked up or it was missed clinically.
But the importance of it is that it is a curable form of pulmonary hypertension so you can get treatment. And that's what our program specializes in here. We specialize in the management, the surgical and medical management of chronic thromboembolic pulmonary hypertension. We're the only program to do that in the [Inaudible 00:04:03], one of the few programs in the country. And you can, again, cure people of their disease by medically managing this or surgically managing this, most particularly. And that's a pretty positive thing.
Interviewer: I'm still confused as to how I would know that I had it. I guess the first thing is . . .
Dr. Ryan: The first thing you'd be short of breath. You'd be short of breath with fatigues.
Interviewer: Those symptoms, yeah.
Dr. Ryan: So you'd have symptoms similar to heart failure. The symptoms themselves are not that dissimilar to heart failure. And then at some [inaudible 00:04:29] along the line, someone will do an ultrasound of your heart and they would do an echocardiogram. And on the echocardiogram, you would see that the pressures in your lungs are high.
Interviewer: Okay.
Dr. Ryan: So then you have pulmonary hypertension. And then, as a workup for pulmonary hypertension, people look to see, did you ever have any blood clots, either by asking you specifically or there are some scans that you can do on the lungs to see if there are any signs of old blood clots.
Interviewer: So you can actually see those also?
Dr. Ryan: Yeah.
Interviewer: So it's a little bit of a detective game that you've got going on.
Dr. Ryan: It is, yeah. I mean when you have the diagnosis, first of all, when you have a diagnosis of shortness of breath or when you have a diagnosis of fatigue, that's obviously, as you alluded to, when the detective game starts. And then, once you get diagnosed with pulmonary hypertension, then you start looking into the different cause of pulmonary hypertension. So a lot of these are going on all at the same time.
When you have pulmonary hypertension, people start looking, it is caused by the heart, it is caused by the lungs, it is caused by the airways, it is caused by the vessels in the lungs, it is caused by clots. So all of these things are being looked at, at the same time. And then, if you find that there are clots in the lungs that have never gone away, in some regards it's almost kind of a eureka moment. You've found the cause of this pulmonary hypertension and now you can treat it.
Interviewer: So if you continue to have these symptoms and they're not just going away and you've received treatments for other forms of pulmonary hypertension, then that's when you consider, "I could be in this 3%."
Dr. Ryan: Yeah. Hopefully, before you get treatment of your pulmonary hypertension, people have figured out what type of pulmonary hypertension you have. The analogy that we use for this is pulmonary hypertension, in many regards, is similar to cancer. There are lots of different types of cancer. There are lots of different types of pulmonary hypertension. We don't treat all cancer types the same. Bowel cancer is very different to leukemia, say, and so on. So that's the same idea that we do with pulmonary hypertension. You find the cause of pulmonary hypertension, then you treat it.
Now that being said, of the people who have pulmonary hypertension, 1% of them will have chronic thromboembolic pulmonary hypertension so it's the least common form of pulmonary hypertension. That being said, it's the most curable form or pulmonary hypertension. So in some programs, in some practices, we'll guess and you can be . . . you're right, 90% of the time or 99% of the time, you might be right. But if you or me are that 1% who don't have these other forms of diseases, then you're getting the wrong treatment.
Interviewer: Got you. And you said surgery is the treatment?
Dr. Ryan: Yeah.
Interviewer: How effective is it?
Dr. Ryan: Surgery is the cure. Yeah, so it's really rewarding that you can go from being very debilitated, very short of breath, very fatigued, not able to do the things you want to do, and you undergo a major surgery. It's an open heart or open lung surgery where the clots in the lungs are removed and you're on bypass. Your chest is opened and the clots in the lungs removed and that changes the blood vessels are fixed. And then you come to the ICU afterward, you come to the floor after being in the ICU, you enter into a rehab program. But down the road, you'd then do very, very well and you go from being very, very sick to essentially having a normal life expectancy and doing very well.
Interviewer: Are there consequences for not treating?
Dr. Ryan: The disease progresses without treating so you got sicker. The strain on your heart gets worse and, ultimately, the heart can go into heart failure, which is what drives a lot of the symptoms. And then, of course, you can have future PEs as well, or future pulmonary embolisms on top of it. So you can have your disease and then have another pulmonary embolism. So those are kind of the consequences of not treating it.
Interviewer: Any final thoughts for somebody that's experiencing these symptoms and thinks they might have it?
Dr. Ryan: Yeah, so two things. One, surgery isn't actually for everyone. There are some people who, even in 2017, still have inoperable forms of chronic thromboembolic disease. That doesn't mean you're out of options, it just means surgery, right now, is not an option for you. But there are other medicines available that can help you do better. They're not a cure, but they can help you do better.
And so I think the main thing is to ask your doctors, "Do you need to look to see if this is blood clots?" Again, oftentimes, as I said, people don't look fresh. It's rare, it's uncommon, there are much more common causes and people don't know that they have blood clots. I think people also assume that they would know if they had blood clots, that you and I would know if we have blood clots. So I think it's worth asking your doctor, "Did you check for blood clots." And this is an easy, straightforward, low-cost test.
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