Dr. Laith Alshawabkeh defines pulmonary hypertension as it relates to adult congenital heart disease and discusses prevalence and clinical approaches to patients using case examples.
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Alright, We're heading into the homestretch now. Uh, some ways we saved some of the most interesting things for the last Laith. Another good friend from UCSD again on my contact list. We probably talk once a month. I was very happy to hear from him that one of my patients with congenital heart disease was transplanted this week at UCSD and is doing really well. So, um, thanks for that lathe is this really specialist world of adult congenital heart disease, which people think, Well, there can't be much of that, but he'll tell us there's about a million patients that have this, and there are still a number of patients that have and misdiagnosis is obviously very complex disease. But again, extremely rewarding. He was trained in Boston, and then we're lucky enough to have him come out here. So he's a great resource for Southern California, and it's good that you get to know him. And as I said, he's very approachable and you give him a call anytime. And he can really help you with your congenital heart patient problems. So take it away. Thank you very much, Doctor. Here. Word for the invite. Very kind words and thank you for the panel. So let's get to it. Um, so today I will talk to you about congenital heart disease and pulmonary hypertension. Um, so I thought a lot about how to structure this talk. But I will focus a little bit more on the masqueraders or people who seem to have only hypertension and end up with the diagnosis of congenital heart disease. Because I do think that's more relevant. Issue in clinical practice. Mhm. Yeah. So really, um, congestive heart disease and adults, it's It's a rapidly increasing cohort. Uh, So in just one decade between the year 2000, the year 2010, there's been a 57% increase in the number of adults, or the prevalence of adults living with congenital heart disease in the U. S. Contrast that to only 11% increase in kids. Uh, so that's reflective of not just increasing the, um in the size of the population, but also the general population of the U. S. But also increase in Survivor hood of these patients, uh, to adulthood to give you a little bit of context. Um, if you are a cardiologist, if you're an internist. If you're a generalist, really any specialty you know atrial fibrillation because you deal with it a lot. And if you look at the aggregate data, there's approximately 1% of patients in the United States with prevalent chronic atrial fibrillation. So contrast that also a 1% prevalence of heart disease in the population. And currently, if you count just the adults living with congenital heart disease, there are approximately, actually two million in 2021 that's by far surpasses the number of kids living with congenital heart disease. So you will see these patients. If you haven't and you have, I'm sure you will see them more. I'm gonna start with the definition of pH and congenital heart disease. And you know, this is really confusing. And I do believe that original heart disease contributes to the confusion of general practitioners when they're approaching pulmonary hypertension, because at the end of the day they are grouped into pretty much all the categories of pulmonary hypertension. Some group one group two with left sided heart disease or post pulmonary hypertension, post capillary hypertension. You know, they don't listen here in Group three, but many patients with heart disease do have intrinsic colonial disease from the childhood operations and the restrictive chest wall. Um, some of them have from metabolic disease, and many of them are multifactorial. Indeed, they recently added a group, which is called segmental plumbing hypertension that is, rather, uh, isolated in those with congenital heart disease. So as you can see, they are represented in all these categories. So the way I would approach it is more patient, focused and path of physiology focused for every patient. If you look at the definition, which was more recently revised to 20 millimeters mercury, a pulmonary artery pressure mean pulmonary pressure of 20 or 25 different matter I want to see it defines following her attention and the problem with heart disease that this is a very broad diagnosis, which probably is appropriate so that you would capture as many as you want for further testing. Um, but probably incorrectly diagnosed as pH in patients with normal pulmonary vascular resistance, particularly those with left to right chanting. And we'll give you examples of that but also does not capture elevated pulmonary vascular resistance in those would not impulsive type pulmonary circulation or single ventricle retaliated to a Fontane. Uh, circulation. The prevalence of forming hypertension in adults with congenital heart disease is high. In one estimate is 18 fold higher, uh, inclusion of heart disease than in the general population. So it is a big problem that we deal with. And I do manage most of these patients. Um, And if there is a more of a friend of type of pulmonary hypertension, I typically collaborate with my colleagues and, uh, the pulmonary vascular program here at U. C s d. So let's, uh, kind of