 disease, but there's enough cases out there that this procedure should be more widely applied. Now, there's a progression from acute PE to chronic thromboembolic from the hypertension. We know that emboli that become persistent can entrap circulating stem cells. There can be misguided proliferation of cells, which lead to vascular remodeling and chronic obstruction resulting in the ravages of pulmonary hypertension. This is our deep venous thrombus. This is often a presentation where we see a CT scan that show a patient with large burden of emboli in the pulmonary arteries. If we follow these patients further up, we can do further tests. This is a ventilation perfusion scan, which reveals absence of perfusion to the right lung with normal ventilation and large perfusion defects also in the left lower lobe. A dual energy CT scan with iodine mapping can also give very similar information. You can see the absence of perfusion in the right lung here with fairly normal perfusion, some defects in the lower lobe still. A new modality that's become available to us. If we perform pulmonary thromboendular rectomy surgery, we can remove a cast of the thrombus that looks like this and open up the pulmonary arteries re-establishing flow into the lung, which fortunately has a dual blood supply and therefore can participate once more in gas exchange. This is our case again that we showed earlier on. You can see the absence of perfusion. This is shortly after the surgery and now you can see there's perfusion in the right lung after removing this thrombus. Here is the complete obstruction, some occlusions in the lower lobe here, and that matches our specimen that we've removed. We know that approximately 95% of acute PEs would resolve and you will not have any hemodynamic compromise. But there are factors, genetic or intrinsic variables and patients that also approach thrombotic tendencies or recurrent pulmonary embolies that can lead to the development of small vessel disease and progressive more small vessel disease and leading to chronic thromboembolic pulmonary hypertension. We know that there are certain comorbid states that predisposes patients more to develop this. We know that the odds ratio for malignancy, antiphospholipid syndrome, for previous history of venous thromboembolism, or thyroid hormone replacement, splenectomy and infected pacemaker leads or VA shunts has a significant increase in the risk for developing chronic thromboembolic pulmonary hypertension. We see that the age distribution is a little bit different to that of primary pulmonary hypertension, which normally occurs in the second, third, fourth decade. Chronic thromboembolic disease is seen more commonly in the fifth and the sixth decade. Although we do have patients from as young as five years old, up to our oldest patient was 87. So if we look at epidemiologic studies, we can assume, and this is using data from US and Italy, that there's approximately 200 cases of acute pulmonary emboli that is definitely confirmed as large acute pulmonary emboli. And if you take a range of around one to four percent developing chronic thromboembolic pulmonary hypertension, we know that we should be doing approximately 1,600 to even as high as 7,000 cases per year in the United States. But currently, there's only about 300 PTE surgeries being performed in the United States. So it's widely under-diagnosed, under-recognized, and surgery is not often frequently enough. What is the natural history of chronic thromboembolic pulmonary hypertension? So also from epidemiologic studies, we know that the higher the mean pulmonary artery pressure, the worse the outcome. And if we have somebody with the mean pulmonary artery pressure of 50, we know that the two-year survival is less than 20 percent. So it's a very morbid disease, like heart failure and more morbid than many of the malignancies. On the right-hand pane is a study that was done in 2001 looking at two populations. One, there was 54 total patients. Four patients were offered pulmonary thromboenaderectomy surgery, and the rest were just followed. And you can see how drastically the survival is influenced by doing pulmonary thromboenaderectomy surgery versus just leaving the patient trying medical therapy. We have a good solution. How would these patients clinically present to us? Typically with progressive dyspnea, chronic cough, they might experience chest pain when they exert themselves, climbing up a flight of stairs, complaining of chest pain. Lower extremity edema is usually when they've already developed right heart failure. Pre-syncope and syncope, this is an obstructive lesion, limits the cardiac outflow. If a patient has a demand increase and he cannot meet this demand increase, they can syncopize. Hemoptysis is seen frequently as the patient develop bronchial collaterals. These are small, thin vessels, high-pressure arteries that can rupture and bleed, lead to hemoptysis. And it's important to remember that there's no history of DVT or PE in up to 30% of our patients. If you examine them, you'll find that they have an increased P2 sound. There will be