Chapters Transcript Video Abstracts FORUM / Audience Q&A Click here to view presentation deck Dr. Matthew Berens moderates 3 interesting cases presented by EVMS Fellows and leads discussion with faculty. Presenting Fellows: Amy Cecchini, D.O.Kyle Admire, D.O.Sam Anderson, M.D. All right. Well, uh thank you all for coming today. Uh One of the things that we have always done as part of the Ph symposium is to have trainees present interesting cases and abstracts and we are blessed to have uh a number of our fellows here today. Um Our fellows traditionally have been very interested in pulmonary hypertension and RV failure. And so we've got some interesting cases. We decided to change it up a little bit this year. So what we're gonna have happen is the fellows are gonna present their cases and periodically we're gonna pause for some discussion and get some input from our expert faculty. And then at the end of each case, we can kind of close with a few concluding thoughts. So our first case is going to be uh Doctor Chinni, one of our second year Pulmonary Critical Care fellows. And she's gonna talk about a, a congenital case. Everyone hear me. Awesome. Um Thank you so much, Doctor Burns. Um and for your guidance uh with this case, so this story starts, um how many do hello? I have a new Pulmonary Consult. Um And this was for a 75 year old male with hypoxia of an unclear cause. Um, essentially the back story. Um, for him, he came into the ed, um, not a common person to come into the hospital for anything. Um, with abdominal pain, constipation and mild shortness of breath. Um, his past medical history included end-stage renal disease on peritoneal dialysis. Um, he did have a history of renal cell carcinoma status post, um, left complete and partial nephrectomy, which is why he was on um, dialysis. Um, he did have a history of aspiration pneumonia, um causing s with septic shock and a prolonged IC U admission um several years prior and then hypertension. Um So in the emergency department, his exam was um really significant for mild tachycardia. Um His uh he had a new oxygen requirement of 98% or sorry of um 4 L of oxygen. Um And it was adding 98% on that. Um Normally at home he's not on any supplemental oxygen therapy. Um And uh otherwise, um he had um uh slot tachycardia and then his abdomen was significant for a, a peritoneal dialysis catheter but no surrounding erythema, not really any other abnormalities. Um And then his lab values were significant for a mild leukocytosis um with a white count of 14.6. Um, his creatinine was 10.7 to be expected again in a perineal dialysis patient. Um And then uh he had a anti pro B MP level of greater than 70,000. Um And then his A BG showed a mild um alkalosis with a PH of 7.519 37.2107 and 30.3 on an honor breathing mask. So, um for further work up, um he did have ac T um abdomen pelvis, um which showed mild bladder wall, thickening and moder ascites. Um They did um uh send his uh perineal dialysis fluid to the lab and um he had elevated um WB CS and Group club Celis. So he was admitted for um IV antibiotic treatment of sepsis se to peritonitis. Now, I know what you're all thinking. What about the dy hypoxia, which is what we're consulted for. So, um this was very vague. Um his history whenever, um whenever he told us about it, um he said it's been going on for many years. Um It's worse with exertion. Um But over the past two days since he'd been feeling more poorly, um uh it was significantly increased. Um And they also had some mild clear to white speed in production. Um which again, kind of vague happens um intermittently with him. Um And then the next piece of evidence that we were able to get was whenever he came into the hospital. Um He did get an echocardiogram and this showed um an ef of 55% with indeterminate diastolic dysfunction, um asymmetrical LVH with apical and septal dis synchrony he had a very enlarged RV chamber and he had severe pulmonary hypertension on echo with a PSP of 105. His bubble study was negative for shunting. All right. So we've got AES RD patient with high P A pressures and a short of breath. Never see that any time. Right. So, uh so for our faculty, what are your thoughts about when you approach one of these dialysis patients who's got elevated pulmonary pressures? Do you change your evaluation compared to your typical Ph work up? Um Yeah, with ESRD, it's, it's hard. Um And there's a lot of changes in just the end, you know, the general, not he has a lot of issues going on, but in just in, in general with ESRD, you do have to look at multiple different areas that you wouldn't if the pa the patient has peritoneal dialysis, if they did, if he had a shunt, that would be a little bit different to see if it was just elevated because of elevated flows and you can be evaluate that. So that's something that we don't have to worry about here. But obviously, the patient had a creatinine of 10.7 and basically, is he getting enough fluid off? And so is that guy um is that, is that um pressure um really driven by a volume? Uh and you, you would, you know, real evaluate that with the right heart cath, but I think you would look at different things on this particular patient. He was also septic. So that would also, you know, some patients do um with any kind of injury or sepsis in terms of ph patients, they're gonna worsen. But I think, um I think doing the echo and then probably completing the full um work up along with some of the things we talked about knowing that he is an ESRD patient and had he magically received the sep 130 CCS per kilo bolus. Such that uh in addition to the under dialysis, he'd already had by virtue being PD and under fluid removal because the amount of dextrose and osmolarity of the fluid was insufficient. He also got three more liters or four more liters of fluid. Um Obviously, they're having an end sty. So they need a left heart cath, right, check that troponin nice reflex. Um You know, um the uh uh all joking aside that certainly is a independent predictor of mortality. Um So that's going to be a problem there not to check the coronary, um fluid optimization, fluid fluid fluid fluid as you're able to aggressively with inotropes. And um you know, getting your nephrologist either to do daily dialysis, which, you know, are thankfully, our nephrology group uh is very user friendly. They don't we ask them to take