think about congenital heart disease. And if you look at the guidelines that were published in 2018 for adult congenital heart disease care, they do classify congenital heart disease too simple, moderate and great complexity. I do think this is a little bit difficult to apply in clinical practice. So for that reason, for the sake of pulmonary hypertension, I do want to draw your attention to this diagram from Dr Helen Tosic textbook, and that shows you the both the systemic and pulmonary circulation and normal people. Uh, so the way I think about angel heart disease and pulmonary hypertension is the location of the shunt. And is it a pre tried cast merchant or is it a post track has been shot? And obviously this pertains to those with four chambers, not patients with complex forms of single ventricle physiology, but generally the vast majority of patients you should think of them as pretrial versus post strike has a chance. So example of pretrial custody chance are obviously the post market is atrial septal defect. But also doctor here would put in a plug in for partial analysis for making his return, which is often missed. Uh, there are other rare forms, like a Sinus defect and other complex convolutions. The most common post bicuspid region is ventricular septal defect or defected, the ventricular septal uh, location. And there are several forms, but also other rare issues, such as patent doctors Rto sis and I would call me with them that challenge with congenital heart diseases. Sometimes there are a lot of combination allegiance, and you really have to account for those when you're approaching these patients. So let's start with cases. Uh, this is a 73 year old male, and actually it's a patient that I shared with Dr Heywood and Dr problematic, as I thought it was properly suited for this, uh, presentation. He has history of atrial flutter and had ablation in the year 2000 and again 2015. Um, he really denies symptoms, but when you sit down and more salient, he talked to his wife. Um, they both mentioned that he does get winded when he climbed stairs or any uphill. So he avoids going any uphill. And honestly, he doesn't have any symptoms on a flat surface. So an echo is obtained. This is an atypical four chamber view for the non Cardiologist. This is the left ventricle. This is the right ventricle, and you can appreciate how the right ventricle is substantially dilated compared to the left ventricle. And both ventricles have normal function. We have a color Doppler, Uh, and this tags the flow of blood around the inter atrial septum. And what you see here is there is no inter atrial septal defect, um, or no atrial septal defect, which is the most common cause for this. So oftentimes patients present like this with these big symptoms, they get an echo, and they have a dilated right ventricle. That's really where we need to pause and think of the differential diagnosis of a dilated right ventricle. Believe it or not, the list is relatively short, and if you remember to from today, you probably will not miss 98% of the issues. So atrial septal defect in the various forms and partial anonymous form a venous return. Both of these legions would result in an exclusively dilated right side of chambers, other forms and Sinus cyanosis defect, which some may consider an acceptable. The effect. But also there's other rare, fiscally like a corner of camera fiscal, like a body ruptured sensible Salva. These typically lead to more than one chamber dilation. So not just the RV. Typically, the LV is also entitled to, but I want you to remember from this talk ventricular septal defects do not lead to dilation of the right ventricle, because the ventricle that is doing most of the work is actually the left ventricle, doing sisterly where the blood comes back through the pulmonary circulation to the left ventricle. So the left ventricle is typically the ventricle that dilates, and this is a common board question. But please remember atrial septal defect and partial announced formally venous return. So we do dedicated adult congenital heart disease echocardiograms that are typically, uh, much longer than a regular echocardiogram. And they're done with congenital heart disease certified stenographer. So what we're highlighting here is that this is the superior vena cava going into now we're focusing on the atria. Uh, the left atrium is here behind the aorta, and this is the right atrium trying to spit, valve and right country pulls up there. So we're looking What's behind the heart? Uh, typically, you see the right upper pulmonary vein tags right here. That typically goes behind the superior vena cava emptying into the left atrium, which is not shown interview here. But what you see here is that the right upper pulmonary vein had emptied into the superior vena cava and the junction before it joins the right atrium. So that's a partial anomalous pulmonary venous return, uh, to the lower SBC superior vena cava. And this is a nice example of 40 flow that we do here for regional patients. And this is the superior vena cava going to the right atrium, right ventricle and the pulmonary artery. And you see, there's a right upper partial, anomalous pulmonary venous return emptying into that. So this patient had partially nervous for moving this return? This is a type of there