an RV tap and a lift. You can get a narrow fixed splitting of the second heart sound. That regurged murmur can be heard when they develop significant pulmonary hypertension. And elevated JVD, hepatomegaly is seen, a cytos can be seen and peripheral edema. And the unique thing is if you listen over the lung fields, you can often hear the bruit of the turbulent flow going through the obstructions left by the chronic clot. In the differential diagnosis, there are a variety of things that we have to consider, primary arterial pulmonary hypertension, then also COPD. And these can also mimic CTF, developing inside to thrombus. And I'll show a couple of slides about this. Fibrosin mediastonitis, pulmonary venous occlusive disease, more commonly seen after EP procedures for pulmonary vein isolation can cause obstruction or fibrosis that leads to pulmonary venous occlusive disease. Pulmonary vein stenosis, congenital extrinsic vascular compression, sarcoidosis is one of the diseases that can compress large lymph nodes that can compress the pulmonary arteries. Large vessel vasculitis is another differential diagnosis that has to be considered. Congenital branch stenosis and then pulmonary artery sarcoma can mimic chronic thromboembolic disease very well. And they also have a similar surgery we performed in our rectomy for the sarcoma patients as well. Here's an example of a vasculitis case and it would look as if maybe there's chronic clot with a little bit of recanalization, but you can see these vessels are long and narrow and diffusely diseased and this patient actually had a vasculitis and had vasculitis in other organs as well. If you do a CT scan of the aorta, this patient had a aortic vasculitis as well. So the guidelines for our diagnostic workup, we have these symptoms, we develop a suspicion, we examine the patient and we think, well maybe the patient has pulmonary hypertension. We do an echocardiogram that we test and see if there's evidence of pulmonary hypertension, right ventricular enlargement. And then we would do a next test and typically after the echocardiogram it would be a VQ scan, would be a good one to do. Nowadays there's a lot of CT imaging being done before a VQ scan, it's just more freely available and there are distinct features on CT scans that can lead you to believe that you are dealing with chronic thromboembolic disease. If the VQ scan is completely stone-cold normal, then you can just rule out CTAR and workup for other causes of pulmonary arterial hypertension. If the VQ scan shows mismatched perfusion defects, you have to consider and contemplate this CTF. And you should refer the patient to a PH or a CTF center with expertise to work these patients up further. Typically in our institution the patient will undergo a right heart catheterization and a pulmonary angiogram or a CT pulmonary angiogram and where we can confirm the diagnosis. So here we have a completely normal perfusion scan, this is not CTF. This is the defects that we see and this patient should be worked up for CTF. Let's do a case study. So this is a 48-year-old woman, she comes to us in functional class 3, maybe going on to class 4, she has no history of DVT, she had an echo with findings of pulmonary hypertension and the right ventricular hypertrophy and you can see this on her chest x-ray as well, that she has enlarged pulmonary arteries and the right ventricular enlargement. She was started at an outside hospital with a trial of soldinophil, but she had no subjective improvement. Resting sinus tachycardia, she had an elevated JVD, right ventricular heave, she had a RS3 and her liver was at 6 centimeters with some edema, so a pretty sick patient. This is an echocardiogram and the outstanding features here is a very large right atrium and then large right ventricle, a D-shape left ventricle with pressure overload and then the velocity through the tricuspid valve indicating high pulmonary artery pressure or high right ventricular pressure. Received a VQ scan and here you can see multiple moth-eating like appearance, multiple perfusion defects with normal ventilation. In our hospital, she had a pulmonary angiogram, we use biplane pulmonary angiograms, so we have lateral and AP and here you can see there are definitely defects, filling defects, there are webs, there are abrupt cut-offs, there are hyperperfused lung where you can see that there's just much perfusion into the lung and then filling defects. Typically the disease is more in the right lung than in the left lung and it's typically more in the lower lobes than in the upper lobes. This is a CT scan of this patient which demonstrates mosaicism, so areas of hyperperfusion where there's obstructed blood flow within areas of normal perfusion. CT angiogram is capable of picking up some of the defects, there you can see a web that's in the pulmonary artery and some filling. A couple of things about CT that I want to make the statements to. One is the absence of proximal clot on the CT angiogram does not rule out surgical accessible CTF for the most part and the presence of central thrombus on a CT does not confirm