Pulmonary Hypertension | Retired

foreign what's up Ninja nerds in this video today we're going to be talking about pulmonary hypertension so pulmonary hypertension is just basically when there's high blood pressure particularly within the pulmonary circulation like we call high blood pressure we call it like systemic hypertension this would be pulmonary hypertension so high blood pressure within the pulmonary circulation now what we need to discuss throughout the process of this video is primarily focusing on the different types of pulmonary artery hypertension and then the associated causes within those types we'll discuss the pathophysiology a little bit and then what we'll do is we'll go into the classic clinical features which is really only two that I really want you to know and then we'll talk about kind of the big thing which is how to diagnose pulmonary artery hypertension there's lots of tests we'll have to go through those step by step and then finally we'll talk about how do we treat pulmonary hypertension but really focusing on on type one so let's actually get into the different types first of pulmonary artery hypertension so we know that the basic definition here is that the pressure within the pulmonary arterial circulation is high we should actually develop a number right so really what the number is I said we were to really look here the pulmonary arterial blood pressure here is somewhere around so the the mean uh pulmonary artery pressure is greater than or equal to 25 millimeters of mercury is how we really kind of look at this so that number within the pulmonary arterial circulation has to be greater than or equal to the mean 25 millimeters of mercury now we have type one now type 1 is a really interesting one and that's the one that we're going to focus on a lot throughout the process of this lecture now type 1 pulmonary artery hypertension is pretty much the basic concept behind this is that the pulmonary vessels are super vasoconstricted and the pulmonary vascular resistance to these pulmonary blood vessels they're super clamped down there is a very high pulmonary vascular resistance now we know that as you increase resistance to blood flow that does what to the actual pressure it increases the pressure so as I increase the pulmonary vascular resistance I increase the pulmonary artery pressure and what do I increase the pulmonary artery pressure to to at least the mean amount greater than or equal to 25 millimeters of mercury so now the pressure within this pulmonary circulation is super super high it's really high and what that does is that creates a strain on the right heart and because if the pulmonary artery pressure is super high that'll put a lot of strain on the right heart which will cause the right heart to undergo right ventricular hypertrophy and then eventually go into right heart failure so you see the process here right pulmonary arteries super vasoconstricted as you cause intense vasoconstriction the pressure within the pulmonary circulation Rises as the pressure in the pulmonary circulation Rises it puts strain and stress on the right heart the right heart will have to hypertrophy to try to get thicker and develop a higher like strength and contractions to push blood out to it and then eventually that will cause it to fail my question to you is is for type 1 what causes this pulmonary vasoconstriction that causes intense vascular resistance great question the basic pathophysiology is that we're actually taking cut this vessel and then what I want us to do is actually look at this and zoom in on it at this view here I'm taking a cut of the vessel we're going to take a cut of this vessel here and we're going to look at it I want you to understand here something that happens naturally to keep our vessels dilated or constricted there's a kind of a homeostasis between vasodilation and vasoconstriction on this top one here what we're going to do is we're going to have these endothelial cells which are in blue and then this is the smooth muscle around it in red these kind of endothelial cells can make a particular chemical here called Endo fill in one and what endothel and one does is it is an intense and I mean potent vasoconstrictor and what it'll do is it'll cause this smooth muscle to intensely vasoconstrict it'll cause it to contract which will cause the blood vessel to decrease in diameter and increase the peripheral vascular resistance thus this process here so it'll increase the Vaso constriction the other thing is that we have these endothelial cells and they also make other molecules called nitric oxide and pgi2 which is called prostacyclin and what that does is that acts on the smooth muscle and inhibits the smooth muscle from Contracting and if it inhibits the smooth muscle from Contracting it should generally cause the muscle to relax increase the actual vessel diameter and decrease the peripheral vascular resistance right in this disease type 1 pulmonary artery hypertension there's a couple different things that can cause this process but the basic etiology the basic pathophysiology if you will is there is an increase in the vasoconstricted process and a decrease in the vasodilation process so there's less nitric oxide less prostocyclin which leads to less inhibition and therefore less vasodilation so there is a decrease in the Vaso dilation and when you take the combination of an increased vasoconstriction a decrease vasodilation what do you get The increased peripheral vascular resistance therefore increase the pulmonary artery pressure putting strain on the right heart causing it to eventually fail you guys get the whole point here now the question that you might be asking is what kind of things actually cause this that is a great question and here's what I want you to understand one of the big things that I really want you to understand is a potential cause here is that we it may be idiopathic but we have to understand that this is a diagnosis of exclusion the second thing I want you to remember here is it could be hereditary so this could be hereditary where there is a particular Gene and some reason they like to ask that sometimes