fluid off, they will um or CV VHD if necessary. And then when you think you can only make obviously, right heart catheterization, I mean, absolutely all those things. And I think the other thing to ask is why do you have renal failure? Do you have lupus? Do you have HIV, what is, what is the risk for that 105 being real? Pah. At least it gives you a little idea. Or do you do cocaine or methamphetamines? But the population now is like, up to 2% of people are meth exposed. So we gotta ask the questions. Yeah, I mean, I, I think everyone's answered it already, but I, I think the PD patient can be particularly challenging in terms of getting them. You aic, right? So, um and that's a patient, you know, if bacteremia, it becomes a little challenging in terms of getting access to get them adequately dialyzed if you can't clear the bacteremia. But um you know, getting them U VIC before proceeding with, with heart catheterization, I think uh Doctor Levine alluded to this as well. But if they've got an A V fistula, do they have some high cardiac output going through that fistula? Do you need a official occlusion study? Um during, during right heart catheterization is something else to consider. And then, you know, uh this patient with a lot of medical issues. Uh I, I think you still have to think about things like caf right? Um And so making sure you're completing the rest of your diagnostic evaluation. Absolutely. So, oh, is it on? Oh, it's on um in terms of the, um, uh, you know, I, I don't know what it is but it seems to me when I've seen patients that are older, it's, the PD is just not really, it gets less and less effective over time and patients love it. You can do it at home. They think they're getting great clearance and sometimes the nephrologist says they get very good clearance but clearance isn't, um, fluid. And I think that's the thing that the, you know, the patient, you know, he thinks I'm great. I don't need to change to HD, but sometimes that's not the reason. And for the patient with PH it absolutely is more, we're more worried about fluid than, than the clearance. I mean, we care about clearance as well too. But yeah, I think A RBS P of 105 is concerning regardless that can't be all fluid. All right. Well, we're gonna give you a little bit more information. All right. So, um what work up would you like to see next? Um The next question? And so I'll show you what we have. Um This is his chest X ray and um I know whenever you see that that's uh it's, it's a pretty cool image. Um So the first thing that uh you would see is he has a pretty deep created trachea here. Um And then he has this significant rightward shift of his mediastinum, his heart and mediastinum. Um The left lung is massively hyperinflated. Um more pictures that come and the right lung is small. And um and uh more fibrotic appearing here is one of the first images I have of AC T scan and believe it or not, all of this is his left lung um again, massively hyperinflated. And this, this little thing right here is his right lung. Um And it's very small and um all of this is his left lung. And then this little thing is his um is his very small fibrotic right lung. So you may ask why does he have um this imaging? And I'm gonna show you a video first um of the entire CT and then I'll show you um uh the contrast enhanced imaging again, all the left lung there with the right word shifted mediastinum and just a little, little bit of the right lung there. And then, as you can see, it's pretty fibrotic, but he's not massively volume overloaded. You know, there's not, there's not um severe pulmonary edema or anything that we're worried about him being um you know, massively volume overloaded. Mhm And here we will see why. So if you watch carefully, you'll see here that he has complete absence of his rat pulmonary artery. And here's an interpretation. So essentially, um this was uh this was obtained in order to rule out a pe because they were um they were worried about him, obviously had a shortness of breath and a new oxygen requirement um there was no pe but they did see no identified right pulmonary artery, which was presumed congenital and then marked hyperplasia of the right lung with volume loss and rightward shift of the heart and mediastinum along with chronic changes on the right bilateral ground glass opacities. Um again, not, not severe pulmonary edema, but just a little bit of volume overload. And then um the left lung um with some uh fibrotic changes, small airway disease. So, um at this point, we had all this information and the Pulmonary Consult team ordered a pulmonary perfusion scan, um uh quantitative. So we can see exactly how much flow that the uh right lung is getting. Um it's 00 flow um which is not surprising. Again, looking after you, you've seen those CT images and here are the images. Um So you, you can see really there's, there's zero flow here. Um So the rot lung is just kind of operating on, on uh on crumbs. Um And uh we did go ahead and complete the work up for um for ph we did um obtain PFTs. Um He had a variable extrathoracic obstruction um and uh overinflation, air trapping and moderately reduced um DLCO and then we um also obtained a six minute walk test. Um He was requiring 1 L of supplemental oxygen to maintain sites above 88% at this point and ambulated 56 of his 56% of his predicted walk distance Um however, he did decline home oxygen. Um and then we did obtain Zies as well, um which were all negative. So, in summary, thus far, um he likely has pulmonary hypertension um and it's likely a multifactorial causes. So the the main one being his complete absence of his um right pulmonary artery. Um but also he um had indeterminate um needs another echo, indeterminate, diastolic dysfunction. Um uh And then he's also an ESRD patient on perineal dialysis. And then also we know he has chronic hypoxia too. So this, it is a difficult case because he has many, many reasons um to have pulmonary hypertension. We really want to uh attack each um each cause and they're just going into um into uh all the causes here that um uh all of you all have discussed today. Um So again, he has indeterminate diastolic dysfunction. He has his developmental abnormality um as well as um uh likely uh chronic hypoxia um and then chronic renal failure on dialysis. So, so many, many types. All right. So it kind of an interesting