J. Thomas Heywood, MD, FACC, identifies how to diagnose pulmonary hypertension, and reviews different types and how they are diagnosed. Dr. Heywood recalls what therapies exist to treat pulmonary hypertension and explains when to refer patients for more advanced testing and treatment.
Back to Symposium Page »
Well, uh, thank you for joining us today for our fifth annual course in the advances in the diagnosis and management of pulmonary hypertension, the pandemic persists. So we're on zoom again. The zoom this year we'll see about next year, although there are there advantages to being on Zoom. Uh, and I think we have more patient more people signed up this year than we ever have had. So in some ways, it's easier, so I'm sure we'll always have. This is an option, but it is nice to meet people live. Um, speaking of the pandemic, I was speaking to a doctor yesterday who is one of our sister hospitals in Chula Vista, who's a pulmonary pulmonologist. He was talking to me about one of my patients that got admitted to the hospital, and he said he had seen his 1000th covid patient that week. So this doctor had seen 1000 patients with covid 19, and he did not get sick, unlike some of his colleagues. So that's a real testament to being well, so, uh, again, salute to our you health care workers who have had to do such good work in this, uh, epidemic that will always remember. That's going to be a defining year for us. So thanks for taking time away from your clinical responsibilities and your families to join us for a few hours. So the conference agenda materials can be found on the seamy conference app. The APP is available free of charge you can download. It is also recorded and will be available for approximately one month after the event. Um, just to remind everybody all participants will be muted during the lectures. Be sure to close other Web pages so you don't slow down your Internet connection. Um, submit any questions on the asked question button on the live feed, and they will be shared with the factory faculty. If the live feed freezes, refresh your browser and hit the play button to resume. Please don't forget to exhibit to visit the virtual exhibits during the break time. Uh, these conference that would not be possible without the generous support of the vendors and allows us to reduce the price for the conferences. Use the Chat board to network with your colleagues, tell people who you are and where you're from, and to get your CMI certificates, you have to complete the online course evaluation and claim your credit. So thanks very much for attending. We have a good, uh, program today. I've changed it up somewhat. I've asked people to make this, um, a more case based approach. I think that especially with the video, I think that will make it more interesting and keep it moving. So without further ado, I think I'll kick it off. Scott, if you can send me my slides. Thank you. See how we're over here. Let's see. Ah Scott or there we go. Okay. He was on my disclosures. So what? Let's start at the beginning. What is pulmonary hypertension? A normal pressure is about 20/10 20 systolic, 10 diastolic. Um, when you get an echo and the pressures are over about 50 uh, in pulmonary hypertension, the pressures are typically about 40 but usually much higher. The mean p a pressure, uh, over 20 represents pulmonary hypertension. Echo is really a good screening tool, but you may need to measure pressures in the cath lab. That really depends on the clinical circumstance. If, for example, you know that the patient has left heart disease and the pressures are elevated, then often you can just Deri's them and see the pressures go down in the right heart. Cath isn't necessary. But if you suspect suspect pulmonary arterial hypertension, then a right heart cat is necessary. So what are we going to learn from this course? One how to diagnose pulmonary hypertension? What are the different types of pulmonary hypertension and how are they? And how are they diagnosed? What therapies exist to treat pulmonary hypertension and then went to refer patients for more advanced testing and treatment? Pulmonary hypertension is really a continuum. Like many diseases, uh, in many cases it's fairly straightforward. And, uh, the treatment is obvious. For example, if you have someone with an EF of 20% and there are pressures are 50 it's very likely that they have pulmonary venous hypertension from a high wedge pressure, and we would just directs them. And that's a fairly straightforward decision, however, when the when the cause of pulmonary hypertension is not known when there's RV dysfunction, that isn't well explained than I think you need more help. And even though we have a large pulmonary hypertension clinic here at scripts, uh, I reach out for help in difficult cases to colleagues at the university and elsewhere because patients need very specialized treatments as you'll come to find out. And so this is really a continuum. But again, if you have patients that are difficult, we want to serve as a resource for you to help you with. Even if you just have questions about pulmonary hypertension, we're happy to help in that regard. So this is pulmonary hypertension Boot