in the exams which the gene is the bmpr2 Gene and this Gene may also again accelerate this type of process where you have genes that are activated cause an increase in the vasoconstriction so an increase in the endothel in one production and a decrease in the nitric oxide and process cichlid production here's the other thing I want you to think about this could be due to connective tissue diseases connective tissue disease that cause intense into my fibrosis and medial hypertrophy so I want you to remember another thing here is it could be connective tissue diseases and the connective tissue diseases that are actually worthy of remembering here is going to be two one is called SLE and the other one is called Scleroderma don't forget these okay the next thing is this this one's really interesting it could be due to HIV we don't really know how this actually happens but HIV May potentially cause this problem as well so I also want you to remember that HIV is another potential etiology here the next one that I want you all to understand here is going to be something called porto-pulmonary hypertension so Porto pulmonary hypertension this is a really interesting process so basic concept here basic concept your liver is supposed to clear vasoactive molecules right so the molecules that cause vasoconstriction imagine for some reason you have a damaged lever right so your liver is damaged the chemicals that are supposed to go through this it's going to clear because then it goes into the inferior vena cava from the inferior vena cava goes into the right atrium right ventricle pulmonary arteries what if these chemicals that are the liver supposed to clear and remove it doesn't remove and these chemicals cause an increase in the vasoconstriction and a decrease in the vasodilation that will cause increase in the pulmonary vascular resistance so think about portal pulmonary hypertension and patients who have under underlying cirrhosis the other thing that I want you to think about here is any kind of situation where there is an increase in pulmonary blood flow and they have to adapt this is a big one in little babies as they get older they may develop this and this is called left to right shunts so as you have left to right shunting so you have a big vsd and ASD a patent foramino Valley what happens is you push a lot of blood from the left heart to the right heart and that overflows the pulmonary circulation and it has to adapt to that high flow so it undergoes intense vasoconstriction thickening of the actual vessels medial hypertrophy all that stuff so here's the things that I want you to remember but what is the most common out of all of these the most common is going to be idiopathic but in other words we have to rule out all of these other ones as well as the all the other types of pulmonary artery hypertension that's why this can be somewhat difficult disease so understand type 1 the basic pathophysiology the basic pathophysiology is there is an increase in vasoconstriction processes a decrease in vasodilation processes causing an increase in peripheral vascular resistance increasing the pulmonary artery pressure putting strain on the right heart idiopathic is the most common cause then heritable and then think about connective tissue diseases think about HIV think about portal pulmonary hypertension think about also left to right shunting as well don't forget these things okay type 2 pulmonary artery hypertension and this part it's actually due to the left heart so it's left heart disease I don't want to even make it too complicated here it's really it's just a patient has left heart failure if they have left heart failure or valvular heart diseases or maybe they have valvular heart disease what this does is this causes a increase in pulmonary Venus backflow if blood is backflowing from the left heart into the pulmonary veins it backflows into the pulmonary veins and then into like the pulmonary area here now this is going to cause so much congestion in this pulmonary circulation here same thing here back up into the pulmonary venous circulation and cause intense congestion in this area that's going to increase the pulmonary artery pressures so that will cause an increase in the pulmonary artery pressures as the pulmonary artery pressure increases what does that do to the right heart strains it what does that cause right ventricular perjury what does that cause right heart failure okay so type 2 is left heart diseases such as left heart failure value of the heart diseases type one it's increase in vasoconstrictors decrease in vasodilators idiopathic most common type 3 pulmonary artery hypertension is a lung problem so in patients who have underlying lung disease all right so for example things like COPD or things of that nature like interstitial lung disease or obstructive sleep apnea obesity hypoventilation syndrome these are the most classic cases but it's a pulmonary disease what happens is there's disease of multiple lung parenchymas so here we're going to just kind of like scratch out here here's some disease lung parenchyma here's some disease lung parenchyma here's some disease lung parenchyma here's some disease lung breakama okay in areas where there is disease lung parenchyma there's inadequate ventilation right so if there is low ventilation here low V here low ventilation here low ventilation here low ventilation here do we want blood flow to go to the poorly ventilated areas no and if I don't want blood flow to go here or here so I can send it to other areas which are well ventilated what's going to happen I have to constrict the pulmonary vessels to direct it away from these poorly ventilated areas and areas of good ventilation so I constrict the pulmonary vessels if I constrict on the pulmonary vessels I'm going to constrict here what it's going to do is it's actually going to cause the pressure within this it's going to cause the resistance to go up so because of this the actual decreased ventilation or the hypoxia that may actually occur here so the decreased ventilation will lead to hypoxic what we call vasoconstriction and that hypoxic vasoconstriction will do what it'll