finding now with the imaging, this definitely changes our approach with his Ph. So uh I guess the questions that lie before us now is what else do we need to check? And if we do a right heart Cath and he has precapillary ph, do you treat him? Yeah. You know the thing that I find really interesting is that he's 75 and he's just now coming in with symptoms of shortness of breath. Uh what happened from age 0 to 74? That he was feeling great. That that was one of my questions. But I think, yeah, you, you'll complete it. I don't know if a cardiac mr would help you at this point. I mean, before doing the right heart Cath or while you're getting him fluid down and before you do the right heart cath, would that help you at all? So I would imagine that this echocardiogram would be complicated. I don't think you get good images. I think a cardiac mr though it would not give you pressures would certainly give you an idea of what the function is. Um I have to be honest if this came across my desk to read, um I'd be like, oh, this is gonna take a long time to read. So, um I think if you're looking for function, which I imagine your uh echocardiogram is gonna be near impossible to get RB function, I think it would be quite helpful. Um And then as the one probably doing this right heart, I'd probably be a little concerned that I can't get a wedge and I probably plan for an lbedp upfront. Very important to communicate the concerns you have on the right heart catheterization to the operator. Make sure he, they know they don't have a right pulmonary artery and they seem like really stupid things however they're going. So fast in the Cath lab that they'll do maybe five or six of my patients in a day. If I don't tell them those things, they will treat them all the same with the same protocol. So I think that would be really helpful and then, you know, somehow communicate likely lbdp this, that left pulmonary is gonna be gigantic. You don't really want them to keep trying to wedge it, you know, rupture A P A but would you guys treat it? Say you dry the guy out, you have everything else, you treat the sleep apnea, make sure there is not no acidosis would you treat? I, I want to hear the rest of the story. Ok. Ok. I guess, you know, the other thing I would consider this gentleman who may or may not. I mean, he's got multiple risk factors on top of being 75 for having, you know, real deal like dye stock dysfunction. On top of all this is even if you were to consider treatment, would that all be accommodate the preload from therapy where we would just really make him worse. And so if you did dry him out, you got him. Perfect. Otherwise, um you know, maybe a fluid bowl is not the best option for this gentleman with um MP D but uh you know, exercise uh hard cath to see, see what happen to that wedge or LVDP. And you've got the other problem that he's now just had eclampsia, peritonitis and you've got to make sure that he is. Peritoneum is a suitable substrate across which to dialy because obviously converting him over to a fistula is unacceptable with those right sided pressures. So that's another problem. And on your CT, you did comment, there wasn't a lot of pulmonary edema though these people do compensate with a lot of um lymphatic drainage. Um He almost certainly could have that's alluded to diastolic dysfunction. Um II, I would be highly worried about that and years and years and years and years of hypertension cause how did these people end up in this situation? They have hypertension. There's gonna II I have yet to meet a dialysis patient yet. That doesn't have some degree of diastolic dysfunction. Uh Doctor Cini any more story. Um Yes. So unfortunately, he is not followed up for rat heart catheterization yet. So doctor, so consider this your hand off. Um So hopefully he'll be coming to you um whenever we can convince him to have that done. Um And to answer your question, Doctor Levine, um he, he was a marine and is just a very um sturdy man and he knew something was wrong with his um lungs but didn't really mind. Um It was manageable for 75 years. So that's, that's uh what's been going on with him up until this time. Um So our next steps, um how are we gonna treat this, um this man? And so all of you have um thankfully um uh discussed this already. So for um his uh inside renal disease, um we really want to optimize his volume status um for his um absent uh right pulmonary artery um with the fibrotic right lung and over distinction of left lung. Um We did um from the consult service add bronchodilators in order to try to improve air flow um uh for his chic hypoxia, we've encouraged supplemental oxygen. Um And again, he's declined that, but we'll see how um symptomatic he is on follow up. Um and see if he'll agree to that and then um for his indeterminate diastolic dysfunction, again, optimizing his volume status, um maintain euralia. Um And then obviously, we'll get um follow up echo um and hopefully write heart Cath um once he follows up. So, um how does this? Yeah. So we, we're running a little bit short on time, but I do want you to present just what you found with the evidence about treatment. Yes. So we can just skip to that. Ok. All right. Um So, um on my literature review, um we uh with unilateral absence of the pulmonary artery, um it's really rare. Um There's actually only about 350 cases reported in the world literature between um 1868 and 2010. Um And it's due to the failure of the six aortic arts to connect with the pulmonary trunk during embryo logic development Um So obviously, with these numbers that is really limited, um there was one retrospective review of cases of 100 and eight patients um that showed that had a unilateral absence of the pulmonary artery that showed 44% of those patients did have ph um and on echo at least. Um and so there are not really any formal guidelines on management. However, this um this uh review did suggest that um your release screening echoes um would be important um to look for development of ph in these patients. And then there was one other um analysis of 65 patients um that suggested that ph treatment with um vasodilator therapy may help significantly. Um And uh that the patients that were in that study that were treated with pulmonary vasodilators um specifically um uh your um PDE inhibitors and your um um er A s um did um show an improvement in uh