An overview of pulmonary hypertension, its signs, risks and treatment
Host Amber Smith: Upstate Medical University in Syracuse New York, invites you to be The Informed Patient, with the podcast that features experts from Central New York's only academic medical center. I'm your host, Amber Smith.
Pulmonary hypertension is high blood pressure in the arteries in your lungs. Today I'm talking about this condition with Dr. Krithika Ramachandran. She's an assistant professor of medicine at Upstate who specializes in critical care medicine and pulmonary diseases.
Welcome to "The Informed Patient," Dr. Ramachandran.
Krithika Ramachandran, MBBS: Thank you, Amber, and thank you for having me.
Host Amber Smith: Now, when we talk about the pulmonary arteries, we're talking about the arteries in the lungs and also those on the right side of the heart. Is that right?
Krithika Ramachandran, MBBS: That's right. So when we're talking about the pulmonary arterial system, we're talking about the blood vessels that come out of the right side of the heart and go into the lungs. So the flow of the blood, the way it flows, is the body sends back the blood, which is deoxygenated -- from which all the fresh oxygen has been used up -- back to the right side of the heart. And then the right side of the heart pumps this blood into the lungs, from where they can get the oxygen they need.
And then this oxygenated blood goes back to the left side of the heart, from where it's then sent to the rest of the body. It's kind of like this neat little circuit. And what happens in pulmonary hypertension is the pressures in the blood vessels of the lungs, to which this right side of the heart is pumping blood, the pressures there are higher than they should be, which poses a problem for the heart when it's trying to send this blood across.
Usually, the way I explain it to my patients is, I tell them to imagine, like, a balloon with a straw attached to the end of it. And if the diameter of the straw just keeps getting smaller and smaller, that balloon has to be squeezed harder and harder to send flow through the straw.
That's essentially what's happening with the right side of the heart in this situation.
Host Amber Smith: That's a good visualization. Well, let me ask you, does a person necessarily have to have high blood pressure throughout their body before developing pulmonary hypertension? Or can you have pulmonary hypertension and no problem with general high blood pressure?
Krithika Ramachandran, MBBS: Correct. That can happen. So, as we get older, since a substantial amount of the population develops high blood pressures, they can develop a form of pulmonary hypertension known as pulmonary venous hypertension. Now, that is not the classic pulmonary hypertension that we talk about when we say pulmonary hypertension.
The classical pulmonary hypertension, or what is now called Group 1 pulmonary hypertension, there, the pressures in the lungs are elevated separate from the pressures in the rest of the body. So to answer your question in a yes-or-no format, no, you do not have to have high blood pressure to have high blood pressure in the lungs.
Host Amber Smith: So what are the symptoms a person would experience if they have pulmonary hypertension?
Krithika Ramachandran, MBBS: That's a great question. and it's one of the things that makes it a harder condition to diagnose because the symptoms are very nonspecific. Typically, you know, you feel short of breath, you feel tired, you feel like you're just kind of running out of oomph.
It's only when the disease progresses, and you kind of get later in the stages of the disease, when the symptoms of right heart problems start to come into play. You can get swelling in your legs. You can get swelling in your belly. You may start to retain fluid everywhere. And then one of the most ominous signs is when you start to pass out.
So that indicates that you have really severe advanced disease and potentially could die any minute.
Host Amber Smith: Well, would people, if they go for, like, regular annual physicals, would their primary care doctor necessarily pick this up, or would someone develop a crisis and end up in the emergency room before they learn that what they've got is pulmonary hypertension?
Krithika Ramachandran, MBBS: No, not necessarily an emergency room situation, but typically, the time frame ends up being about six months to a year before we figure out that that's what's going on. Because they go to their primary care doctor, and the classical patient, again, they tend to be younger women or middle-aged women. Sometimes their symptoms get written off as, "Oh, you're anxious" and "Oh, you know, you're just tired from doing all of these things." Sometimes it gets treated as asthma, and it takes a while before you come to the realization that, "Hey, we've looked at all of these other common things, and we haven't really gotten to the root cause of this."
And that's when an echocardiogram gets done, and you come up with, "Oh, my gosh, this is what's been going on this entire time." It does take a while.
Host Amber Smith: An echocardiogram is what is done for diagnosis?