actually cause the pulmonary vessels to actually vasoconstrict that will increase the pulmonary vascular resistance if you increase the resistance in these vessels what do you do the pulmonary artery pressure you increase it then you put strain on the right heart that causes right ventricular hypertrophy eventually it goes into right heart failure so type one idiopathic increased vasoconstructures decrease vasodilators type 2 left heart disease type 3 pulmonary diseases type four okay in type four this one's actually really an interesting one as well let's imagine you have chronic pulmonary emboli right here so here's some tiny little distal pulmonary emboli if there's distal pulmonary embolize so this is actually what kind of situation here the patient has chronic pulmonary emboli that's going to cause again blood is supposed to go from the right ventricle over into the pulmonary circulation but as it does it's going to hit some resistance to blood flow as there is an increase in this peripheral vascular resistance due to clots so you see how we're getting the difference here that vasoconstriction in type 3 was because of hypoxia or poorly ventilated areas this kind of like area where it's not really a vasoconstriction it's an increase in peripheral vascular resistance because of clots getting in the way whereas type 1 is an increase in peripheral vascular resistance Because the actual vessels are Contracting because of those Pathways so understand the difference type 1 increased vasoconstriction decrease vasovilization due to these particular diseases type 2 pulmonary venous backflow congestion type 3 hypoxic vasoconstriction due to lung disease and type 4 is increase in peripheral vascular resistance due to clot burden distal so now I'm going to have an increase in peripheral vascular resistance due to these clots that's going to increase the pulmonary artery pressure that's going to put strain on the right heart causing right ventricular perseverance over time right heart failure okay type one increased vasoconstriction decrease vasodilation due to an increase in endothel and one decrease in prostocycles due to idiopathic mainly type 2 left heart disease pulmonary venous congestion and then pulmonary artery pressure increases type 3 lung disease causing hypoxic vasoconstriction increasing resistance Type 4 clots blocking the flow of blood from the right heart increasing resistance increasing right heart strain type 5. in type 5 this is usually multi-factorial but I only want you to remember two particular causes if someone has a like a big granuloma right and Sarcoidosis so they have sarcoidosis and this is compressing on some of the pulmonary vessels so you have a patient who has what's this disease cause here this big granuloma this is sarcoidosis and what it's doing is it's compressing on the pulmonary vessels if you compress on the pulmonary vessel here what are you going to do the resistance in this vicinity increase it because you're compressing it from the outside so I'm increasing the peripheral vascular resistance or if someone has a tumor maybe they have a tracheal tumor a bronchial tumor they have lymphoma something of that nature a mediastinal tumor that's compressing the pulmonary artery if I have a media style tumor these will compress on the pulmonary vessels and do what to the pulmonary vascular resistance increase it if I increase the pulmonary vascular resistance because I'm compressing the vessels from these granulomas or tumors I do what to the pulmonary artery pressure I increase the pulmonary artery pressure I put stress on the right heart causing it to have right ventricular pertrophy and eventually go into right heart failure that's how we explain pulmonary artery hypertension now we have the patient with pulmonary artery hypertension how does this increased pulmonary artery pressure the right heart strain potentially right heart failure how does that actually present let's talk about that all right so how will these patients present well here's the big thing you already kind of saw I talked about this a lot is that patients May progress to right heart failure from chronic pulmonary hypertension because they're put on a lot of stress right in the same way patients May develop heart failure from systemic hypertension so left heart failure due to severe chronic hypertension same thing can happen with right heart so as you have chronic pulmonary hypertension you can lead to right heart failure so obviously one of the classic things that we would look for is evidence potentially of right heart failure right so whenever patients have right heart failure it's obviously something that we've kind of talked about before but how will these patients present it's not super complicated if the right heart fails blood backs up right so blood will back up where it'll back up into the superior vena cava and then up into the jugular veins and then to sting these bad boys so the patients could potentially develop jugular venous as an engine so look for jugular venous distension it could also back up into the right atrium and into the inferior vena cava swell up the liver leak into the abdomen and cause ascites and hepatomegaly so we can look for ascites we can look for hepatomegaly it could also kind of cause fluid to leak backwards into the interstitial spaces of the lower extremities cause pedal edema so they may also have some peladem or peripheral lower extremity edema so look potentially for evidence of that so these could be some of the potential features that you may see from the right heart failure from that high stress but here's the other thing when you put that heart under so much strain the other thing that you can see is evidence of right ventricular hypertrophy right so if the heart imagine you take the stethoscope put it on the chest where like the pulmonary artery is and you're trying to listen to the different heart sounds if the pulmonary blood pressure is so high that whenever you shut for example if patients have really severe systemic hypertension what would that do to the actual aortic valve it would snap that sucker close really hard it would cause a