the diagnosis of CTF either. Here we can see a patient with acute clot, very clearly visible the features of an acute pulmonary embolism and a year later there's a tiny little very subtle change that might be construed as even a branch point but this is the organized thrombus that becomes very subtle, so not so easy to diagnose chronic state with the CT scan. Another giveaway that you might be dealing with chronic thromboembolic disease is metastinal collaterals and here you can see around the airways the body is trying to just get blood flow to come into the lung and you get metastinal collaterals that is prominent. Also vessel asymmetry clearly there's more vessels on this side than on this side most very approximate obstruction to leave a pattern like this. This is a case of inside to thrombus and if you look here you can clearly see the clots here and but if you look at the VQ scan it's a completely normal VQ scan. If you take this patient to the operating room you won't make any change to the patient's condition you would just give them a sternotomy and potential risk for complications but there won't be any benefit here. This is an inside to thrombus that developed in a patient with arterial pulmonary hypertension. So this is the hemodynamics from our patient she had a right heart cath, the RI pressure is 26, pulmonary arterial pressure 90 over 35, mean of 57, wedge pressure 16, cardiac output 2.5 and the pulmonary vascular resistance of 1294 and pulmonary arterial saturation was 43%. There were multiple segmental level disease as we've already shown and now you have to ask yourself do the hemodynamics fit the angiography? Does the amount of pulmonary hypertension match the defects that you see? Because if they don't you might be dealing with arterial pulmonary hypertension on a capillary level that won't get better from a surgery. So what are the options for this patient here? We can just continue with medical therapy and I'm not going to go into too much detail but there are drugs approved for patients that are non-surgical candidates. You can do a surgical pulmonary endotherectomy and we'll talk a little bit about operability assessment and then balloon pulmonary angioplasty is a technique that's been used for many years and especially in Japan but nowadays being used more in the United States as well. It is an obstructive disease and it's kind of counterintuitive that you can use balloon pulmonary angioplasty but it definitely has a role to play especially in a more distal disease. And then there's even talk about maybe you can do pulmonary artery denervation but that won't really help for an obstructive disease. The operability discussion it's kind of difficult to address and every time we have a case we have a multidisciplinary team that meets and we discuss the case and it's impossible to just have general guidelines that we can put out there and put it for every center to just use this. But factors that would influence the decision is if there's a clear diagnosis of CTIV, if there's no clots then there's no surgery but if there's a clear diagnosis then you contemplate doing the surgery. Then you look as the comorbidities that will limit the patient's ability to gain benefit from the operation. Does he have severe COPD and you take out the clots he's still going to be limited. Most patients shouldn't do it. Is he so old and frail that he won't be able to rehabilitate after the surgery? Does he have severe kidney disorder? Is there other comorbidities? And then you have to look at the anatomy and the hemodynamic correlation. It has to match up how high the pulmonary artery is with how much obstruction you can relieve. And you also have to consider the surgical experience at your center. Do you have surgeons that are capable and familiar with doing very distal disease if you present a case with distal disease to them? And then the center's experience, both anesthesia, the critical care whether they can manage these patients afterwards. So inoperable at UCSD about 2-3% of cases that get referred to us we turn down for surgery. So not many patients get turned down. We don't think there's any degree of right heart failure that makes a patient inoperable. We will even take a patient on ECMO because of right heart failure to the operating room if we are sure that we have the right diagnosis that we have operable disease. We also don't think that there's a pulmonary hypertension grade that is so high that we would not take the patient. So generally if the etiology is thromboembolic we would offer operation. I do want to mention that preoperative pulmonary vascular resistance is a predictor of mortality and this is data from the article that the Paris group that showed that the higher the pulmonary artery pressures the higher the operative mortality. And clearly if you have patients over a thousand it's a fairly steep angle there on the curve. So this is our angiogram that we showed. This is the specimen that came out of the patient so this is level three so segmental level disease and there's multiple segments that were obstructed. And this is the