shortness of breath and their who functional class um in 50% of patients with congenital P A absence and pulmonary hypertension. However, um the uh the number of subjects was very small. Um I'm thinking uh like uh almost single digits small. Um So not a great, not a great sample size here. Um So here are my references and um I'd love to hear any other comments that you all have. Just um we did have a young girl who did have um I guess she was one of 350. I didn't know it was so rare but she was young. We did try to treat her. Um, um, and it was with S the NFL. Um, she got no improvement. I mean, she was very, very functional. She was a runner. I mean, she was only 25. So, I mean, I, I don't know how she is now because I'm not in that program anymore. But I think, um, I think as people, you know, you know, get older, I don't know. But I, I think it's very interesting about um about treatment and, and when you would, when you wouldn't, I think the, the way you um um gave this case was great. The, the pictures are beautiful. If you get the right heart Cath, you've been doing an MRI, I think it would be a great case, not only for um to be, to be presented at a conference, but also even to write up. It's a great case and you did a great job. Thank you. Thank you so much. Our, our next presenter is gonna be Doctor Admire and he's gonna present a, a case of a patient with Pah who has some malignancies. All right, good morning, everyone. So I am, I'm Kyle. Thanks, Doctor Burns. And I'm gonna tell you about a 53 year old female who has a past medical history of Sjogren's disease. Drat myositis A BM, I greater than 40 with some sleep disordered breathing groups, one and three pulmonary hypertension, immunosuppressant regimen that she is on is pretty intense. She takes azaTHIOprine methotrexate, mycophenolate and predniSONE. All of which we are told is at the lowest possible dose to control her rheumatologic disease by her rheumatologist. And as far as her inhaled medication, she takes uh Tizo and Saldana for her pulmonary hypertension, she gets ac T scan for routine monitoring that shows us she has a new left upper lobe nodule that our radiologist must have gotten a new ruler and measured it at exactly 11.14 millimeters that day, but no other obvious lesions. Follow up. Pet scan did show only uptake in this region. So here we have a heavily immuno suppressed ph patient, new nodule, pet avid. So I guess the, the quick question because we've got more of the case to get to is how do you approach this? Do you biopsy it or do you just ask them to treat it? I don't know what they would treat with. So I think we'd have to, I think it's close enough to the wall. Um We don't know much about their, the rest of their um, disease dates. I'm, I'm assuming that there's more um that we'll know about in terms which we've just got now, I guess, um, just showed up. So I think, um, I think you would have to biopsy it with the probably trans th acid. That's probably your safest bet. Right. I think you're fortunate here that it's, it's peripheral enough, right, that you're, uh, operative risk from doing, uh, a needle biopsy, um, in a patient with severe peach, which doesn't make any radiologist excited. Uh, it's probably on the lower side. So that's probably the right way to go. But also communicating that you're worried about infection. The communication part is so important because we can't poke her twice. Absolutely. So, I think those are excellent suggestions. This patient was ultimately deemed too high risk even for a transthoracic biopsy by our radiologist because the pet showed avidity but no evidence of metastatic disease. She was planned just for an emer empiric radiation treatment with acceptable response obtained and this is her follow up ct that does show an interval decrease in the size of this nodule. Following completion of her radiation therapy, she was found to have this necrotic hard palate lesion in which we can see some necrosis. We see um some purulent material here and some part of her posterior pharyngeal muscles as well. This was biopsied as an outpatient that grew actinomyces. And then when she was in our facility of subsequent biopsy grew ESPL Club ciela Vancomycin resistant tero coccus candida species and Aspergillus species. After a lot of deliberation with our infectious disease colleagues, she was planned for treatment with Daptomycin, Meriem and Boro Conazole, which likely would require modification of her current pulmonary hypertension measurement. And after this was completed, she did decide to pursue surgical debridement maxillectomy and a peg tube placement for feeding. So obviously she's gonna be undergoing a pretty big surgery. It's gonna be involving her airway. Uh Right now she's on Sildenafil and Tyvaso. So Sildenafil could potentially have some issues with her medications. Uh And what would you do as far as optimizing her for the or doctor admire? Could you bring back her hemodynamics cause that might help answer the question? I can't perfect. Thank you. So we see, obviously she is se severe uh estimated pasp of 73. She's got poor Respi phasic variation in her cava with an estimated rap of 15. She has an anterior pericardial effusion. Her f is decreased, her RV chambers moderately severely dilated with it, you know, with the basal diameter of 5.6 and she has a thick PV R of 10 with ac I of rough between 1.5 and two. Um otherwise known as a hot mess and a pas a of 54. I mean, um Doctor Owen went over risk stratification very nicely, but I don't think you need to calculate uh the reveal 2.0 to say that this patient is uh probably undertreated and, you know, with Tiso and silent uh alone to begin with. And so I think, you know, she's gonna need augmentation of ph therapy. Um You know, so pross obviously be a consideration here, you know, vic Conazole obviously could cause an issue there with the Sildenafil, um you know, from an ID perspective, uh I Bansi is another alternative, maybe with less interaction there. Um So you could alter there. But um I think the soil, you know, being held is the least of concerns here. I think the fact that she's, she needs more aggressive ph therapy is the take. Right? I think when I think uh um doctor Owing and I did a chest uh um what do you call it, uh, session on, you know, what not to do. But, um, the, one of them I think was in, you know, what, what