loud S2 particularly the aortic component and the same way when the patient's pulmonary pressure is really high and they go into diastole it smashes that pulmonary valve closed very quickly so the other thing that you may potentially see with these patients as evidenced of really their right ventricular hypertrophy is you may hear potentially one of those things is a loud S2 but particularly the P2 component all right so the P2 component of the S2 because you know it consists of the aortic valve and the pulmonary valve closing the other thing is that if you go to place your hand on their chest on their kind of like right parasternal uh portion there or just a little bit to the side here you may have a heave right so they may have a right ventricular heave so look for any evidence of a right ventricular heed as well due to that intense right ventricular hypertrophy so these would be some of the things that you may see now this is not classic right you know what the most classic symptom is for patients who have pulmonary hypertension and I really don't want you guys to forget this the most common symptom is exertional dyspnea and you may be like well that kind of sounds like heart failure or coronary artery disease and things of that nature that's one of the things that most oftentimes people have maybe missed the diagnosis of you know pulmonary hypertension because it looks like CHF or can you know some type of coronary artery disease it's really important to remember this so exertional Disney it happens to be the most common feature now why if the pulmonary artery pressure is crazy high your right ventricle is going to have difficulty getting blood into that high pressure system and delivering blood to the left heart so the whole concept here is that with this high pressure there's going to be less blood moving through the pulmonary arterial circulation and entering into the left heart less less blood is in the left heart now that means less left ventricular cardiac output that means I deliver less oxygen to the tissues now so less oxygen delivery to the tissues now imagine here you have the patient exert themselves so now their tissues are going to consume more oxygen so if I have less oxygen delivery and on top of that let's say for this patient I increase their O2 demand how do I do that I exert them I have them run I have them walk I have them climb up stairs when this happens you create a mismatch and that mismatch there whenever they exert themselves so this would be during exertion and this decreased oxygen delivery to the tissues is just basically because of less blood getting to the left heart because of that this combination will lead to dyspnea and this dyspnea is particularly dyspnea on exertion this is the most common feature for these patients they may have some syncope on exertion as well due to less oxygen delivery to the the brain tissues they may have chest pain to less oxygen delivered to The myocardium but this is going to be the most common type of symptom which is dyspnea on exertion please don't forget that all right so look for evidence of right heart failure and look for any exertional symptoms Disney on exertion exertional syncope exertional chest pain but this one is by far going to be the most important but if you wanted to don't forget syncope okay on exertion and angina on exertion let's also move on to the next part here which is going to be talking about how do we actually diagnostically approach pulmonary hypertension we have a patient who comes in they present with evidence maybe of right heart failure some distance on exertion or some dyspnea some exertional syncope some exertional chest pain you work them up for all these different types of disorders and you're trying to figure out do they have a pulmonary artery hypertension and if they do is it type one is it type 2 is it type 3 is it type four is it type five but really the one that I absolutely want to figure out is is it type 1 pulmonary artery hypertension so let's talk about how we get to that all right so now let's move on to the next part which is how do we diagnostically approach patients who have some type of pulmonary or hypertension so first off is clinical suspicion right so for example if you have a patient who has exertional exertional syncope exertional angina that's definitely high yield stuff to think about but then on top of that I also think it's important to be able to understand do they have any evidence of right heart failure due to that high pulmonary pressures right heart strain right ventricular pertrophy that progress them into right heart failure okay then think about the classification type 1 type 2 type 3 Type 4 type 1 type 5 right so type one is it some type of like problem where they have increased vasoconstrictors decrease vasodilators right we talked about that is it a left heart disease is it a lung disease is a chronic thromboemboli uh with pulmonary embolize basically or is it a type five less likely but some type of compression due to Media Center tumors or sarcoid so how do we do this first thing is if you have a degree of clinical suspicion this is usually not going to be a patient who comes into the hospital or to the clinic with rip roaring hypoxemia to kidney and things like that it's exertional exertional syncope exertional angina start off with a plethora of tests to really try to elucidate this one of them may be an echocardiogram a VQ scan a high resolution CT a polysomnogram pfts EKG in Labs you're like what the heck so first one I do the echo why do I do the echo okay I do the echo because the whole point of the echo is to first off rule out that this is a type two pulmonary artery hypertension because what does type 2 left heart disease is there any wall motion abnormality is there any reduced ejection fraction is there any valvular heart disease right there I can automatically determine that the other thing is the echo is very helpful for looking at that right heart if you have high pulmonary artery pressures what are you going to expect with the right ventricle you may see some right ventricular hypertrophy but over time you may see evidence of