postoperative VQ scan. Postoperative early postoperative there's a lot of changes but you can basically see some of the defects that have resolved and dark areas that are now bright and there's actually some steel phenomena that can happen in the early postoperative phase. Typically this VQ scan will become fairly homogeneous and normal looking after approximately three to six months. This is the postoperative echo you can see that the RV pressure is markedly down now and that the D shape of the left ventricle has resolved. So what are the rational for this operation? To restore perfusion to functional tissue. The tissue is functional because they have dual blood supply, they have bronchial circulation keeping the lung tissue alive so it's not necessarily fibrosic necrotic or scarred. The aim of the operation is threefold. First one is hemodynamic we want to relieve the right heart failure. And the second is alveolar respiratory we want to restore ventilation and gas exchange. And the next one is palliative we want to prevent the progressive right ventricular failure in development of small vessel disease. Similar to overflowing an area as a congenital case with a shunt the areas of the lung that is not obstructed is receiving a significant amount of flow and there's some evidence that there's small vessel changes happening in that unobstructed lung so you want to prevent that. The surgical principles as well established we do this operation through a median's anatomy on cardiopulmonary bypass with circulatory arrest and there's a lot of debate everybody always asks me why do you have to do circulatory arrest but once you do the operation you will know why you need to do it because blood just wants to get out and if you open a pulmonary artery that's the lowest point it's exposed to atmospheric pressure and blood is coming there in your field so you definitely do need circulatory arrest. This was definitively studied by the Papworth group and they basically did a randomized control trial between circulatory arrest and no circulatory arrest and the outcomes were better in the circulatory arrest both the neurologic neurocognitive testing as well as the reduction in pulmonary vascular resistance you have to typically do a bilateral endoderectomy it is very rare to get a disease that obstruction that is just unilateral almost always there's some emboli that goes into both lungs and you should at least inspect both lungs it's very important to identify the correct plane the plane is in the tunica media and it has to be identified to make a complete endoderectomy and you have to take all the tails out you cannot leave some of these tails because that will still leave obstructed so you follow each one of the tails all the way distal this is our surgical setup and the team did an excellent job yesterday they were doing this just like the book we have endotracheal tube large border endotracheal tube because you have to be able to use a diagnostic bronchoscope TE monitoring we use a pulmonary artery catheter the patient has a radial arterial line and there's a femoral arterial line there's bladder and rectal temperature probes as well as nasopharyngeal or tympanic temperature probes that we use during this operation because we're going to do deep hypothermic circulatory arrest for anesthesia is very important these patients can like a critical aortic stenosis patient on induction can very easily arrest because it's a flow limiting lesion so target hemodynamic stability it's based on the right ventricular function do not attempt to lower the pulmonary pulmonary vascular resistance you should attempt to ensure that you have adequate perfusion and contraction of the right ventricle so you use inotropes extensive pre-oxygenation of the patient so let him breathe oxygen for a long period of time even hyperventilate him then we use fentanyl medazolamate and various doses as muscle as well as muscle relaxant at the time of patient induction it's important to select patients if you have a really sick patient the surgical team should be in the room ready for going on urgently if the patient decompensates right so a couple of signs of an pending doom if you see on the preoperative cap that the diastolic pressure in the RV was more than 15 you should be very worried at the time of induction this is typically a patient that's going to fail or if you have severe tricuspid valve regurgitation preoperatively or if the pulmonary vascular resistance is above a thousand surgical team in the room inotropes typically dopamine epinephrine in line and running at the time of induction intraoperative TE we use it to look at the right heart chamber size the function you look for abnormal motion of the introventricular septum intracardiac shunts typically if there's a PFO we're going to try and close it because that shunting blood from right to left can cause hypoxemia identify any thrombus that might be in the right atrium in transit you should try and remove those as well we not infrequently see plots still attached to either the the tricuspid valve or even the by the IVC so one of our intraoperative