not to do in the, or the patient that was my session she gave me that I said, and, um, you know, I think what we, you know, we talked about was, it was, um, you know, using, getting someone, you know, admitted started on, um, short acting, um, you know, um, probably we put prostin all and getting through the surgery, getting that done and then, um, getting out, um, I keep looking at you but I'm supposed to be looking, you're like, yeah, I know. But I think that, you know, even for that period of time while he's, or she's in surgery having her on IV, and then figuring out what works better after, I mean, she's getting a peg, she's getting all these things, you know, she's gonna have probably some absorption problems, you know? I don't know. But I think that's probably the best way to go and to the operating room and everything, maybe, um, transitioning to a, to a longer half life like modules. Yeah, I agree. I think you just be able to titrate her up quickly with Ebo. Right. And then, and then flip to something more because long term this patient is going to need parenteral prostin. Nos. Yeah. Yeah, that's, that didn't happen. Just wait, you know, there's more. So as we, we did discuss Salafi does have a pretty significant interaction with the Borica also, so we did need to switch her to IV Remodulin and this was prior to her planned procedure with what we can see is a significant improvement in her cardiac output. And don't, don't be alarmed, obviously, by the fact that there is that is literally during the transition. So the fact that you've got both IV re module and inhaled tyvaso up there is not a typographical error that is simply that was done during. So we could see how she was doing. All right. So now obviously, her airway is gonna be quite a challenge. So she's gonna have to get induced for anesthesia. She's gonna have to be on the vent for a decent length of a case. And then how are we gonna manage her airway postoperatively? I'll tell you the ent folks debated whether or not she would need a tra coming out and they kind of decided they were gonna make a game time decision in the, or depending on how things looked. So, any, just any quick thoughts about, you know, your approach with induction and what you would advocate for as far as your airway. Wow. Um, that, that is horrible looking. I mean, um, I don't know how long that surgery would take but, you know, there's all so, so it's not like, you know, when we have our pregnant patients and we can do a, a spinal and a continuous. It has to. So I think in my, in my eyes, I don't see any tube going in the oral or Naryn um with that thing. Uh it looks horrible. I would, I would think probably tr would be important. Now, induction so difficult with patients with Pah, we've had, you know, I had a patient going to lung transplant, a young girl, 25 years old who so excited for her lung transplant and on induction for the transplant, um crashed and, and died. And that was probably 11 of the worst things I've ever seen in my life. I was a brand new attending and I'm like, oh, how am I gonna talk to the parents? Um uh her husband was a dog trainer for puppies for the military in San Antonio. It was, he brought all these puppies with him all the time to clinics. You know, I like them. It was a really hard situation. But so induction has to be a huge team of um cardiac anesthesiologist, um cardiologists as the emo team um on board to figure out the best um in, in, you know, um um uh um regimen. So this patient was started on a low dose epinephrine prior to the or for prior to induction. She did undergo general anesthesia with the Tate and a 6.5 intraoral endotracheal tube was placed, she had an extensive debridement and did remain intubated, postoperatively very briefly, induction agents with Ph the choice is extremely important. It tomo typically is used because of its low effect on systemic and pulmonary vascular resistance. And when combined with synthetic opiates, you can further improve the outcomes for our patients. Propofol should not be used because of its propensity for hypertension and myocardial depression. And ketamine initially thought to increase PV R it does so at high doses. But at lower doses, news as a co induction agent, the patient outcomes actually are improved. And this is that again, just in a picture form for those of you who are more visual learners like myself. This is post-operative after she was extubated and this is what her surgical site looks like. Now, we also got some cytology back from her case that shows she has a CD 20 CD 30 positive diffuse large B cell lymphoma. So the standard of care for this therapy is arch chop riTUXimab cyclo phosphide, DOXOrubicin, then Christine and predniSONE. However, because the DOXOrubicin is essentially contraindicated in pulmonary hypertension patients. She received a topic side for this. There are a long list of anti plastics that can affect pulmonary hypertension patients. The most prevalent ones are the anthracyclines like your DOXOrubicin. Um The astute listeners may have noticed that I did mention cyclophosphamide and been Christian in the last slide. However, the standard of care doesn't change. There are some recent data to show that the R CEO P regimen doesn't cause any increased mortality in these patients. This is her pet scan, pre and post chemotherapy. So we have before on the left, all of this is increased uptake. And then after on the right where you connect and you can see the maxillary um debridement and maxillectomy that was done here. However short, during the same pet scan, we now see a recurrent finding where her left upper lobe shows an increase in uptake essentially in the same area that she had before that we treated with the stereotactic radiation. This time, we could convince radiology to biopsy for us and we found that she had keratinized squamous cell carcinoma with a 15% TPS, which means that keytruda would not be first line to be second line therapy. Again, deemed prohibitive risk for surgical resection. So she is actively being managed with stereotactic radiation. So in summary, this patient with high dose immunosuppressants that cannot be decreased for her rheumatologist had a left upper lobe nodule that we presumed was cancer and treated as such radiation then developed a lymphoma of the hard palate with a superimposed polymicrobial infection