right heart failure which is right ventricular dilation or right atrial enlargement so these can be some big boom in right side of the heart that's not going to be big all right so look at a big right ventricle big right atrium the other thing here is usually in these patients this would be the first thing that I would like to do so let's actually take a quick look at an echocardiogram of what that would look like in a patient who may has pulmonary artery hypertension all right so if you take a look at this Echo here what we're looking at is we're looking at the right side of the heart so you can see here left ventricle left atrium right atrium right ventricle so this is a nice kind of like apical four chamber View and what we really see here is look how huge the right side of the heart is so there's definitely like some right ventricular dilation even a little bit of hypertrophy here and then there's pretty like a significant right atrial enlargement and if you were to look at the inferior vena cava to apply flow off of this right atrium it would also probably be pretty plethoric as well and pretty big so we can tell that there's high right sided pressures right ventricular dilation right atrial enlargement as well so this would be pretty supportive of a pulmonary hypertension a pulmonary hypertensive patient especially if it's a chronic finding we see this on repeat Echoes all right let's get back to the Whiteboard okay so we got the echocardiogram it ruled out type two showed us right ventricular dilation right atrial enlargement some high right ventricular pressures assuming that so we assume that and then we also assume that they have some high right ventricular pressures the next thing I want to do is I want to consider a VQ scan okay so I'm going to start off with an echo rule out type 2 and then calculate my right ventricular pressures to assess for any right ventricular hypertrophy right uh ventricular dilation right atrial and large the consensus of right heart failure then I'm going to do the VQ scan the VQ scan will rule out type and let's see if you guys know would it be type 3 Type 4 nice it's type 4. because VQ scans are good for pulmonary emboli so it'll help me to rule out Type 4 pulmonary artery hypertension now you might be like why wouldn't I do a ctpa because that diagnosis pulmonary emboli or a pulmonary angiogram VQ scans actually have been shown to be a little bit better than ctpas in patients who have chronic distal pulmonary emblem so think about that so that's one reason I would do that okay rule out that they don't have a Type 4 pulmonary artery hypertension due to Chronic pulmonary emboli and you'll be able to see that due to areas of decreased perfusion in different parts of the lung on the VQ scan pretty cool stuff the third and remaining stuff that I would also do is I get that high resolution CT scan and I would also get a sleep study so I'm going to get a sleep study which is a poly somnogram I'd also do some pfts an EKG and I'd finish off with some general Labs which may be somewhat helpful so when I do a high resolution CT scan this is actually an important one when you do a high resolution CT scan you're probably like oh wait I thought they do chest x-rays they can do chest x-rays chest x-rays will kind of show somewhat of the similar features of a CT scan but a high resolution CT scan will also help me to rule out a Type 3 pulmonary artery hypertension so if I use this high resolution CT scan it'll help me a little bit it won't guarantee me but it may help help rule out a type 3 and what else mediastinal tumors sarcoidosis like granulomas other compressive things type five type five pulmonary artery hypertension isn't that pretty cool so that's one thing I get the CT scan I'll be able to get a good image of the lung parenchyma of the mediastinum the other thing here is when you're looking at pulmonary artery hypertension there's classic features and it's like a weird dang name but they look for what's called dilation so you have just big pulmonary arteries or we use the term pruning I don't know why they call this but pruning of the pulmonary arteries these suckers are huge and it's because the pulmonary artery pressures are super super high so sometimes they kind of like dilate a little bit so you may see evidence of a dilation of pruning of the pulmonary arteries on chest x-ray or high resolution CT it's just better to get the high resolution CT because you can rule out mediastinal types of tumor sarcoidosis and maybe even assess for type 3 like COPD interstitial lung disease all right my friend so here we're actually going to be looking at a CT scan of the chest now this happens to be a CT scammer you can actually see some of the contrast kind of moving through the pulmonary vessels look how huge this pulmonary artery is so there's like dilation or pruning if you will of the pulmonary arteries and that's just due to that extremely high pulmonary artery pressure so these are relatively common findings that you can see in patients with pulmonary artery hypertension look for that high right-sided pressures right ventricular dilation right atrial enlargement maybe a plethoric IVC dilation pruning of the pulmonary vessels and again you know in these situations we're going to continue to keep going through the rest of the test kind of ticking off the different types of pulmonary artery hypertension really trying to elucidate the type 1 idiopathic pulmonary artery hypertension all right let's keep talking about this let's get back to the Whiteboard but that's not the best one because guess what I can get from a polysomnogram I can rule out type three which type three pulmonary artery hypertension Osa and maybe even to some degree possibly an Associated obesity hypoventilation Etc so you see we're crossing things off the board okay come down now let's talk about what we'd also see here so we'll rule out a lot of things step by step trying to really elucidate which one of these is type one that's really the question I want to get to type one pulmonary hypertension because that's a diagnosis of exclusion I told you that it's a diagnosis of exclusion so pfts