echoes and you can see again massive right atrium right ventricle squishing the left atrium in the left ventricle moving away here but typically the same picture other important thing during anesthesia is to be setting up for your brain protection the brain is the index organ during circulatory arrest you should be monitoring this with near infrared spectrometry as well as EEG monitoring to make sure that you completely suppress the EEG prior to circulatory arrest we use this wrap cooling wrapping unit it's actually originally made for knees but works very well it circulates water at 4 degrees Celsius and keeps the the head very nicely cold you can do ice packs as well but this is the way we do it the perfusionist then has to use bypass solutions that is used for typically for circulatory arrest we use plasma light 25% albumin mannitol and bicarbonate heparin and solimedral in in the prime for the neuro protection we also for prophylaxis of seizures we use phenytoin intraoperatively and we also give propofol immediately prior to doing the circulatory arrest we administer 2.5 milligram per kilogram after completion of the endoderectomy when we start recirculating again we give methylprednisolone 500 milligrams as well as mannitol this is to try and stabilize the membranes and reduce the possibility for brain edema the cooling should be uniform you aiming for a body temperature so core bladder temperature of 20 degrees Celsius typically the brain or tympanic temperature is sitting around 16 degrees at that point in time cooling usually takes around an hour depending on body size we maintain a gradient of 10 degrees Celsius you don't try to cool too rapidly otherwise it won't be a uniform cooling and really talked about the propofol and the methylprednisolone we limit the deep hypothermic circulatory arrest to 20 minutes at a time if we do that and we reperfuse after the 20 minutes and then we can do multiple episodes of 20 minutes and we've been as far out as 120 minutes of total circulatory arrest with excellent outcomes no neurologic effect just make sure that you remember to turn the stop cocks off when you drain the patient and you don't entrain air and confirm that the head is cold that your EEG is flat and then you try and ventilate squeeze the blood out of the lungs prior to the surgeon starting the endoderectomy so this is our surgical setup here we are cannulated we have a high ascending and order cannulation we have bicable cannulus with the cannula coming up into the SVC this one goes down into the IVC we use a pulling jacket that we wrap around the heart and then we have a retractors modified cerebellar retractor that opens up the space between the aorta here and the superior vena cava and this is where you're going to approach the right pulmonary artery and you're going to make an incision in the middle of the right pulmonary artery after you cross clamped and you give del nido cardioplegia is what we use surgeon's view now you're looking inside the pulmonary artery and these are some of the features you see blood coming from the bronchial circulation coming up at your store sometimes there can be some more acute thrombus this is chronic thrombus and it layers it forms webs this is a lumen that should be open here but a thrombus has gone across it and got organized and scarred it down making two little orifice here this is where you would hear the flow murmur as well if you examine the patient then this is the development of the plane we have small instruments that we lift up a plane here and we're going to dissect get circumferential you pick in the media of the vessel it usually strips fairly easily it's a shiny purly white appearance and color if it gets too pink it means you're getting too close to the adpentisia to close to the lung tissue and that you can exit the vessel this is then looking into the pulmonary artery you have this thrombus here you are pushing the patient away from the thrombus and then you are opening up and you take each one of these branches all the way as deep as it go if there's a bifurcation you make sure that you take all of the tails of the bifurcation as you dissect deeper down this is the instruments that we use and they these double action forceps make the holding the clot much easier this is the aspirator dissected that we use to dissect and aspirate a little elevator to start the plane and then long instruments for sewing up the pulmonary artery again this is a video where we just showing this is earlier this week this is actually a patient from Texas that came to us looking inside the pulmonary artery this is the thrombus that we see and we're going to start the dissection plane use the elevator initially I was a little bit too deep so I'm finding the right plane getting in it you can see it strips fairly easily at this point in time and we're going to I'm cutting it off here I'm going to do the lower and the upper lobe separately I'm trying to get a circumferential specimen going and then work our way into the pulmonary arteries here each one of them individually and eventually you can uncork and pull the