and then a subsequent left upper lobe squamous cell carcinoma, which we are presuming is a recurrence of her previous lesion. So, multiple malignancies, highly immune suppressed ph patient. Do you get more worried about malignancy in the PH population? Yes, especially those with the Sjogren's and Ere's Priest risk of lymphoma. So they get a nodule. You gotta be very, very clear with them. You're not a smoker, but we're gonna follow that, right. Um And if you can get a biopsy that would have, that would help. But you got, you got two different things going on. And the other thing is that high dose immune modulation. So I I just have a kid like a kid who has this invasive cancer of his face. Uh There's no going back and the argument was literally to leave him on that versus take him off. I'm like he's not dying of his rheumatoid arthritis. I promise he is going to die of this and he will now. And we thought that the other thing that was really important about this case was, you know, ordinarily why does this lady have all of these processes? She has a oropharyngeal lymphoma, which is a rare presentation and a rare site with superimposed polymicrobial infections, several of which are rare, including Aino and Asper and a non Feig Goddess Aspergillus. And in the setting of, you know, and then has pulmonary hypertension. Um And so, you know, how many and has a neo, a presumed neoplasm that requires stereo tactic therapy for management. And so how unlucky does she have to be? And so as Ockham's razor fail, no, it really doesn't because at the end of the day that QD ruple combination, immunosuppressive therapy was clearly enough to upset her immune surveillance such that she could not defend against infection nor could she obliterate malignant clones in a patient with Sjogrens. And so that's how and so that was, you know, we felt that was key to understanding this. It wasn't that a razor failed. It actually, once you got down to the cause, which was the immunosuppression, it made sense that um uh the other thing is in malignancies, especially hematological malignancies. Um looking at the treatment and the therapies are often, you know, um uh if it's, you know, it's often lots of fluid and um actually even undergoing bone marrow or stem cell transplantation, um looking at all of those things um in a ph patient is very difficult because of the fluid balances and the, the issues that occur. And I've had several hematologists call and say, are we able to do this? And you look at the bags and bags of fluids it's gonna take and it's, it's, you know, it's a very, it's, it's usually they're seeing these, getting these therapies outpatient, you know, they're not getting them in, in a monitored situation, I mean, monitored but not inpatient. So I think that's something that um is important. Absolutely. And so, and I think this was very well said by the, by the panel that really what we're seeing here is Ockham's Razor where our patient was so profoundly immuno suppressed that she lost her ability to have her bone marrow fight off any of these neoplasms or these infections that she has. And we do think that this could have been prevented if we've been able to modify her immunosuppressant regimen if I get, yeah, make one comment. Um you know, Doctor Owen mentioned this already. But you know, we, we know in our post transplant patients, they have an increased risk of malignancy and we're often right, doing yearly skin exams and we're doing, you know, colonoscopies every few years uh on these, on these folks um to screen for malignancy. But people often forget about our connective tissue disease patients who are on as much immunosuppression sometimes as those that are post transplant. And so you have to remember you have to still be aggressive about screening for malignancy in, in those folks. And then, yeah, you, you, you talked about it but propofol bad, right? Uh So, I mean, I, I we actually had, I recall I had a, we had a fellow that rotate uh with us through Walter Reed and uh she called me in a panic one day on the weekend and said, hey, I'm in the IC U here and they're about to do a bedside endoscopy for one of your PH patients. And the G I doctor is about to push propofol and I grabbed his hand. No, we can't do that. And I said, all right. Well, you learned something, you know. Um, at least. And, uh, so just, just remember that. Yeah. Uh, we still see that not infrequently, especially in community hospitals, you know, uh, anesthesiologists, often times that are not cardiac anesthesia trained. Don't understand that point. Yeah. Yeah. And that, and that has to do with any surgery. I mean, I had a patient in Austin that she was calling me on, on her phone, called myself on the way to the, or, and said, oh, the doctor's gonna take out my gallbladder and, um, all this stuff and I said, let me talk to him. So she gave him the phone and he's like, this is what I'm gonna do. I'm gonna use all these different things. Pro I said, can you just send her to us and we'll just do the, do the cholecystectomy? Absolutely. You've got it. That's a great. So, overall, why is this important? And this figure, and the next few figures that I have are from a study that was put into pulmonary circulation last year. And this is on the, the Y axis here. You have your patients with the cancer diagnosis and years from the index state of the study. And in the 11,000 patient, the 11,000 pulmonary hypertension patients and the 11,000 non pulmonary hypertension patients who are age matched to these, you have a pretty significant increase. And even from year one, you have a statistically significant deviation in the amount of cancer that you see in the pulmonary hypertension patients compared to the non group. Now, this chart, I really just wanna focus on three things here. Overall cancer total compare in the pulmonary hypertension group is much more significant than in the non pulmonary hypertension group. And this is something that we see as age increases specifically. But even in the younger patients, there is a signal here, we're not quite a statistical significance, but there is some something to suggest that pulmonary hypertension in itself could predispose to cancer or the therapies for it could predispose to cancer. And as we just heard about skin cancer, very high hazard ratio here in terms of the pulmonary hypertension patients