may be helpful here why because pfts will rule out roll without it will rule out your type 3 for sure skis your type three pulmonary artery hypertension such as COPD and interstitial lung disease which are obstructive and restrictive lung diseases respectively pfts are the best for that because all I got to do is I say I look at the fev one man this is such a good space repetition right fev1 over FEC I look at the ratio is the ratio less than 70 or is it greater than or equal to 70 if it's less it's obstructive okay if it's greater than it could be restrictive or it could be guess what pulmonary hypertension could be pulmonary hypertension really the ways that we kind of further classify these if you guys remember is an obstructive they have a very high total lung capacity and a very high residual volume right and very low fev ones and then if you really wanted to if you guys remember the dlco you could check the dlco if there's a low dlco that was emphysema and if there was a normal dlco that was chronic bronchitis if you do restrictive you want to see do they have a very small total loan capacity in a very small residual volume and a very low functional vital capacity and then you can check the dlco and see do they have a low dlco that would be interstitial lung disease and do they have a normal dlco that would be those extra pulmonary so chest wall pleura some type of neuromuscular disorder obesity for pulmonary hypertension the only thing that we do is we see normal fev1 over FEC a normal total lung capacity residual volume here's the only other thing they have a decreased dlco that's what's interesting is that these patients have a decreased dlco which would be different from someone with completely normal pulmonary mechanics okay if they had a normal dseo normal Title Loan capacity normal residual it would be a normal low mechanics but they have a decrease remember that that's super helpful our ECG really it's not that hard we're not going to use it super super helpful but it's not going to be a crazy great test but it may be supportive what are we looking for right heart strain so you may see a thickened right ventricle right ventricular aperture if you may see right ventricular dilation or right atrial enlargement so you could be looking for right atrial enlargement you could see evidence of right ventricular hypertrophy and if you really kind of dilate The ventricle and stretch it out what also you may see you may stretch those bundle branches and get a right bundle branch block so really all you're seeing is evidence of right heart strain not super diagnostic though but helpful here's where another test comes in Labs okay I've really ruled out a lot of these right and I've really come down to which tests are really going to help me with type one one is Echo tells me that there's high right heart pressures right ventricular dilation radiator enlargement high resolution CT tells me if there's a lot of dilation pruning pfts helps me to kind of lead towards pulmonary hypertension here's where some other soft Labs I can check a bunch of different Labs such as lfts what would lfts rule out porto-pulmonary hypertension cirrhosis HIV remember I told you HIV can cause type 1. I can order an A A what is that going to tell me lupus and I can also order the Scleroderma antibodies what you'll tell me about scleroderma if I get all of these it comes back elevated lfts I confirm that they have portable pulmonary hypertension boom HIV test them they come back positive okay there we go a a they have Lupus Scleroderma you see what I'm getting to I'm doing all of these tests and if I go through all of these and I rule out every single one of these disorders what am I left with my friends idiopathic or heritable and I can even really I really wanted to check that gene mutation but idiopathic pulmonary artery hypertension the way to truly most so if I've gone through all of these I have ruled out type 2 type 3 Type 4 type 5. and all the other etiologies that are not idiopathic for type 1. what I can really do to completely confirm the diagnosis I can take a catheter I can run it down the IJ I can run it down the superior vena cava down the right atrium down the right ventricle into the pulmonary artery and at different points I can measure pressures okay but the big one that I'm really kind of concerned with is the pulmonary artery pressure and the pulmonary capillary wedge pressure that's really what I care about and so what I'm going to do is I'm going to measure the pulmonary capillary wedge pressure and I'm also going to measure the pulmonary artery pressure and I'm going to take the difference between them so the difference between so if I take the actual it's called the trans pulmonary pressure gradient transpulmonary arterial pressure is equal to the pulmonary artery pressure minus the pulmonary capillary wedge pressure now I'm going to kind of go through all this right and these patients what's going on with their pulmonary artery pressure we already said it's high thus they have pulmonary artery hypertension pulmonary artery hypertension can be super high what number do we need greater than or equal to 25. I need you guys to remember this please don't forget this okay let's do this second thing what is pulmonary capillary wedge pressure indicative of please don't forget this is it indicative of you know left atrial pressures left heart pressures yes so if the pulmonary capillary wedge pressure was high that would be type 1 or type 2 pulmonary hypertension type 2. so that would tell me it's type 2 wouldn't help me to determine if it is type 1 remember type 2 is heart disease left heart disease that would tell me that the heart is the problem it can't be the heart it has to be the pulmonary vessels that are the problem so in this situation I want the pulmonary capillary wedge pressure to be low less than there's a particular number random but less than 15 millimeters Mercury okay now if I have less than 15 millimeters of mercury for the pulmonary capillary wedge pressure it's low the pulmonary artery pressure is high and I take the difference if there's a high pulmonary artery pressure