whole thrombus out of there going to be nice and unobstructed here we're working on the upper lobe and we this is a little bit of a dance that you have to do with your OR team here and I have a little video that will show it there the surgeon cannot take his head away he has to look right at the plane of dissection he will lose time if he looks around so the team is very important to put the instruments in his hand and that the communication should be smooth we only limited to 20 minutes at a time to be able to do the dissection here we're going into the left side now surgeon stands on the patient's right side looking into the left pulmonary artery you can see the thickening on the left side I often use a beaver blade and a knife to cut in the back part of the vessel to scrape until I get into the right plane and then developing it circumferentially we keep dissecting down another specimen removed we're looking inside and that's very normal appearing pulmonary artery so after the obstructions were relieved we had a very nice normal pulmonary artery when you then try to separate from bypass you have to rewarm to a normal thermia typically takes up to two hours warming takes much longer than than the cooling you go through your checklist typically we start dopamine infusion sometimes epinephrine we use temporary pacing wires typically we pace the patient around 80 to try and help the right ventricle your duty assessments and you check the endotracheal tube to see if there's any evidence of bleeding if there's throaty sputum or airway bleeding you have a problem and you're gonna have to deal with this complications that can happen reperfusion pulmonary edema up to 30% of cases airway bleeding it's not that common but if you have it it's the third most common cause of death for these patients hard to manage because you can have a lot of blood coming into the airway preventing the patient from oxygenating properly so Frank blood implies a mechanical violation here's a test it's called the bubble test and we were doing it yesterday in the operating room you basically give a valve salva while you're looking inside after you've gone on to bypass and you can clearly see air coming into the pulmonary artery so this patient has a break in the pulmonary artery you have to control that you try and dissect you can either dissect on the outside into the hyalum and see if you can occlude put a clip on that specific branch where the air is coming from see if that controls it sometimes you have to do an intraluminal obstruction of that branch and you have to sacrifice that segment so the management of massive airway bleeding the goal is to prevent exsanguination because they can pour a liter a minute out of their airway you want to maintain oxygenation we can do conservative management if there's just a little bit of blood in the airway and you can just beat the blood back with peep and reversal of heparin and correction of of coagulopathies so typically you have blood available if it's massive bleeding you have to use a blocker here we have a unique blocker that goes into the airway and we block off one of the lungs fortunately for this patient is doing okay we can oxygenate on one sometimes you can get one and a half lung if you can isolate exactly which airway the bleeding is coming from and then you can reverse and wait for a period of time and then you can take the blocker down occasionally the bleeding is so much that you cannot oxygenate the patient and you have to completely block off the lung if you don't have adequate oxygenation you have to put a vino vino ECMO cannula this is what we've done in this case here you can see the cannula sitting here and we don't use anti coagulation at all we completely reverse them normalize them and typically this heals up the injury heals up and you can come off bypass hypoxemia after pulmonary endotherectomy typically it's atlactasis they might be still VQ mismatching or it might be a reperfusion lung injury that causes hypoxemia reperfusion lung injury is a lot of debate okay what is it and how exactly do you is it hypoxemia with the infiltrate hypoxemia with infiltrate after 48 hours or is it when it's after four days there's a lot of debate but what we do know is that if you see infiltrates like this where you have a whiteout segment and on the preoperative area you had a mismatch that you corrected this is typically a reperfusion injury and this patient might be quite hypoxemic this is the definition that we use for our publications P02 and an F02 ratio of less than 300 opacity on the chest x ray in a region that has been endoderectomized and reperfused and no alternative explanation for the hypoxemia and we do know that it has clinical impact the patients stay on the ventilator for longer they stay in the ICU for longer and in the hospital and we know that the mortality is also different but there's no no injury this is better mortality outcomes than having a reperfusion lung injury it's a high permeability edema basically and if you do a bronchoalveolar lavage you're gonna see a lot of neutrophils and proteins in that specimen