Krithika Ramachandran, MBBS: An echocardiogram is your screening test. That would be where you pick up clues to whether this is what's going on.
Let's say I go to my doctor, and I say, "Hey, you know, I haven't really been feeling very well. I'm short of breath. I could, you know, run four miles before, and now, I'm just really having a hard time walking a mile."
And I get asked all these questions. I'm not wheezing, I'm not coughing, I'm just nonspecifically tired. They look to make sure I'm not anemic or something, and then they're like, "You know what? Let's just get an echocardiogram. That might show."
So an echocardiogram can actually indirectly measure pressures on the right side of the heart, and that will give you this clue to, "Hey, maybe the pressures in your lungs are high."
And so that's what we call a screening. test. Now, about 10% to 20% of the time, the echocardiogram can either underestimate or overestimate the pressures, which is why you always have to confirm the diagnosis by what's called a right heart catheterization, where you actually go into these blood vessels, into the lungs and the heart with a little catheter (hollow tube), which has a balloon at the end of it, and that measures the pressures all the way and tells you for sure that, "Hey, yes, this pressure is up," or "No, the echocardiogram was a red herring."
Host Amber Smith: Why is pulmonary hypertension dangerous?
Krithika Ramachandran, MBBS: Pulmonary hypertension is dangerous because it causes you to have congestive heart failure, so the end result of untreated pulmonary hypertension, just like the end result of untreated systemic hypertension, is that you end up with these big dilated heart chambers, which are unable to push blood forward through the lungs anymore.
So now you have lack of blood flow forward, blood flow to the rest of the body, because the left side has inadequate blood to send forward. So you have low oxygen everywhere. Your brain is not getting perfused (properly supplied with blood), your kidneys are not getting perfused. So you can actually end up with congestive heart failure, and terminally with cardiogenic shock, which is when, essentially, all your organs are shutting down.
Host Amber Smith: I've also heard some people might have a problem with anemia if they have pulmonary hypertension?
Krithika Ramachandran, MBBS: Anemia can worsen pulmonary hypertension because you have low blood counts, right? And there are fewer red blood cells to carry oxygen everywhere, but pulmonary hypertension and congestive heart failure, because of the chronic inflammation they set up, can cause anemia also.
So, it's almost like this vicious cycle. In recent years, they've been screening patients for anemia and iron deficiency, and it's been shown that patients who are iron deficient actually have a higher chance of having bad outcomes from pulmonary hypertension or even from regular congestive heart failure.
So, we look to see if they are iron deficient, and we can actually supplement them, which improves their functional status, and it also helps improve their disease and their overall mortality.
Host Amber Smith: What about blood clots? Are they a higher risk for someone with pulmonary hypertension?
Krithika Ramachandran, MBBS: This is a little bit of a controversial field. So, earlier the thought process was that because the blood flow through the pulmonary blood vessels is slow in pulmonary hypertension, because the heart's not doing a great job of pumping blood, you can form blood clots in the lungs, and so they used to place all of these patients with pulmonary hypertension on blood thinners to make sure that that doesn't happen
More recently it's been shown that this actually doesn't affect outcomes, so we've moved away from putting all patients with pulmonary hypertension on blood thinners, but a patient with pulmonary hypertension who develops blood clots in the lungs on top of already having pulmonary hypertension, they can really get into very bad trouble, because all of a sudden now, this heart, which has already been struggling with pumping blood through these small, narrow tubes, has this big blockage on top of it, which has completely cut off areas that it can pump blood through. So this back pressure can actually make it balloon out, dilate and sometimes cause cardiac arrest.
Host Amber Smith: I wanted to ask you something else about the echocardiogram.
Krithika Ramachandran, MBBS: Uh-huh.
Host Amber Smith: Are you able to see the vessels, and do they look smaller on the echocardiogram?
