Good morning, everyone. My name is Rakesh Subramanian, I'm one of the pulmonologists. I think most of us are here. Welcome to Curious in Case Report oral presentation. I think we can get started. Spencer. Hello, everyone. I'm Spencer Mullen. And my case is pulmonary hypertension of the carcinoid heart, a fleeting affair, or enduring relationship. These are my co-authors, Dr. Johnson, Dr. Summers, and Dr. Eggert, and I have nothing to disclose. So the learning objectives for this talk are looking at the relationship between carcinoid valvular disease and pulmonary hypertension, and situations where your echo estimations might not be accurate, and then balloon valvuloplasty as a bridge to definitive management. Two important kind of relationships that I think are important in this case are your right-sided valvular regurg and its relation to pulmonary hypertension, and then whether or not primary valvular disease is related to your measured pulmonary hypertension in different situations. So this patient is a 43-year-old female with a history of stage four ileocecal neuroendocrine tumor, with METs to several different locations. Here's a slide of her pathology. She was diagnosed with elevated serotonin, chromogranin A, back in 2020, and she has been treated with long-acting octreotide and tryptophan hydroxylase inhibitor. She was hospitalized with syncope and presyncope symptoms. And so carcinoid disease causes several issues that affect the right side of the heart, some of them being elevated serotonin levels in the pulmonary circulation, which lead to vasospasm and pre-capillary pulmonary hypertension, also fibrous plaques that deposit within the pulmonary arteries, and then specifically for valvular involvement, causing both stenosis and regurgitation. And it can involve both tricuspid and pulmonic valves, and causes deposition of fibrous plaques in the actual valves as well. All the details of the mechanisms aren't completely understood, but we know that it's secondary to damage by circulating vasoactive substances. So our echocardiogram for this patient showed an EF of 67% and an enlarged RV with normal systolic function, and both tricuspid and pulmonic regurgitation, and a PASP in the 60s. So the intervention that she received was a pulmonic valvuloplasty, and that was performed using this 2440 VITA balloon with a transpulmonary gradient of nine, a PAP of 25, and a PVR of two after the procedure. So this was a bridge to definitive intervention at a specialized carcinoid center for valve replacement. So here's a diagram of the before and after measurements, and the red arrow indicates where the valvuloplasty was done. And you can see that the measured pulmonary artery pressure was 35 over 20, and that's significantly different than what was seen on the echo. And then after valvuloplasty, we have an improvement in the RV from 60 over 25 to 30 over 15. Here's a before and after waveform of the right heart cath. It kind of more clearly shows the area under the curve and the big decrease in pressure. So then subsequently, this patient was treated with bivalvular surgical intervention at a specialized center, and continued to be treated medically with octreotide and the tryptophan hydroxylase inhibitors. So cardiac carcinoid disease, like I mentioned earlier, usually spares the left heart, except in cases where you have metastatic spread or involvement of the actual cardiac tissue with the spread of the metastatic disease. There's several other infrequent cardiac manifestations, including coronary artery disease, arrhythmias, and direct spread. So in this situation, I think is a very good example of where the modified Bernoulli isn't gonna be accurate. The PASP was estimated at 65 on the echo, but then we saw a PAP of 35 on the actual right heart cath. And so in a situation where you have a low flow, low gradient, and a torrential regurge, your Bernoulli is gonna overestimate your pressure. So I think this is an important example of when to think about, your numbers on your echo are not gonna be accurate. So your true PA pressure may or may not actually be elevated. And again, the effects of carcinoid disease are not just valvular on the right side. You also have a direct pre-capillary increase in the pulmonary pressures from carcinoid and fibrous plaques in the PA wall. These are my references. Thank you. Hi, everyone. My name is Daniel Strick, and I'm a clinical research coordinator at Tufts Medical Center in Boston. I'm here today to present an unusual case of pulmonary hypertension associated with T-cell LGL leukemia. And I have no financial disclosures. Our patient initially presented at 76 years old with a history of LGL leukemia that was diagnosed 10 years earlier, which was indolent and untreated, as well as osteoarthritis of the knees. She presented to her primary care physician with increasing shortness of breath and was referred to a pulmonologist where she had an echocardiogram, which showed that her right ventricle was enlarged and had reduced function, and her estimated pulmonary arterial systolic pressure was 74. She was then referred to a pulmonary hypertension center where all known causes of groups one through four pH were ruled out, and she underwent a right heart catheterization. The right heart cath revealed