and we do recognize a bimodal distribution about 60% of the cases that happens immediately in the OR immediately you come off bypass and there's pulmonary edema coming out of the endotracheal tube as a frothy and then there's another one that happens about 48 hours later and it's about 30% of reperfusion injuries present like this everything looks good we actually extubate the patient there's a couple of signs if you see they become febrile and the white cell count is really high have a high suspicion that you might suddenly develop reperfusion edema and you have to be ready don't send that patient to the floor keep them in the ICU watch them a little bit longer and then occasionally we have seen late like a week later developing a reperfusion injury how do we prevent it we try to suppress inflammation we inhibit cytokines inhibit neutrophils we give big doses of steroids around the time of the surgery and hemodynamic strategies we try to limit the cardiac output we try to not have like a eight nine ten liter cardiac output going through that lung that's just been reperfused so we use a little bit of inotropy in the beginning we try to use dopamine which is not as potent and we check the cardiac output we aim for a cardiac index of around 2.4 that's enough you don't need more at this point in time you can actually be harmful if you have a bigger cardiac output and then ventilator strategies is basically sent to around positive pressure ventilation deep development of edema the management we diaries them sometimes we have to prone them steroid dosing inalternetric oxide is used for it and occasionally we have to deploy ECMO if it is severe we have seen a decline in the incidents of reperfusion from the edema and I don't know if it's related to the surgical techniques the newer bypass circuits that we have that's less inflammatory but clearly the incidence of it this is going down just a couple of photos here talking about the levels of disease we say level one is when it's in the main pulmonary arteries where you start the dissection plane in the main pulmonary artery and that's a big old specimen that came out of this patient the level one and level two so here you can see it's main pulmonary artery upper lobe and lower lobe where we took it out there's some fresh but the important thing is getting these tails the chronic the white fibrous leathery stuff out this is level two started in the lower the dissection and then go down further here's some fresh plot that sometimes is present it's much more difficult to remove this but this is a chronic PE that has been present and then the patient had a me more recent acute embolism on top of it also a particularly challenging group of patients when they have fresh clot that you have to because you cannot hold on to it it's much easier to take out the chronic fibrous disease is level two and level one see one see stands for complete occlusion so this all thrombus completely occluded flow to the left lung and once we took the thrombus out deeper down there was previous incidents of more chronic disease that had to be removed as well calcifications sometimes you if it's been really long-standing that the thrombus can actually calcify and you can see it on the CT or even on a chest x-ray more calcified disease also challenging because it doesn't collapse and it's not so easy to take it off but as long as you push the patient away from the specimen you should be able to complete the end of the rectomy more level two disease level three is when it's in the segmental and this is level four when it's in the sub-segmental so really small little plots why they're so small because they originated from this pacemaker which had a inside out erosion this is a riada lead that developed plot on it and it kept embolizing over a long period of time we removed the pacemaker system and put an epicardial system in for this patient at the time of end of the rectomy this is level zero disease so here is a misdiagnosis you can see it looks a little bit more like aorta tissue actually and it's somebody with pulmonary arterial hypertension that got to the surgery you can see as is way earlier in the experience we're in the 4 000 cases now we don't get to misdiagnose too too much anymore but there's no tails and this patient's pulmonary artery pressure was the same after the surgery and didn't do so well all the trousers it's not tails but trousers it just ends blunt at the bottom pacemaker clots we already said there's a high risk especially if it gets infected here's a vegetation that's stuck on and the patient had multiple embolies ports vascular ports very frequent if the port mold functions like this one you can see this clot in it and every time he gets an injection of a drug he first gets a a nice embolism and because this embolism sits in here for a while it's fairly organized already so you give them level three level four disease with every injection so we remove these ports at the time of the surgery as well the ucsd experience from 1984 and standing on the shoulders of many people that have gone before Dr. Moser, Dr. Auger, Dr. Jamison