camp. This is what you need to know for sure. When you leave this course that there's two types pulmonary venus or pre capillary or post capillary pulmonary hypertension, this is the most common type. 95% of the patients that you will see have this type. If you just said every one of your patients that has formally hypertension has pulmonary venus or left heart disease, pulmonary hypertension, you'd be right 95% of the time. Because left heart problems are so common. Heart failure is common. Millions of people have heart failure. Millions of people have mitral regurgitation or aortic stenosis or atrial stiffness problems usually, and this is something you should look at the echo when you see high a P a pressures. Look at the echo. Next, An enlarged left atrium usually means that the wedge pressure is high, and that's a sign of mhm left heart disease. The less common type is pulmonary arterial hypertension. Less common about 5% mostly women, uh, comes from many, many, many diseases that cause pulmonary vascular disease. This is a disease of the lungs, and the one of the defining features of this on the echo is that they have a normal left atrial size. So by once you see that there are hypia pressures, look at the atrial size and that can give you a tip about whether you're dealing with pulmonary venous hypertension or pulmonary arterial hypertension. Even though this is the less common type and in many ways the more deadly type. It's very treatable. And that's again an important lesson from this. Uh, talk, of course, today is that making the diagnosis is critical because whatever type of pulmonary hypertension you have, for the most part, it's treatable in some way or another, and those those are the things are going to learn today. So let's talk. Start with the case. This is a 60 year old woman that comes to your practice for a checkup. She has a busy, active lifestyle but has a family history of of heart disease. And you know, when you turn 60 you start during that clock ticking. And you think, Well, maybe I should make sure that I don't have the same problems that my parents had as they got older. The exam is not really very remarkable, but you do an E k G and there's a right axis, which is unusual and you're not sure about that. So you go upstairs to the cardiologist upstairs and talk to her. Show her the echo and she said, Pretty abnormal. E K g. I suggest we get an echo, you get an echo, and there's a moderately enlarged RV. The pressures are about 45 which you know are abnormal because you've been to this course. So you officially refer this patient to the to your friend cardiologist. Upstairs she does the right hard calf, right? Atrial pressures are 10. The pressure is 50/12. The wedge pressures 10 cardiac outputs. Hi, Eight leaders normal is about 4 to 5 liters a minute, and the pulmonary artery saturation is 87% and the saturation in the superior vena cava 72%. So somewhere blood from the left side of the heart, which has high oxygen, is being added to the right side of the heart. So there's a problem. There's a shunt someplace, so she orders a CT scan that shows an abnormal connection of the pulmonary vein to the superior vena cava. So this is a one of the unusual causes of pulmonary hypertension. But it's pulmonary arterial hypertension because of adult congenital heart disease, which you'll hear a lot more about from length today. But we know this is pulmonary arterial hypertension, because the wedges high and the PVR is high as well, which we'll talk about. So this is what the CT scan looks like. There's a pulmonary vein and the superior vena cava are connected, and that should not be the case. Now, this is slightly This is not my patient, but you may have heard this in the news. This is Amy Grant, uh, famous country Western singer who just had open heart surgery two to correct this rare congenital heart condition. Partial anomalous pulmonary venous return. So normally, the pulmonary veins. There's four of them. They should connect to the left atrium, but sometimes one or one or two of them connect to the superior vena cava. Another structure. And this is what this person. This is probably the most common cause of mist adult congenital heart disease. And it's not common, but it's common enough that I've seen it four or five times, and even some of my friends have it. So this can happen. And this was picked up because the K G was normal. Somebody got an echo because of the abnormally kg. The RV was abnormal, and they just didn't write it off and say, Well, the RV isn't that important anyway. Even though this patient didn't have many symptoms, they can have symptoms, and if left untreated, it can cause more severe problems so important to pick this up. But these are the things you learn from the course. So here's the second patient. This is an 85 year old woman presents to your practice with shortness of breath worsening over the last year. So she's she's more sick. She's been active in the past, but now limiting her exercise and not sure if it's because of her age or because of mhm. Something else going on. She's thin. She has a history of atrial fibrillation and hypertension on exam. She has some edema. Her neck veins were