and a low pulmonary capillary wedge pressure what's the overall transpulmonary pressure going to be high or low high so I would have a very high trans pulmonary pressure gradient these are the ways that we kind of secure the diagnosis of pulmonary artery hypertension now that we've gone through all of this we've secured the Diagnostics we figured out how to determine which type it is but really getting to the bottom of is it type 1 idiopathic we've done that now how do we treat primarily type 1 idiopathic pulmonary artery hypertension all right so now treating pulmonary artery hypertension so I talked about we're going to only talk about type one and the reason why is for type 2 guess what treat the underlying left heart disease for type 3 treat the underlying lung disease for Type 4 treat the underlying chronic chronic thromboemboli for type 5 obviously treat the underlying side chordosis you know mediastinal tumors etc for type 1 you know obviously if you can try to treat the HIV the portal pulmonary hypertension you know they're left to right shunting their Scleroderma their lupus Etc you guys get the point but for those patients who have like idiopathic really the thing that we got to think about here is how do we reduce the intense vasoconstriction and increase the vasodilation so we can reduce the peripheral vascular pulmonary vascular resistance reduce the pulmonary artery pressure put less stress on the right heart and put a limit less risk of right heart failure right so the first thing we have to determine is is this a vasoreactive pulmonary artery hypertension so how do we do that it's really cool what we do is we introduce a little bit of a pulmonary vasodilator so you give them for example let's say we we first calculate okay we calculate their mean pulmonary artery pressure we've calculated it okay once we have calculated so we calculate this we we check that okay we know that that what that pressure is then what we do is we give them a vasodilator a pulmonary vasodilator and after we give them the vasodilator what we do is we want to know were they reactive to the vasodilator in other words did it dilate the pulmonary vessels enough that it reduced their mean pulmonary artery pressure right and how do we determine that here's a great question so how do we know if they were Vaso reactive in other words they've responded well to the vasodilator and if that's the case if they test positive for that we can put them on something called a calcium channel blocker for example things like nifedipine this is a really good one people usually love this drug if you if you have a positive Visa reactive test you can do something called nifedipine This is generally kind of a prophylaxis to prevent the further progression of the disease so how do we determine if someone is vasoreactive so we check their mean pulmonary artery pressure before we give them the vasodilator after we give them the vasodilator what we expect to see here is post dilator we expect to see the mean pulmonary artery pressure the absolute value so the absolute mean to be less than 40 millimeters of mercury that's one so one finding the second thing is after we give them the vasodilator we expect their mean pulmonary artery pressure to decrease more than 10 millimeters of mercury after the vasodilator and we also expect the right ventricular cardiac output to increase or stay the same if you meet all three of these criteria in other words you measure their pressure prior given the vasodilator afterwards the mean pressure absolute is less than 40. they dropped from their original to more than 10 millimeter mercury drop from their initial one and the right ventricular cardiac output either increased or did not change that is a positive vasovreactive test put them on a calcium channel blocker if there is a negative test then you move on to the vasodilators other vasodilators besides nifedipine so that's the next question how do we determine the other vasodilators so here's what I want us to go back to there's three Pathways that we can Target I know you guys remember this one so here's this endothelial so we've got three little blood vessels that we took a cross section out and the pulmonary vessels one of them releases something called endothellan one and endothel and one activates this smooth muscle and causes vasoconstriction right so we know that the end process here is it causes Vaso constriction okay that increase the pulmonary vascular resistance that's one of the problems with this disease the other pathway is that it releases something called nitric oxide nitric oxide works on the smooth muscle inhibits the smooth muscle and basically is supposed to trigger Vaso dilation and then the same thing is we have one more pathway which is the prostacyclin pathway and they release this thing called pgi2 that works on the smooth muscle relaxes the smooth muscle and causes vasodilation in the type 1 pulmonary artery hypertension these pathways are inhibited and this pathway is increased so there's usually less of this and more of this that's the problem what if I gave drugs that blocked endotheon receptors preventing this vasoconstriction so in other words I give drugs that'll inhibit this process I give drugs that'll act like nitric oxide or increase nitric oxide so now I want to give drugs that will actually do something else I want to give drugs that will increase nitric oxide which will cause more of this muscle relaxation vasodilation and decrease resistance I want to give more drugs that'll increase the process cycling so that's my goal is I want to increase the vasodilation processes and I want to decrease the vasoconstrictive processes by inhibiting endophilin 1 from working all right so what are the drugs that could actually give to basically block endothel and one inhibiting vasoconstriction causing pulmonary vasodilation reducing peripheral vascular resistance within the pulmonary vessels and then reducing that pulmonary artery pressure these are endothelial one Inhibitors so these endophilin 1 Inhibitors would be drugs such as bosentan I just remember the Zen Tans bosentans and Ambry zintan so if you remember