severe pre-capillary pulmonary hypertension as assessed by a mean pulmonary arterial pressure of 50 and an elevated pulmonary vascular resistance of eight and a half wood units. She was a functional class three, and she desaturated on her six-minute walk test without the use of supplemental oxygen. She also had an elevated BMP, white blood counts, and LDH, and a decline in thrombocytes. Since all known causes of groups one through four pH were ruled out, she was classified as group five pulmonary hypertension related to her hematological malignancy. According to the most recent European Respiratory Society guidelines, there are no approved treatments for group five pulmonary hypertension, and instead, treatment should be directed at the underlying condition, in this case, leukemia. However, if symptoms in these patients persist and hemodynamics remain elevated, clinicians may consider empiric therapy with pulmonary vasodilators, even though none are currently approved. So this patient was given two liters of supplemental oxygen for her hypoxia, cyclophosphamide for her leukemia, and Tadalafil and Masatentin for her pulmonary hypertension. Red Heart Cath, one year later, showed a significant improvement in her hemodynamics, as assessed by a decrease in her mean pulmonary arterial pressure and a decrease in her pulmonary vascular resistance. Her functional class improved from a three to a two. She was able to complete her six-minute walk test without desaturating and without the use of supplemental oxygen, and her BNP dropped substantially. She also had a decline in white blood counts. As a result, she no longer needed supplemental oxygen. She completed her planned 18-month treatment of cyclophosphamide six months later, and remains only on Tadalafil and Masatentin. However, about two and a half years after stopping cyclophosphamide, she again presented with worsening shortness of breath. Surveillance Red Heart Cath was performed, which showed an increase in her mean pulmonary arterial pressure and an increase in pulmonary vascular resistance. Her functional class went back from a two up to a three, and again, she desaturated on her six-minute walk test. Her leukemia, however, remained clinically stable, even two and a half years after stopping cyclophosphamide. So she was restarted on supplemental oxygen for her recurrent hypoxia. Decisions made to keep her off of chemotherapy since that was deemed to be stable, and the bulk of her issues were now from purely a cardiopulmonary standpoint. So instead, Selexipag was added on top of Tadalafil and Masatentin. We don't have any more Red Heart Cath data after Selexipag was added, but we do have several non-invasive parameters to show that the patient improved and achieved a sustained improvement after adding Selexipag. So two years after starting Selexipag, her functional class went back down to a two, and she was able to complete a six-minute walk test without desaturating. She was taken off of oxygen, and now at our most recent visit is nine years after diagnosis and five years after starting Selexipag, and the patient remains clinically stable on triple PAH therapy without the need for supplemental oxygen or chemotherapy. And an echocardiogram reveals that her right ventricle is both normal in size and function for the first time since her diagnosis. So in conclusion, it appears that triple PAH therapy appeared to have long-term benefits in this very rare case of PAH, which raises the possibility of a shared pathophysiology between group five pulmonary hypertension associated with hematological malignancies and group one pulmonary arterial hypertension. However, it still remains unclear if pulmonary vasodilators alone can improve pulmonary hypertension in this case or improve hemodynamics in this case. As the patient started her pulmonary vasodilators at the same time as her cyclophosphamide, which was also believed to improve her hemodynamics, and then after the patient started Selexipag, we don't have any more hemodynamic data to follow up with. And lastly, the effects of pulmonary vasodilators in this type of patient has never been formally studied, as she has a very rare form of leukemia, which is a part of an already rare pulmonary disease, which makes clinical trials virtually impossible in this patient group. But our patient appeared to have a substantial benefit to this treatment, so it's important to present these cases and publish these cases, so clinicians around the world at least have some literature to rely on when they face these difficult and unusual patients. Thank you. All right, so I'm gonna be talking about our case series on high-altitude pulmonary hypertension at moderate altitudes. My name is Keith Albrexton. I'm a third-year fellow at the University of New Mexico, and I have nothing to disclose. Our learning objectives today, so we'll start by reviewing a little bit about high-altitude pH and what it is. We'll also talk a little bit about pH in New Mexico, and then we'll finish by reviewing our series of seven patients who we suspect have high-altitude pH. So starting off with some background on high-altitude pH, so this is a condition that's both felt to be caused by and characterized by chronic or intermittent exposure to hypobaric hypoxia at high altitude, and