elevated a bit. Um, she doesn't have rules. You order an echo because of the high neck veins. Reef is normal, but pressure is 65. So she really has significantly elevated pH pressures and this history of atrial fibrillation. She gets the right hard cat, too, because she's pretty symptomatic. And because of the pulmonary hypertension on Kath, rre pressures 10. Her, um, wedge pressure was 75/24. Her mean pressures 41 her wedges 19. Her cardiac output is terrible. Remember, in the first case, the cardiac output was high because there was a shunt from the left side to the rest to the right side. So blood was recirculating through the heart. Um, and but in this case, the heart pumping only half as much blood as it should. And the pulmonary vascular resistance, which is the difference between the mean pressure and the wedge pressure is almost his 10 woods units and normal is about 1 to 2. So this person is very sick now. This is one of my patients. And, uh, this is actually a wife of a man that I've been taking care of for about 12 years. He said, You know, my wife's getting short of breath. Can you see her, too? And so I said, Of course I'll see her. And, uh, you know, I was surprised at how sick she was. She had this history of of a fib and had been active most of her life, and it looks really much younger than her 85 years, but was really quite sick. And her exercise was being extremely limited. And I can see I can see why after the heart, Cath, because her cardiac output was only half normal and pressures were extremely high. Now, this is a more complicated case. This is certainly a case that, uh, you know, uh, unique. I think consultation with a pulmonary hypertension program would help because she has a mixed type of pulmonary hypertension. She has features of both pulmonary venus and pulmonary arterial hypertension. She has elements of pulmonary venous hypertension, the common type, and pulmonary arterial hypertension. The uncommon type. She's pulmonary Venus because they're wedges high, but she's pulmonary arterial because her, um, her Woods units are so elevated. This combination is not seen often, but it certainly happens. It's part of my mantra that bad things happen to good people. She's very nice and doesn't deserve this terrible, complicated problem to have. And so we began treating her. So one of the ways that we treated her was to implant this monitor and you'll hear more about this. This is called the Cardio Memes. MM stands for mechanical electrical monitoring system. It's really kind of a very elegant device you can. Here's our If you took a cross section of the device, there's loops of wire and there are these two plates that form a capacitor here, and the wall of this device is flexible. So when you implant this in the pulmonary artery, this wall moves in and out as the pressure wave goes. So when they insist early, the this moves in and diastolic moves out. And this affects the resonance frequency which you're seeing here through this complicated equation here. So basically how this works and this is really all this is is a loop of wire this capacitor in This device that sits in the pulmonary artery sits in a big pulmonary artery. It only takes up about 10% of the space. When you send a radio wave against this device, it powers it up. It induces a current in these wires, and this creates what's called a resonant circuit, and it sends a radio wave. That's an effort that's a very faint FM signal back to the antenna of the device that sent the radio wave to this. So we send a radio wave, a pulse of energy out and a faint radio wave comes back, and that tells us what the pressure is. So we get this way for back on the Internet, so the patient lies on a pillow. The pillow sends this pulse of energy out, and then this radio wave comes back and the pillow picks it up and puts it on the Internet. So when we put this in, her pressures were pretty high. They were over 60 and her diastolic was high as well. So first we diarist her to get her wedge pressure down. And then we put her on a pulmonary arterial, uh, hypertension drug Vaso dilator called PDE five inhibitor, or uh, to data fill, which you know is Cialis and that immediately lowered her pressures and by lowering her pressures, we increased her cardiac output, and she called me the next day and said how much better she felt like she felt better from one day to the next when we added that drug, and then this was back. This was two years, almost two years ago now, and we've been monitoring her since. She has a little elevations occasionally, but her pressures have come down from the sixties down into the forties, where they are now, and she's feeling much, much better. So the way we managed her was first reduced her diastolic pressure with diuretics. And then we lowered her systolic pressure with the PDE five inhibitor. She has more energy. She's much less short of breath. She even has a personal trainer Now. Her husband's a little bit jealous about how well she's doing. He has more severe problems, and she does and can be as quite as successful with him as we are with her. So these are two women with pulmonary hypertension. The first step in both of their cases was to recognize