compared to non pulmonary hypertension patients. And even a lot of these other different organ systems, there's some signal, there's not necessarily a a correlation or a proven um cause between the two. But there is something there between pulmonary hypertension and cancer. And I think that this is something that may be under recognized, at least in the the general population as far as what we are doing with our therapies for these patients and what we could be leading them to Thank you, Kyle. All right. So I'm sorry, we're running a little behind time, but we've got one last case, we'll try to, to get through quickly. Um, Doctor Anderson is gonna talk to us about a PH patient who developed a surgical issue and how do we get him through the, or? Um, so this is mainly gonna be on perioperative management uh around Pah and a patient that some of us have seen already um in the hospital. So we'll start off. She's a 74 year old female, recently diagnosed PAH with group one features on Tizo and Senna Fil. Um coming in uh in June initially, a Pulmonary Clinic, pulmonary hypertension clinic has a number of other um risk factors for other groups. Uh Group three, she has moderate COPD emphysema os A not treated um some mild um fibrotic changes on her CTFFF had a splenectomy back in 2011 for large cell lymphoma also um Factor Five Leiden. So, uh risk for group four disease as well. So when she was seen in June, she had a full work up VQ scan was negative. CT shows some mild uh central lobular changes in her A P CS and some mild basal or particular opacities but nothing terrible. Um PFT is some mild restrictive and obstructive changes and DLCO of 30%. And um she initially was referred over to Pulmonary Clinic when she presented to her PC P with six months of dyspnea on exertion. He got an echo which showed this RVSP in 92 and a fractional area change of 15%. So that's why she originally got into the pulmonary hypertension clinic. So in July, she had a right heart cath of a fluid challenge. Uh initial mean pulmonary pressure was 42 with a wedge of 10 cardiac index of 2.9 and a PV R of 5.4. Um post fluid challenge wedging up to 16, uh pulmonary pressure up to 54 and her index dropped to 1.5 with a PV R of 12. So she initially presented uh October 12th to an outside hospital with nausea, vomiting for two days along with some lower abdominal pain. Uh vits were significant for not really uh remarkable blood pressure went on 2/70 heart rate, 88 02 sat 96% on her 3 to 4 L home oxygen. Uh She had a myo leukocytosis of 13.6000 and a pretty significant A K I with B un creatinine 56 and 4.1 respectively. Baseline creatinine is around two which underlying CKD three. So timeline she has CT abdomen done. Uh findings are consistent with the spo with the transition point of her distal jennum. She was, she had an NG tube place at that time, um was placed MP O. General surgery is following, was hoping for non uh non surgical resolution of this issue. Um she had a significant volume losses without replacement and her A K I worsened. Um She had PPN started at uh on the 14th. So a couple of days after and on the 16th, she still was not improving her A K I was worsening. So she was transferred uh over here uh for per operative optimization of her pulmonary hypertension for likely a small bowel resection. Um Here's her echo um on presentation. So E a 60% taps C of 1.8 taps V 19.5 and her f was 45% um had some flattening of Herceptin consistent RV, pressure overload. So we, we talked about this just a little bit before and in the sake of time, I'll ask that we just kind of hold our comments till the next question. Uh But just so, you know that this patient was uh transition to IV therapy. Her volume status was very difficult to manage because of all of her gastric losses. Um And obviously, we're concerned about her surgical risks. So we'll skip ahead but we can comment on this a little bit once we get into the or sure. So transferred over here BN is now 138 cretin 8.8. Um She was started on TPN and she had IV fluid uh replaced to match her gastric volume losses. Uh had a small bowel series uh which confirmed the SBO she was transitioned from PAD to IV or module um on the 18th and didn't have any issues with that. And on the 19th is when she was taken to the, or um for her procedure, it was complicated by uh contained bowel perforation and uh some issues intraoperatively. Um So she started the case. She went in on her module 11.7. She was already on vasopressin going into the case. Uh She started out with a map in the low hundreds and a mean uh pulmonary in the mid forties. Uh She had a pulmonary artery cath uh placed prior to going to the or um induction was relatively smooth, no issues with that and she had maintenance anesthesia with iso fluorine. Um after induction or maps did drop in the low seventies which was um combated by Epinephrine and Leva Fed. Um And she was actually doing ok on EP three and Levi at five. so started to get some sinus tachycardia into the one twenties. She's given EMA bolus which decreased her map and was given phenylephrine afterwards to that map decrease. And at this point, she had equalization of pressures, the mean P A into the fifties and her map dropped into the fifties. Um She also had about 800 mL of fluid given during the time. So that's uh that's one way to do things. Um Do you have any uh comments and maybe uh an alternative approach? And then how do we rescue this patient now that we're in the spiral and this is definitely where we can take a little bit of time before the next, the last two lectures to, uh, uh, get the, the, the opinions of our expert. I think one thing is, um, you know, what not to do, be going to a complicated orks, what not to do is not to have a huge, um, multidisciplinary team meeting before even stepping foot in the, or what's gonna happen. What do we do before? What would happen if we do this? What exactly do we wanna use? Talking with the anesthesiologist, the critical care guy, exactly what you wanna use and not use. Um And maybe even undergo uh a little um pre uh what that could happen. And what would you do? I think um ECMO obviously would have to be there all of the people, all the things um really um and maybe that was done but in sometimes in emergencies it can't. Um But I think that would be the first thing. This is characteristically been an issue