is no Sinus windows. This defect, um and this is a diagram representation of both games. But typically, it's the right upper vein that stops short into the spc. So what would you do for these patients? Well, for this particular patient, he underwent cardiac catheterization. So pardon me here, but try to keep track and post any questions and comments if you have any, but I will go through this kind of slowly. Uh, this patient has a left to right shunt here, depicted in the right upper palm A being emptying into the spc. And there's no other shelves. So we look at these mixed venous saturation, which was 68% contrast that to the P A saturation 78% and his pulmonary veins were fully saturated at 97% which was equal to that. You were exaggeration. So using these numbers, you can off to calculate the QP qs, which means QP is the pulmonary flow divided by the systemic flow. This is a left right shunt loading the right heart and the lungs, so the pulmonary flow is expected to be higher. And what that means is that the pulmonary flows 50% higher than the systemic blood flow. Hence the overloading the RV and causing RV dilation. There is one important caveat when you're managing these patients. So if you look at the pne pressure in this patient, it's 25 millimeters mercury, which meets criteria for pulmonary hypertension. The way just love. However, you know, with the increase in flow, there's increase in the pressure. So you really have to account for the increase in the flow. And you have to calculate the pulmonary vascular resistance. And when you calculated the calculations basically PM any primary Armenian pressure minus the wedge divided by the cardiac output. Typically, that denominator the cardiac output is a fixed number, a single number when you Kath patients. However, when you're dealing with a shunt, you have to look at things separately. So don't rely on what the system comes out. You really have to look. Is this a systemic output, or is this the pulmonary output? So if you use the pulmonary output, which is 8.2 liters per minute the pulmonary vascular resistance is 1.8, which you know, which is the upper limit of normal, if not borderline elevated, so it's not really significantly elevated. But if you do put in a denominator in this case it's 25 minus 10, which is 15. Divide that by five. That puts the patient in the category of pulmonary hypertension. So for people who do these cardiac catheterizations, you really have to account for the pulmonary blood flow in your denominator when you're estimating pulmonary vascular resistance. So this patient has a high shunt, dilated right ventricle and is symptomatic. So for this particular patient, surgical repair is class one, and the way we repair these patients as we present them in the multidisciplinary conference, we have a congenital heart disease surgeon, uh, that operates on these patients, and there are several methods to repair. Partial enormous point Venus returned. The most common method is, um, tunneling of the pulmonary vein by creating an ASD in the post war world, the atrium channeling it into the left atrium. But sometimes, if the pulmonary vein is complex and its relationship to the SBC, you do use the SBC as a conduit. Uh, this is called the Warden operation. It's a much more involved operation and really most of the time tasks the surgeon decides when they're operating on the patient. One caveat is that an inexperienced surgeon may actually put, um, searchers into the pulmonary veins, which, unfortunately, that results in pulmonary vein stenosis. And hence why you may sometimes have to do these elaborate surgical repairs to avoid disrupting the primary Venus flow. And that patient is currently lined up for this kind of operation. Uh, the second patient is a 28 year old patient, previously very healthy, live a normal life. For the past years, she's had very rapidly progressive DeSean exertion to the point where she's functional Class three, So an echocardiogram is obtained. This is parsed in a long view. What you see here is the most anti structure of the heart, which is the right ventricle that is significantly dilated, and the left ventricle is relatively small to that, and the mitral valve and aortic valve look normal. But then there is the structure that is sitting above the mitral valve, obstructing the flow from the left atrium to the mitral valve and the left ventricle. When you look at the short axis view, you appreciate how massively dilated that right ventricle and there's Malco adaptation of the trackers would have on on fast view. And there's also for your cardio fusion. A lot of ominous signs here for this patient and the Article four chamber view again demonstrate significantly dilated by 20 point right atrium. And again you see this membrane separating the left atrium into two chambers is really to differential diagnosis here. Corporate tree, autumn or supernatural ring for this patient. But this patient also has a severe track, considerable agitation. So the echo peer pressure estimate was 60 millimeters mercury. Add that to a 15 millimeters mercury of right atrial pressure that gives her a category of severe pulmonary hypertension if you only base your diagnosis on the echocardiogram when we did an MRI, and here you're rotating the heart to the back and you see a vertical