high altitude has been traditionally defined as altitudes above 2,500 meters. For those of us that are metrically challenged, that's about 8,200 feet. I think it's worth pointing out that this does seem to be a fairly arbitrary altitude cutoff, and we know that patients can experience the effects of hypobaric hypoxia below altitudes of 2,500 meters. Some other important points about this condition, it's been defined hemodynamically as a mean pulmonary artery pressure of greater than 30 millimeters of mercury, or a pulmonary artery systolic pressure of greater than 50, and this comes from the 2005 expert consensus, where basically a group of altitude-related experts got together and defined these different altitude-related disorders. Some other key points about this condition, so this is a diagnosis of exclusion, so you obviously have to do a thorough job of ruling out other causes of pH in these patients, and indeed we did that in our case series here, and another point is that these patients should not have excessive erythrocytosis. That is suggestive of another chronic altitude-related disorder known as chronic mountain sickness or Monge's disease, and that can cause pH, but these are felt to be two separate clinical entities. And then the final point I would make about high-altitude pH is that it's grouped under WHO Group 3 with our other chronic lung diseases, and so as of now, there's no approved treatments for this condition other than chronic oxygen therapy or suggesting that the patient move to lower altitudes, which is often not a realistic option for our patients. Moving on to a little bit about pH in New Mexico, so we do think that we have a higher incidence and prevalence of pH in New Mexico compared to the rest of the United States. Our group in New Mexico published some data in 2020 around this topic, and there's a couple of possible explanations for this. One is our relatively high incidence of methamphetamine use, although it's probably not quite as high as you would think with our Breaking Bad associations. The other possible explanation is the fact that Albuquerque, where the University of New Mexico Medical Center is, and the surrounding areas sit at an elevation of what most people would consider a moderate altitudes of 1,500 to 2,500 meters, and our cath lab at the University of New Mexico sits at about a little bit above 1,500 meters, and the average altitude in the state of New Mexico is a little over 1,500 meters. And so one of the key sort of hypotheses underpinning this case series is that, could our patients being exposed to this chronic hypobaric hypoxia at moderate altitudes, could it be contributing to their pH, and could it even be driving the underlying pH in some of our patients? And I don't think this small case series proves that hypothesis at all, but it maybe supports it a little bit. So moving on to our cohort specifically, so we were able to identify seven patients between 2016 and 2022 who presented to our pH clinic at UNM Medical Center for various reasons. This table on the right here summarizes some key demographic factors, functional class of presentation, altitude of residence, as well as relevant comorbidities, and one really important point I wanna make about our case series, and I think this is a key limitation of this series, is that we did use more, you know, quote, unquote, modern hemodynamic definitions to diagnose these patients with pH, as opposed to the mean pH pressure cutoff of 30 that's suggested by the expert consensus guidelines from 2005, so I think we had three patients whose mean pulmonary artery pressures at the time of diagnosis was in the 25 to 30 range, rather than over 30. It's also worth pointing out that our cath lab is at 1,500 meters, as I mentioned, and many of the patients who we suspect have this condition live at altitudes higher than 1,500 meters, and so, as you can imagine, it's logistically difficult to do right heart caths at the exact altitude that these patients live at. And then, obviously, we retrospectively went back and looked at relevant demographic, hemodynamic, and laboratory data on these patients, and we'll get into that next, so. If I had to describe our cohort, I would describe them as a relatively elderly male cohort, which is sort of a unique phenotype. Our mean age was about 68 years, and six of our seven patients were male. Another important and interesting thing that I want to point out about our cohort is all of them had concurrent sleep disordered breathing, whether that was central sleep apnea, obstructive sleep apnea, or nocturnal hypoxia. And that is interestingly consistent with the existing data on high altitude pulmonary hypertension. Both advanced age and sleep disordered breathing have been independently associated with this condition. So it was nice to see that our cohort sort of goes along with what's been seen in the past on this. And another interesting point about these seven patients is nearly all of them described varying degrees of intolerance to altitude in the past, including one gentleman who had developed high altitude pulmonary edema when traveling to Everest base camp. Another gentleman who developed hypoxia and exertional dyspnea whenever he would go skiing in Santa Fe. And another gentleman who would get