on our campus with the limitation of the anesthesia, the noncardiac Anest Asia group, not wanting to effectively interface with uh the PH program in general. Um This has gone up at various times to the president of the medical staff as well as our VP MA for the campus and the Chief Medical Officer. Um And so we gave them a number of opportunities described in a two page chart note about what to do and how to respond. Um we, we were called but when a cardiac anesthesiologist had converted over to general anesthesia was managing the case and had just given Esmolol because he thought sinus tachycardia was bad in this particular case. And rather than actually maintaining, you know, forward flow. And uh so two attendings went to the or from the PH group and managed the case in the or until it was completed. So that was our solution. So, uh there was some effort at interdisciplinary uh work but it needs that effort, needs more work. You know, I know this is gonna be kind of out of the, the uh kind of off the beaten path about this. And we can talk about pressures and fluids and um blood loss and all that stuff and, and he the hemodynamics. But, you know, I think more and more with difficult cases like that, you know, the idea that somebody like this could very easily crashed to um ECMO is, is something that I've seen more and more, not so much where I am now, but just in other places is, is, you know, preoperatively getting uh ready for it. And I think um that the um the I or even putting someone, I think at another hospital, I was gonna be evaluating a lung transplant candidate who had PH and um she was doing poorly, but they wanted a tr her and they actually just put her on ECMO, did the tr and took her off ECMO because she didn't need it. But, I mean, I know it sounds weird but you have to think of everything that you could do to get the patient through and if it's like an inter operative eco situation, just like you would do for other surgeries and then you take them up, it could be something in the future that we'd ever, ever think about. Not something that'd be first thing on your mind. But I know that was just a thought. So I think we have to think about people in the or as the potential for acute right heart failure, right? They have chronic right heart failure and we have to address all the same things we do in the IC U. But in that very rapid succession, we gotta make sure we keep their inotropy. We uh and their chrono tropy, we can't go get them too slow. We can't get their white heart heart too weak. We can't get them too much fluid, we can't give them anything that negatively affects those two things and we can't give them anything that increases their RB afterload. And if people can't understand that part of the physiology really, they shouldn't be doing their cases. And that sounds really harsh. But I think that if you, if you're in that situation, you may have to say to the patient, I don't think you should proceed. Not here. Yeah. Yeah. Yeah. And so I, I think that's the hard part where you have to put up some guardrails and, um, try to guide them. But the problem is, is sometimes the patient will be in the, or before you even get the notification. And then how do you guide it? Like you did, you might have to go to the, or you might have to call a cardiac anesthesiologist that, you know, and say, hey, we've got a bit of a crisis I have one that I work with and she, I tell her every case is gonna go to the or so she can be watching them and make sure they get the right person assigned to them. But still things seek, sneak through, but that's my two cents. Yeah, I mean, like in this case here, you have a patient already with a dysfunctional RV and PH and uh you think, you know, for someone who doesn't understand why that patient might be tachycardic, right? Uh trying to maintain their cardiac out, but maybe it's driven a little bit by ep but you take away this drive for cardiac output by polishing them a beta blocker. And then you, uh and then you hammer them with a bunch of fluid uh uncoupling the, the RV and you give them a bunch of afterload and it just is a, you know, a, a triad of, of things that occurred to, to just really push that RV um over the edge. Our, our, our approach has been, you know, cardiac anesthesia to be involved for, for all these cases and then to always have sort of the ECMO doc on call that day aware of what's going on. We tend to do these, any of our cases in patients on pros noid specifically um in our um in our cardiac uh ors as well. So we know that the emo team has to mobilize to, to, to intervene, that they're, they're literally just down the hall um in those circumstances. OK. Um Sam, why don't you go, how did you rescue the patient? Uh The elected rescue was a combination of increased epinephrine diuretics and um rapid conclusion of the surgery would transfer back to the IC U and for the sake of time, I'll just go through this pretty quick. Uh You guys already touched on most of it. Um Not much literature as far as trials go, most of its expert opinion and case reports around intraoperative uh management of ph um A few cases showed continuous vasopressin before, during and after have helped um throughout the case to maintain pressures. Um And also the importance of having va eo on standby. Um When these patients go into the or um as far as overarching hemodynamic goals, this is a pretty much just a summary slide. Um You wanna avoid systemic hypotension to ensure coronary perfusion, prevent RV ischemia. You wanna keep your map 20 above your mean pulmonary pressure, maintaining sinus rhythm is very important. As we talked about beta blockers are poorly tolerated. Um avoid other factors known to increase your pulmonary vascular resistance, uh which can include epoxy hypercarbia acidosis pain, uh avoid high airway pressures and peep on the ventilator uh maintain a strict uh volume status and um maintain your ph therapies throughout. And that is pretty much it. And I'll conclude with that these references. And thanks, thanks very much and I thank our fellows for their great presentations and thank the faculty for all of their uh fantastic input. Published November 28, 2023 Created by Related Presenters Matthew D. Bernens, M.D. Sentara Pulmonary Critical Care and Sleep Specialists View full profile
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