vein taking the pulmonary venous flow all the way to the nominate vein, draining it into the right heart. So this patient has a massive left to right chanting and what this is in embryology. There is a vertical vein that connects the pulmonary veins to the abdominal pain and typically regresses. But because this patient was born with Cortright tree autumn or membrane preventing the pulmonary venous flow from reaching the right ventricle, uh, blood will go down the path of least resistance. And that vertical plane remained patent. And for that reason, she has a QP qs of four. That is, the pulmonary flow is 400% more than the systemic flow. So very little blood is reaching the left ventricle. That's a cartoon diagram of this condition. And you see here the courts are actually autumn. Um, of course, chambers, Treachery items three. Essentially. What it means is that because of this condition, uh, atrial and the pulmonary venous chambers are divided to three chambers, which is the left atrium below the membrane and above the membrane and the home. It means that the third chamber and here you see that vertical vein connecting to the nominee being leading to left, right, chanting. So this patient had a complete repair. The core human brain was resected and the tri casted valve had repaired. And in four months this patient had an echocardiogram. This is America. Now, this is a remarkable recovery of that way. Friendship. All that looks completely normal now. And the L V as well is now preloaded adequately. And you no longer see the inter ventricular septum bouncing in the context of her pH. So that's an example where you could actually do a curative operation to fix these kind of agents. The third case, um, is a 53 year old woman presented with syncope E and was found to have profound, exceptional, the saturation She's functional Class three and an echo was obtained very similar echoes to the previous patients, but very different underlying congenital heart diagnoses. So this is significantly under filled left ventricle, massively dilated, right ventricle and high, petrified and right atrium and the R V S B estimates from the trick with regurgitation Jet is in the severe pulmonary hypertension category. And when you look at the cost of you, you see a flash of red taking blood from the left atrium to the right atrium. So she does have an atrial septal defect. Um, in the context of her pulmonary hypertension on Cath, her PM in was 56 with a wedge of nine and a QP qs of 1.3 when you see a low QP qs or low pulmonary, versus a systemic flow in a patient with an SD that is big and the SD size here is big, this is not a good sign. It means that the right side of chamber pressures are high enough to compete with the left side of pressure chambers to the point where not a lot of blood is going from the left atrium to the right atrium. So that's severe pulmonary hypertension. So this patient was started on Massey Tintin, and today Louisville and a six month follow up cardiac catheterization showed. And this is the previous numbers here, for comparison should really not a significant change in the P a mean pressure or the word pressure. But what changed dramatically is a QP qs. So now when you treated her, she is shunting more left to right. When you shan't more, there's more flow. The pressures do not necessarily change. That's where you really have to calculate the PBR and pay attention to these patients. So, you know these are very complex cases and it requires several cardiac catheterizations and considerations because really closing that ASD precipitously. Acute RV failure to have to be very careful and have a high, uh, a multidisciplinary, a CHD center. Evaluate these kind of patients. But there is some data, actually relatively older now, but salient, uh, asd closure in patients with pulmonary hypertension. So, um, really, as patients develop true elevation in the pulmonary vascular resistance and they do have a large ASD, the more severe forms of pH, the less likely they are to normalize their peer pressure following asd closure. But the way you close the STS complex. So for these kind of patients, we do consider finest rating a device before we close the ASD. And the idea is, you eliminate the volume loading, but at the same time, uh, you leave a pop off valve allowing the right atrium to vent into the left atrium in case there is a significant increase in the peer pressures with activity or anything that increases pulmonary vascular resistance. But when we did the tea, we noticed that the patient has really two separate, uh see condom atrial septal defects that are separated by a very strong ridge of tissue. And, uh, that was actually a good sign. So we didn't finish straight device, so we, uh, made lemonade when we found this. Um So what we did is we closed the larger of the two holes when we left her with one posterior, relocated its B two, um, and that allowed her to have this pop off valve and at the same time allowed us to up titrate her family vascular, uh, coming to visit the alligators without increasing the shunt fraction. So the functional status improved significantly within one month and a six month follow up Peer pressure was lower was in the mid thirties, but she does remain with only hypertension. So it's very, very important for these