exertional hypoxia and dyspnea whenever he would go elk hunting in the mountains around New Mexico. This is sort of an ugly table that summarizes the relevant hemodynamic echocardiogram, NT prone P and six minute walk test data for all of our patients. And as you can also see, we did elect to treat all seven of these patients with a varying combinations of vasodilators. So you can see three patients got single oral therapy. We had three with dual oral therapy and then a single patient who got triple therapy. And I think this is sort of the big takeaway from this small case series is that we elected to treat all these patients and we saw what seemed like pretty good responses to treatment. So all seven patients had improvement in their six minute walk tests and NT pro B and P. And also importantly, none of them experienced any adverse effects. And so again, I think this is the biggest takeaway from this small case series is that these patients potentially could benefit from oral vasodilator therapy unlike our other group three pH patients. So wrapping things up, as I mentioned multiple times, we do know that patients can experience the effects of hypobaric hypoxia below altitudes of 2,500 meters. So it stands to reason that some patients may develop pulmonary hypertension as a result of this or at least altitude could be contributing. Again, our cohort is consistent with other cohorts that have explored this condition and that both sleep disordered breathing and advanced age were seen in all of our patients. And then finally, all seven of our patients responded well to oral vasodilator therapy without any adverse effects suggesting that there may be a role for oral vasodilator therapy in the future. Yeah, we have a lot to learn about this condition still and I think further research is warranted. That's all I got. Thank you. Good morning. My name is Caitlin Batzoff. I'm one of the fellows at Mayo Clinic in Rochester and I'll be presenting Hidden in Plain Sight, a Case of Pulmonary Venous Occlusive Disease. That's my face with much longer hair. So brief roadmap, we'll have an introduction to PVOD, our case presentation discussion and then some learning points. So first, pulmonary venous occlusive disease. This is a rare subgroup of pulmonary arterial hypertension and it's primarily characterized by post-capillary vascular lesions. However, you can have all components affected of the vascular bed, just something to be mindful of. When you're thinking about the venous involvement, it's typically intimal fibrosis and then narrowing that happens subsequently. And then three to 12% of cases that are initially described as idiopathic pH are actually then changed to this pulmonary venous occlusive disease. So as we think about prevalence, we have one to two cases per million and then incidence 0.1 to 0.5 cases per million. So you might be thinking, why are we talking about this rare disease? But ultimately, PVOD carries a poor prognosis and so there's a one-year mortality quoted as high as 72%. And then interestingly, patients at time to diagnosis to either death or lung transplant, the average is about 12 months and then their symptom onset to either death or lung transplant is about 24 months in a timeline. So as we go into our case, this is a 64-year-old woman that presents to our interstitial lung disease clinic in July of 2022. Brief background, developed a cough scant hemoptysis in dyspnea in 2015. A CT chest at that time demonstrated some kind of septal thickening ground glass and got a wide net in the radiology report outside of pulmonary edema versus infection versus DAH. The patient was treated for a community-acquired pneumonia, had INCA vascularity serologies that were negative and had a follow-up CT scan that was improved but not resolved. And importantly, she felt mildly better. So it was kind of just pushed off towards an infectious etiology at that time. Unfortunately, her symptoms progressed. So she presented to an outside pulmonary visit on 2017 of July. Her CT scan at that time had some nodularity, some mediastinal lymphadenopathy. So they were thinking more related to potentially sarcoidosis. Underwent bronchoalveolar lavage that was unrevealing. And additionally, a right heart cath given this concern on CT of PA widening. And so she was diagnosed subsequently with presumed kind of sarcoidosis, interstitial lung disease and WHO Group 3 pulmonary hypertension. Unfortunately, she had progression of her disease process and developed chronic hypoxemic respiratory failure in 2018, was put on three liters nasal cannula. And then subsequently in 2019, underwent a right lung wedge resection and was diagnosed with respiratory bronchiolitis associated ILD. Specifically, the pathology showed organizing pneumonia, pigmented macrophages in a bronchiocentric pattern. And then she had some thickening of her pulmonary arteries that they were thinking might be consistent with healed thromboembolic disease. She was started on glucocorticoids, transitioned to rituximab infusions, felt kind of mildly better and then got worse again. And this is why we're seeing her in clinic. So at her time of presentation, she had progressive dyspnea with an MMRC of three, kind of a WHO functional class three and was on six liters nasal cannula. In