with the people that really got it going for us and the volume is gradually increased we're doing around 200 cases a year at the moment and it seems to be this we we are we are growing the the hospital a little bit sometimes difficult to get patients in but i do think that there's a disease that is widely prevalent and that we should have more centers that perform this so congratulations to Dr. Luire and team for starting yesterday as well this is the mortality rate at ucsd over time and you can see we're sitting around 2% mortality at the moment and that's even though we get most of the more challenging cases and complex cases get to refer to us at the moment and we're still trying to learn how to even manage the the more difficult cases because there's not too many level two disease coming to us anymore survival at ucsd we know that the five-year survival is more than 80% and it whoops ended off let's see if we can come back there we go the 10-year survival is still 75% so much better than lung transplant survival this is a treatment that should be offered to patients prior to lung transplant consideration what happened to the hemodynamics this is a a systematic review by Rana Vardy looking at all the publications and summarizing the mean pulmonary artery changes from pre to post-op the pulmonary vascular resistance changes and he demonstrates that most authors report improvement in hemodynamics a 35 percent reduction in mean pulmonary artery pressure a 1.5 to 2 liter increase in the cardiac output 1 to 1.5 increase in the cardiac index and pulmonary vascular resistance almost routinely decreases to around 300 so very good ucsd pre and post-operative this is our outcomes this is an ongoing thing so systolic pressures coming down from from 79 to 45 the mean pa pressure 47 to 26 pulmonary vascular resistance average 897 down to 245 the cardiac output goes up from 3.5 to 5.8 so really significant changes for patients but there's morbidity and mortality here you can see that same systematic review shows the mortality and it's ranging here mostly around the 5 percent there's a couple of people that have in the double digit mortality rate so is a procedure that you have to have a good team and all the pieces in place if you want to get good outcomes this is how long our patients stay typically they stay around 10 days in the hospital and it's hard to get them out much quicker they take some time they have to recover from the the lung injury basically during the surgery so it's a little bit longer than a regular cabbage the survival here you can also see the changes in functional capacity a significant improvement the six-minute walk test significantly improve after the surgery and then the survival also in the systematic review has been reported as very good predictors of worse outcome age if they're older if they need valve surgery if they need bypass surgery at the same time if they have a very high pre-operative PVR more distal disease if they come into the OR with a high right atrial pressure female gender has a higher risk for the surgery and if you don't use deep hypothermic circulatory arrest and room saturation coming to the operating room at 85 percent or presence of comorbidities it's all been by different authors identified as risk factors we already mentioned this slide the higher the PVR the higher the risk but what about post-operative or residual pulmonary hypertension from the Papworth group we know that they looked at their outcomes and they follow patients back at three months and they found that 31 percent had residual pulmonary hypertension so still pretty significant which was a mean pa pressure of more than 30 they found that these patients had worse exercise capacity had more symptoms than the patients who did not have residual pulmonary hypertension but their survival at five years was similar to the patients who had no pulmonary hypertension so they might still be somewhat limited but they are most probably going to have a survival benefit in the long run residual pulmonary hypertension so it is 15 to 35 what can you do treatments for it in the ICU use isotropes you support the circulation you can use inelipidic oxide isoprostoepoprostonal we very seldom use it we'd normally try to diurese the patient use the inotropes and then gradually wean it as as the lung remodels and the ventricle remodels so in conclusion then the pulmonary endoderectomy is and remain the treatment of choice for chronic thromboembolic pulmonary hypertension we can support and long-term outcomes that can be achieved as long as we adhere to the surgical principles in the future i think we need more high quality prospective data to just make sure that we have good safety that we can approve the efficacy and identify more prognostic factors this is actually still despite the almost 30 year history of the disease at UCSD not widely disseminated throughout the whole country and i think we need to work hard on having multi-institutional registry which now does exist and hopefully we're going to soon get more data thank you very much happy to entertain questions