Krithika Ramachandran, MBBS: No. So the way they kind of indirectly measure it is, where the right heart pumps blood, there's this little valve in between the two chambers of the right side of the heart. And when the heart pumps blood, it's supposed to flow forward into the lung arteries. Instead, because the heart's not pumping efficiently, some of the blood flows backwards into the smaller chamber of the right heart, and so they can use this measurement of the velocity of this backward flow to determine how much pressure is being faced by the right heart because of these smaller blood vessels. So you can actually sometimes kind of see when the blood vessel looks larger, because what happens is not that the pulmonary arteries get smaller and smaller in their size. It is that the internal diameter, or the hole through which the blood flows, that starts to shrink because the vessels start to scar down. So while the artery itself might look bigger, when you, say, do a CAT scan or an MRI or something, the actual size of the vessel through which the blood is flowing, the surface area of that decreases, which is where you have the trouble.
Host Amber Smith: What sorts of arrhythmias may develop because of pulmonary hypertension?
Krithika Ramachandran, MBBS: So the most common arrhythmias that you'd see are what we call supraventricular arrhythmias. So they originate from the atria. So you can have atrial tachycardias, like atrial flutter, atrial fibrillation, multifocal atrial tachycardia.
The terminal rhythm for everyone, of course, is a ventricular tachycardia, but usually, the patient's dying at that point. But the onset of arrhythmias, again, is a bad prognostic sign for patients with pulmonary hypertension.
Host Amber Smith: This is Upstate's "The Informed Patient" podcast. I'm your host, Amber Smith, talking with Dr. Krithika Ramachandran. She's an assistant professor of medicine at Upstate specializing in critical care medicine and pulmonary diseases, and we're talking about pulmonary hypertension, Now, how does pulmonary hypertension develop? Does it take a lot of time to develop or does it appear overnight?
Krithika Ramachandran, MBBS: No, it certainly doesn't appear overnight.
It's a slower process than most, I would say. It typically happens over the course of at least a year or two, and the patients I would say who are at higher risk for these kind of explosive developments of pulmonary hypertension are patients with scleroderma.
They can sometimes have very rapid onset of very severe disease, but in most patients, it's the slow, sort of, "Oh, I don't feel good." "Oh, I'm just feeling worse." And then over the course of several months to a couple of years is when they truly start to feel the disease and when you'll truly diagnose it.
Host Amber Smith: So other than scleroderma, are there other conditions that would increase a person's risk of pulmonary hypertension?
Krithika Ramachandran, MBBS: There's always this category of idiopathic pulmonary hypertension, which is we don't know why they have it, but they have it. We can't find any risk factors for it.
Then there are patients who have inherited pulmonary hypertension. There are some genetic factors which can predispose you to get pulmonary hypertension. Other than that, the most common causes would be the use of stimulant drugs like methamphetamines. Back in the '60s, '70s, there was an epidemic of pulmonary hypertension caused by diet pills, like pills that they used to take to lose weight, phentermine, fenfluramine. So they were all taken off the market at that time.
Cirrhosis is a big risk factor. HIV. And then, you can have some blood conditions, like myelofibrosis, thrombocytosis.
There's really a very long list of autoimmune problems like scleroderma; that's a big one ... lupus, rheumatoid arthritis. These are all risk factors for having pulmonary hypertension.
Host Amber Smith: What happens to someone if this condition develops and it goes untreated?
Krithika Ramachandran, MBBS: Like we talked about before, they would develop right-sided congestive heart failure, so they'd start to see swelling.
They'd start to notice severe shortness of breath, weight gain, arrhythmias, and, eventually, it progresses to death.
Host Amber Smith: So it is something where if it gets diagnosed, there are some things that you can do to try to manage it?
Krithika Ramachandran, MBBS: Absolutely. So the landscape for treating this disease has changed dramatically In the last, I would say, 10 to 15 years. Earlier, and by earlier I mean maybe back in the early '90s, late '90s, it was almost like a death sentence. If you were diagnosed with it, more than 50% of patients were dead before three years were out. But now, it can be one of those diseases that you kind of die with instead of die from.
So there are multiple medicines now which help dilate these blood vessels in the lungs and bring the pressure down so that the right side of the heart feels less stress while pumping blood through the lungs. So definitely there are multiple, multiple treatments now, as compared to a few years ago, which has changed the outlook of the disease.
Host Amber Smith: Does treatment differ for someone whose pulmonary hypertension is related to lung disease, as opposed to someone with a mitral valve or aortic valve disease?