thinking about other exposure, so she had prior marijuana smoking, but had been abstinent for about two years, no tobacco exposure. She did have pets in the home and then a wood burning stove and then a family history of autoimmunity with a sister with Sjogren's, a niece with rheumatoid arthritis. This kind of prompted additional lab workup, which showed essentially negative autoimmune panel aside from mild elevation in her rheumatoid factor, a negative incavasculitis panel and HP panel. She had PFTs that showed a mild reduction in FEV1 and a CT chest, which I'll show two representative cuts for. So the CT chest essentially demonstrates mosaic attenuation. It's worse from her prior images. And she also has some air trapping as well as some mediastinal lymphadenopathy, which I recognize is more challenging to see in these windows. Additionally, we repeated an echocardiogram that showed normal right ventricular size, but a mildly reduced systolic function with an RVSP estimated at 87 millimeters of mercury. And then we additionally got a VQ scan with this question of kind of healed thromboembolic disease and pathology. That represented small, numerous perfusion defects, bilateral lungs that did not have a matching ventilation abnormality. So ultimately pursued right heart cath. And here you can see her pulmonary artery pressures elevated 46 and 44 post vasodilator therapy with a wedge of 15. And then her pulmonary vascular resistance did improve mildly with nitric oxide administration. And so ultimately kind of taking this into a wide scope view and kind of thinking about, you know, is this sarcoid? Is this RBILD? Is this something else? And looking at her pathology again, it was felt that this was actually more consistent with pulmonary veno-occlusive disease and that she had patchy organizing alveolar hemorrhage instead of this kind of pigmented macrophages associated with RBILD. And then that this image was actually a vein and not an artery and was kind of miscalled. And so briefly to talk about pulmonary veno-occlusive disease, very difficult to identify, often misdiagnosed. And I think that's related to the overlapping symptoms that we see in pulmonary hypertension. So you have this insidious onset of fatigue, breathlessness that progresses to right heart failure and end stage disease by the time we're capturing these patients. And while hemodynamic measures can't differentiate PVOD from PAH, things that should increase your suspicion would be tobacco exposure, chemotherapy, organic solvent exposure, and then connective tissue disease, specifically systemic sclerosis. And your BAL can be helpful if you see a cult alveolar hemorrhage. And then finally, the triad that you're looking for on CT chest is central lobular ground glass, septal thickening, and mediastinal lymphadenopathy. As we think about treatment, this is quite limited because standard PAH therapies can actually worsen patients with pulmonary veno-occlusive disease. So specifically that vasodilator can lead to pulmonary hemorrhage, or excuse me, pulmonary edema. That's life-threatening. And so we think about supportive care and then ultimately lung transplant as definitive therapy. And so primarily, I would say early referral to lung transplant evaluation is key because you can have pretty rapid progression in PVOD patients. And we see that if you compare the risk of removal of lung transplant waiting list from PVOD patients to PAH due to death, there's actually a hazard ratio of 2.05 if you compare PVOD to PAH patients. So in conclusion, PVOD is a rare subgroup of PAH that remains under-recognized, and it's primarily due to similarities in clinical presentation. And so we must remain diligent and consider this rare diagnosis when we evaluate our PAH patients. And we have to think about the deviations in treatment from standard therapies, specifically because you can get that pulmonary edema associated with a vasodilator attempt. Here's my reference. Thank you, and questions. Happy to talk about her wrap-up too. Thank you. Aloha, everyone. My name is Lisa Pham. I'm a second-year pulmonary critical care at Creighton. I have no financial disclosures, and I think my case will be a nice follow-up to Kaitlyn's case. I'll talk about a case of PVOD and its suspected inciting pathology, and I'll also talk about diagnosis as well. So we'll begin with a 28-year-old male who came into the ED complaining of shortness of breath. He also had dyspnea on exertion. He had a past medical history that was significant for DVT in the setting of factor V Leiden and activated protein C resistance. He also had a history of metastinal lymphadenopathy. His social history was significant for former tobacco use, as well as vaping use and marijuana use. When he came into the ED, he required three liters of nasal cannula. His CT chest with the pulmonary embolism protocol showed that he had bilateral pleurifusions, right greater than left. He had a septal interlobular thickening, as well as extensive alveolar infiltrates. He had a new high-alert lymphadenopathy, and radiographically, it was shown that he had pulmonary hypertension, and his TTE was unremarkable. So we performed a diagnostic and therapeutic thoracentesis on the right. It showed a transdated pleurifusion that was lymphocytic predominant. And