either that the pressures are higher, that the RV is enlarged, and that's not normal. I want to emphasize that the echo is more than just the L V E f. And the question is, Why are the pressure's high? And once you have a diagnosis, if you can make a diagnosis and usually can, is there left heart disease is the right heart diseases their pulmonary venus or pulmonary arterial hypertension, then lowering the pressures when possible? And the bottom line is like most things in medicine, the right therapy for the right person. So let's step back a bit. How common is pulmonary hypertension? Well, unfortunately, we really don't know because patients often are very, very subtle symptoms. And they're not sent to clinicians. Certainly early enough in Australia, where they had fairly a captive population on the west coast of Australia, they did a prevalence and mortality trial because they're the main providers of service in this area. Most people had pulmonary hypertension from left heart disease. You can see here is pulmonary arterial hypertension, idiopathic scleroderma, congenital heart disease from blood clots. So there was there was certainly a scattering of of ph, but 90 to 95% of the patients, uh, had left heart disease. This is a population of 165,000. So they had 350 per 100,000, so approximately 500 patients in their collective area had pulmonary hypertension. If you think of San Diego with our three million people, that would mean about 10,000 people in San Diego County and probably greatly underestimates the true prevalence. Our pulmonary hypertension clinic, per se has about 70 people, but we treat several 100 people with pulmonary venous hypertension. And so, uh, we have Well, we have several 1000 just scripts. So I think it's the numbers are much more. And as your pressures go up, your survival goes down. This is especially true with left heart disease, but it's it's true with pulmonary arterial hypertension as well, so that you know a at five years, about half the people are dead. I tell people not to look at these data because I think they're old and away. And if you aggressively treat the disease, I think you you improve outcomes. This is people with half caf. Pulmonary hypertension is very common. You can see that 83% of the people with half CAF at, uh HIPPA pressures, and there's at least 3 to 4 million people with half PEF in the United States. The important thing is, if your pressure was over, 48 are then at three years, your survival is only about 50%. So it's a really serious disease. And, uh, has a high mortality probably higher than heart failure with low Yet because we have fewer ways of treating the disease. Uh, it's, uh, it's a big problem, so, but there are glimmers of hope. It will continue to talk about those in the course, and I think there's a reason to be very hopeful. So in San Diego, 10,000 with pulmonary hypertension, probably much, much more, 95% have pulmonary hypertension due to left heart disease. This is about 9000 to 9500 individuals. We have certainly thousands in the script system. About 5 to 10% have formally hypertension due to pulmonary vascular disease. This is 500 to 1000 individuals. Again, I think this probably underestimates the true prevalence. So in the next four hours again, we're going to talk about how to diagnose pulmonary hypertension. How you use the echo when the echo is not enough when you have to do a right heart, Cath. And this has to be done very carefully by somebody who's really looking at pulmonary hypertension and knows how to do a vassal dilator study that knows how to do a good shunt run. Who knows when a shunt runner might not be accurate? Okay, how is pulmonary hypertension managed and went to refer. So I want to thank you again for spending your Saturday morning with us. Uh, I think you and your patients will be glad you did. Uh, this is my cell phone number. I I actually enjoy getting calls from clinicians when they have, uh, issues with patients. Often we can just address them over the phone. And I can give you some guidance about what to do next, or if you know what's going on with the patient. What? Who you might send them to and give you some help with referrals and stuff. I can cut some red tape for you and make sure your patients I get what you need. So I think we had one question. Can we go back to slide 14 for a second, but I think I can discuss this. So on slide 14, I showed a graph of the P A pressures chronically in the patient, and the pressures are the So the lines are the top line was the pulmonary systolic blood pressure. The middle line is the mean pressure. And the bottom line, uh, is the p a diastolic pressure. So we get to see what the systolic mean and diastolic pressures are. And and for most people, we use the bottom number, the p A diastolic pressure as our guide of therapy to know when their volume status is normalized. But in people with pulmonary hypertension, we look at the mean pressure and the top number to know when we've gotten the P A pressure is normal. I'll be showing more of these types slides during the conference, and, uh, I will go over them a little bit more slowly and make sure that everybody has a good idea about that. Okay,