Krithika Ramachandran, MBBS: Yes, yes. So, the way we look at it now, it's divided into five different groups. So, Group 1 is the one which we call kind of classic pulmonary hypertension, where there is isolated increase in the blood pressure in the lungs alone. And most of the medicines that we have are tailored to this specific issue. So they dilate the vessels, bring the pressure down, and so we try really hard to find those patients, so we can treat them earlier.
Now, things like mitral valve disease, aortic valve disease, these are all patients that we categorize as Group 2 pulmonary hypertension, which is also known as pulmonary venous hypertension. So this is trouble on the right side due to the left side of the heart, and these patients actually tend to comprise about 60% to 70% of the total pulmonary hypertension patient load.
They typically tend to be older, and they have what we used to call secondary pulmonary hypertension, which means that the trouble is not originating in the blood vessels of the lungs. It's originating somewhere else, and it's affecting the pressure in the lung. So in these patients, we try to treat the primary problem, and not the pressures directly. Because treating the pressure directly can sometimes have an adverse outcome. So let's say they have mitral valve regurgitation, then you would try to fix the mitral valve. Or if they have tight aortic stenosis, you would try to have aortic valve replacement. So, those patients, again, you would try to treat the contributing factors instead of trying to treat the pressures in the lungs.
Similarly, for patients who have lung problems, they have pulmonary hypertension from primary lung disease, so let's say it's from sleep apnea. So, then you try to fix it with CPAP (an assisted breathing device), or if it's from COPD (chronic obstructive pulmonary disease), then you try to treat their COPD, make sure their oxygen levels are adequate.
You try to treat the underlying risk factor.
I'm just going to add one more thing here because I think this is one caveat that even when I do, like, a lecture on this topic, I want to make sure that people come away with this.
There is one form of pulmonary hypertension which is actually curable, and I try to make sure that everyone knows that. So when you develop pulmonary hypertension from having chronic blood clots in the lungs. There are people who, over a course of years, have had tiny clots in their lungs, which have gone undiagnosed, and over time, they've just kind of crowded the circulation, made the vessels narrower and narrower.
Those patients can actually have surgery to get these clots removed, and then they're essentially cured of the disease. So that's, again, a category of patients we try to find, because for them it can be life changing.
Host Amber Smith: Does the presence of pulmonary hypertension complicate other medical issues that may come up?
Krithika Ramachandran, MBBS: Yeah, I mean, having pulmonary hypertension makes a mess of everything, really, because there's all these medicine interactions. So, let's say you have a cold, for example, and you want to use a decongestant, right? It's hard because it's going to increase the pressure in your lungs, and it may make it harder for your heart to work properly.
Let's talk about atrial fibrillation. Most of the drugs used to treat atrial fibrillation try to slow the heart down, which can actually end up having a detrimental effect on the right heart function because it depresses the ability of the heart to contract properly. So, for sure, having pulmonary hypertension complicates your life in multiple ways, and not just because you have pulmonary hypertension.
Host Amber Smith: If someone has been diagnosed with pulmonary hypertension do they need to be seeing a pulmonologist or a cardiologist regularly, or is this ...
Krithika Ramachandran, MBBS: Absolutely.
Host Amber Smith: Really. OK, so ...
Krithika Ramachandran, MBBS: Absolutely. Every three to six months is the recommended follow-up with an expert on the disease. And so, 100%, if there's someone who's got a diagnosis of pulmonary hypertension, they should be seeing either their pulmonologist or their cardiologist or whoever is comfortable managing their disease.
Host Amber Smith: Are there some general red flags that would tell a person with pulmonary hypertension that they need to seek emergency care related to their pulmonary hypertension?
Krithika Ramachandran, MBBS: Yes. So I would say if they are short of breath all of a sudden, more than normal; if they're starting to retain fluid, more than normal; if they pass out, that's a huge one. Palpitations. These would be the four big ones.
Host Amber Smith: Well, let me ask you about the general outlook for someone, because you alluded to this, that this diagnosis is life changing. So let's talk about the ways. You mentioned, you know, a cold, and the choice of over-the-counter medications, or, I guess, even herbal products would be a concern for this person, right?
Krithika Ramachandran, MBBS: Absolutely. Absolutely. So any and everything that they ingest needs to be double-checked to make sure it's not going to affect them adversely.