after that thora, it resolved his hypoxia and his symptoms, so we sent him home. He returned to the hospital a month or so later with reaccumulation of that right pleurifusion. We did a bronchoscopy, which showed inflamed airways, as you can see photographed. His BAL was about one third neutrophils, lymphocytes, macrophages, respectively, and his eosinophils was about 9%. His lymph node biopsies and his transbronchial biopsies were just, it was unrevealing. We did infectious workup in the autoimmune battery, including like onca, and that was all negative. The working diagnosis was organizing pneumonia, and of course, they were considering E. voli in him, because at the time, he was still vaping. And he was discharged on a prolonged prednisone taper with good effect. Unfortunately, he would keep coming back to the hospital, so on average, one admission per month. He did have a transient reduction in his systolic LV function at 45%, but that would recover. We did try diuresis as well, with mild improvement in his hypoxia. Notably, he had peripheral eosinophilia. It peaked at 1.4. On initial admission, it was 0.6. And he had an elevated IgE level, around 300, and we noted that his CRP and his SED rate was overall uptrending throughout his admissions. So our diagnosis did shift over to non-asthmatic eosinophilic granulomatosis with polyangiitis, or eGPA. Rheumatology was tapering down his steroids with plans to start rituximab. However, it was ultimately just held all together because we pursued a wedge biopsy. So with that biopsy result, it showed granulomatous vasculitis concerning for eGPA, though we did send the path to a different institution, and the read came back as PVOD with pulmonary capillary hemangiomatosis and focal vasculitis with granulomas. After that diagnosis, he did have a right heart path that showed pre-capillary hemodynamic pattern, and he was started on sidenafil with improvement of his dyspnea on exertion. We tested him for the genetic mutations that are seen in PVOD, which came back negative. So I won't belabor PVOD just because Caitlin did a great job, but I just wanted to add that PVOD and pulmonary capillary hemangiomatosis are now considered the same disease entity. And the pathophysiology is elusive and likely multifactorial, so you'll have an inciting event that causes endothelial injury that promotes fibrosis of the pulmonary veins and venules. An example is tobacco use, like in our patient, since PVOD has been associated with higher tobacco exposure when compared to pulmonary arterial hypertension. There was a case study in the 80s of a 21-year-old who was a marijuana smoker who had PVOD, and his pathology had shown granulomatous venulitis. So with the diagnosis of PVOD, there's been a paradigm shift from surgical biopsy to clinical evidence, as well as exam findings, plus or minus bronchoscopy. On bronchoscopy, Caitlin hadn't mentioned like the hemocitarin-laden macrophages, but you can also see, like in the picture on the previous slide, that you can have hyperEMIG airways in your lobar and segmental bronchi, because it drains into the pulmonary veins. And you can also see bright red longitudinal streaking as well, and that's secondary to vascular congestion. And then she also, Caitlin also talked about radiographic features. So you're looking for evidence of pulmonary hypertension. You're looking for signs of post-capillary venous congestion, such as the smooth septal thickening, pleural effusions, pericardial effusions, et cetera. And then mediastinal lymphonopathy as well. That can be explained by veno-lymphatic shunts and circulating angiogenic factors. So to go back to our case of our 28-year-old male who initially came with shortness of breath, dyspnea on exertion, and found to have PVOD, because of his peripheral eosinophilia, his elevated IgE levels, his pulmonary manifestation, and marked response to systemic steroids, we postulate that he has non-asthmatic eGPA that led to PVOD. However, it does complicate the picture that he smoked tobacco and he used marijuana as well. So he was undergoing pulmonary rehab. He was on a baseline oxygen of three liters. He was on his pH medication. And about a year since we first met him, he went up to eight liters of nasal cannula. And fortunately, around that time, he received his double lung transplantation. He's doing really well. He's on Rumeir, and he's on prednisone as well as the other immunosuppressants and other drugs. So this was a slide on general management of PVOD that he was getting. And in summation, while avoiding invasive biopsy is ideal in this very high-risk population, lung biopsy offered a definitive diagnosis in our case, but it should be noted that we're moving away from biopsy, and that can be replaced by a constellation of symptoms, clinical symptoms, the signs of venous congestion on CT in the absence of left heart disease, signs of pulmonary hypertension on CT, and pre-capillary hemodynamics. And our patient had all of those constellations. It's just the right heart cath came a bit later in his course. So, thank you. God bless. Thank you.

case reports

pulmonary hypertension

carcinoid valvular disease

pulmonic valvuloplasty

T-cell LGL leukemia

high-altitude pulmonary hypertension

pulmonary veno-occlusive disease

PH