patients that you do not stop their medications. They require medications for the rest of their lives. And please do not close the A s. D s without consulting with adult congenital heart disease center. Because there's a lot of cases reported of acute RB failure and death following these devices. The last case is a 28 year old patient. Um, he has a very longstanding effort, intolerance and more recently presented with hypothesis. And an echo is obtained. And this is his echocardiogram. Personal Longview showing That's right ventricle, which is petrified left ventricle here. What you see is a large communication between the two ventricles, and if you pay close attention, you see that there is blue and red flow, meaning that there is back and forth flow across that defect. And indeed, when you do a cardiac MRI, you notice that there's a very large, uh, ventricular septal defect. Uh, that is causing this patient's symptoms. So this is a membrane is ventricular septal defect with an outlet extension? It's a very large VSD. Essentially, that patient has Eisenmenger syndrome. This was described originally by Victor Eisenmenger in 18 97 and what it means is that there is a bidirectional shunting across a defect in the heart most commonly related to pulmonary hypertension. So that patient, um, you know, this is a difficult population of patients to manage. There's a lot of nuances to their care, but essentially there are more and more available when we visit violators, which he was started on. Uh, it did improve his quality of life in terms of functional class, but ultimately this type of patient will require lung transplant and VSD repair, if not heart, lung transplantation, depending on a lot of different factors. So in conclusion, uh, echo estimates of pulmonary artery pressure are often misleading In patients with congenital heart disease, you really have to have a detailed an atomic survey and take that into context when you're evaluating these patients, and the combination of medications and surgical or trans catheter interventions are often complementary. These patients with congenital heart disease and public hypertension and most patients with pre trip inspections and true pulmonary hypertension. They do often benefit from partial inclusion of the oceans, if bland very well. And women question, please don't hesitate to ask for help. This is our larger group here, phenomenal people, and this is our contact. And I'll take any questions now, uh, where people are waiting for questions. Um, when you put in this administrated as, uh, for the A S d. Can you ever close the administration, or does that just stay open forever? Great question, Dr Hayward. Yes, the answer is yes. We do close administration eventually if the patient demonstrates that we do a lot of cardiopulmonary exercise testing in these patients for two different reasons. One is to look at the saturation so they saturate. That means that there's something right to left. We shouldn't close it. Anybody who shuns right to left with these administrations shouldn't be closed. That's an actually adequate pop off if they don't de saturate if the right ventricle size and dynamics are much improved. Um, then typically, we do Catherine at least twice, uh, to assess their PVR before closing. We do closing. There's another question. Uh, could we explain why? I mean pulmonary arterial pressure does not capture patients with left to right chanting, Um, you know when there is increasing the flow in a pulmonary vascular bed, the pulmonary vascular that is high capacitance bent. It doesn't accommodate a large volume before there is increasing the downstream resistance and which translates to increase in the pulmonary pressure. But invariably people hit their limit. So as you increase the flow across that vascular bed, the pressure has to rise. And that's really what it implies. The mean pulmonary artery pressure increases, but the actual resistance across that bed. You have to account for that flow in your fit calculation that you pick us. And there's another question. Why do you think these pulmonary hypertension meds work in the A CHD patients and not the other categories. Another great question. Um, well, I would qualify that with it depends on the question of heart disease. Legion. Um, there is reasonable and good data supporting news with these medications and patients with Eisenmenger syndrome. Um, and the underlying thought which it may, it may actually be counterintuitive. If you have a large VSD and you give people from the visa detonators, aren't you increasing the shunt and worsening the patient's symptoms? But indeed, contrary to that dog man, the late nineties and early 2000, there were several randomized studies looking at medications such as was sent in. And indeed, patients did achieve a longer six minute walk test and had improved quality of life. And the you know, the concept is these patients do have some level of family visa reactivity, uh, when they're dealt with high pressure and giving up on the basis that later may actually alleviate that allow them the capacity to exercise without hyperactivity in the pulmonary vasculature. Um, but other forms of congenital heart disease that are mixed you're really addressing the pulmonary arterial hypertension component, not the other types of shunts