The other thing that we always stress: Patients with pulmonary hypertension should never get pregnant, because their cardiovascular system cannot handle the stress of pregnancy, and it can kill them. So that's another big no-no. The other thing is a lot of the drugs that we use to treat pulmonary hypertension can cause fetal defects. So that's another reason that we advise against pregnancy and to make sure they use contraception or have some method of contraception when they have this disease.
Host Amber Smith: So getting back to the over-the-counter medicines, can, like, Tylenol and ibuprofen and those types of things, can those cause problems?
Krithika Ramachandran, MBBS: Depends on the severity of the disease. So if this is someone with advanced heart failure, for example, Tylenol can affect their liver function. If they have congestive liver from their heart failure, Ibuprofen can definitely affect their kidney function, if, again, their kidneys are in a delicate balance.
And these are people taking water pills, trying to maintain their salt and fluid balance. It's a very precarious line they toe when they're trying to keep themselves healthy. So, for sure, anything and everything needs to be evaluated as a risk-benefit ratio before taking it.
Host Amber Smith: What about vaccines like the flu shot or the pneumonia shot as you get older?
Krithika Ramachandran, MBBS: They're all recommended. They should be getting them, because any sort of infection, again, is an extra stress on the system. So, certainly, vaccines are indicated and are recommended for these patients, including the COVID vaccine.
Host Amber Smith: Is their immune system compromised, though? Are they considered to have a compromised immune system?
Krithika Ramachandran, MBBS: No, they're not.
Host Amber Smith: Now what about exercising with intensity? Can someone with pulmonary hypertension train for a marathon? Can they do things like mow a large lawn? How much are they restricted?
Krithika Ramachandran, MBBS: We always recommend beginning exercise under supervision for these patients.
Lifting weights for them is usually a big no-no. Anything heavier than 10 pounds is not recommended, because, again, that is increased stress that the heart has to push blood against. In terms of cardiovascular exercise, there are no clear boundaries laid out that way, you know; it's conditioning, really.
So if they're slowly building up their exercise capacity, It's recommended that they do that. But if a patient comes to see me today, I wouldn't tell them, hey, go out and run a mile every day, because that's not how it would work. It would be a gradual buildup of exercise capacity with monitoring, making sure their oxygen levels are OK, their heart's not racing, they're not having arrhythmias.
So it's a whole bunch of multiple things.
Host Amber Smith: Would they be permitted to travel on an airplane or to vacation at somewhere in the mountains?
Krithika Ramachandran, MBBS: They would probably need supplemental oxygen if they did that. So at (high) altitude, because the concentration of oxygen is lower, hypoxia, or the lack of oxygen itself, can make the pressures in the lungs go up a little bit.
And actually we are able to do that at Upstate. It's called a hypoxia altitude simulation test, where they can simulate the altitude and see if your oxygen level drops. And if it does, we can prescribe supplemental oxygen to be used at the time.
Host Amber Smith: Well, I know this may apply differently to different patients, but in general, how should their diet be? Is there anything, any food, that they should avoid or any food that they should add?
Krithika Ramachandran, MBBS: Salt. Yes. So just like any patients with congestive heart failure, low-salt diet, less than 3 grams of salt a day, 2 to 3 grams, depending on the size of the person. Again, this kind of depends on if the patient is in congestive heart failure or not. We may ask them to restrict fluid intake to less than 2 liters a day, but otherwise, a healthy diet should be good.
Host Amber Smith: Well, it's encouraging, as you were explaining, the options for treatment are so much greater now today than they were 50 years ago or 20 years ago.
Krithika Ramachandran, MBBS: Absolutely, Absolutely. It's a whole different landscape.
Host Amber Smith: Well, that's really good to know and I really appreciate you making time for this interview, Dr. Ramachandran.
Krithika Ramachandran, MBBS: Oh, thank you very much for having me. It's my pleasure.
Host Amber Smith: My guest has been Dr. Krithika Ramachandran.. She's an assistant professor of medicine at Upstate, specializing in critical care medicine and pulmonary diseases. "The Informed Patient" is a podcast covering health, science, and medicine, brought to you by Upstate Medical University in Syracuse, New York, and produced by Jim Howe.
Find our archive of previous episodes at upstate.edu/informed. This is your host, Amber Smith, thanking you for listening.