the Zen Tans that'll help you to remember the endophilin 1 Inhibitors reducing the vasoconstriction the next category here is the nitric oxide so I want to give drugs that'll basically Act like nitric oxide or increase the nitric oxide so how do I do that I give drugs that basically help to increase nitric oxide and these drugs are called pde Inhibitors phosphodiesterase inhibitors so phosphodastrease Inhibitors will help to be able to increase the nitric oxide and so these drugs are going to be things such as for example sildenafil sildenafil and tidalophil to Dallas now what's really important here is whenever you put patients on PDF Inhibitors so phosphodus Inhibitors it's important to remember to avoid let's actually do this in red avoid giving these medications with nitrates or avoid giving these in alpha blockers you wanna know why you can drop the patient's blood pressure because this drug will also cause vasodilation if you give it a combination with a nitrate or an alpha blocker maybe you're going to drop dead blood pressure so watch out for that okay the last pathway here the process cycling pathway I can give drugs that actually are going to act like prostacycline so I'm going to increase the amount of process cycling acting there to increase vasodilation so these are going to be what kind of drugs these are actually pretty interesting ones these are called your prosta cyclin kind of analogs if you will so these are going to be your prosta pgi2 kind of analogs and they're really really cool I really like these ones so these ones will be like ILO Prost ILO Prost the other one is called troprostanyl and the last one here is called EPO prostanol so these are really really cool drugs to consider if you see The Prost in there that's generally going to give that one away so if you see Prost think about prostocyclin analogues if you see the fill the a fill that would help you to be able to remember potentially oh here it is here's the affil if you see this this will help you to be able to think about pde um so phosphodastrous Inhibitors increase in the nitric oxide pathway and if you see zintans you can think about the endothel and one Inhibitors so now we have a pretty decent understanding about how we would treat the type 1 pulmonary artery hypertension the last thing here is if the patient is on all of these therapies or they've received one of these therapies and they failed then the last option here especially if they're getting worse and worse and worse it's super super sad but we may have to do what's called a bilateral lung and heart transplant just because there's so many pulmonary vessels that are affected and the right heart is super affected so now we have to take these diseased pulmonary vasculars from these lungs get rid of them put in some new ones and then we're going to need kind of like a whole new fresh right heart so unfortunately these patients if they fail all of these medical therapies they'll require a complete bilateral lung and heart transplant which is unfortunately not too common for these patients all right my friends that covers this let's get into the cases all right my friends let's do a case here so we got a 60 year old male with pertinent past minute with no pertinent past medical history who presents to the clinic with exertional distance so we know with pulmonary hypertension the most common clinical feature is Disney on exertion or maybe syncope on exertion but either way this is definitely classic pulmonary hypertension and there have no past medical history making me think that if they don't have any like heart disease lung disease pulmonary emuli or any other kind of like compressive problems in the chest cavity or mediastinum this is likely a type one right so idiopathic but let's keep going so physical exam findings again is usually nothing super super obvious their blood pressure's appropriate heart rate's normal and respiratory rates normal temps normal espio2 is normal so nothing is going to pop up um if they've had chronic pulmonary hypertension they may have some degree of evidence of right heart failure from the high pulmonary artery pressures so do look for any evidence of right heart failure in other words do they have any jvd do they have any hepatomegaly ascites peripheral or pedal edema look for any evidence of that so that is important to think about but sometimes that may not be present so how do we really go about you know obtaining a diagnosis of pulmonary artery hypertension so we're going to have to do a lot of things we got to rule out is this type 1 type 2 type 3 Type 4 is it even type five so with type one as I've told you before this could be idiopathic this could be heritable this could be due to HIV this could be due to Scleroderma this could be due to some type of like abnormality such as rheumatoid arthritis or SLE this could be due to hepatopulmonary syndrome there is so many different things that this could be so let's throw off some Labs do they have any evidence of lupus or Scleroderma HIV lft abnormalities no so no portal pulmonary hypertension no HIV no Scleroderma no kind of Lupus that we see here is obvious we get an EKG because this will help us to determines they're kind of like any heart problem or is there any evidence of right heart strain which would be supportive of pulmonary hypertension as well not diagnostic but supportive so they don't have any evidence of like any Mi or any kind of like acute abnormalities here but we do see evidence of right heart strain their right Atria is Big because of high pulmonary artery pressures and high right atrial pressures they have right ventricular perjury because of high pulmonary artery pressures and they have a right bundle branch block because of the you know remodeling of the right ventricle due to high pulmonary artery pressures so all evidence of right heart strain so if we get an echo we'll see potentially a very large right ventricle a very large right atrium and high pulmonary artery pressures but we should not hopefully see any left ventricular diseases in other words do they have a decrease to left ventricular ejection fraction any valvular heart diseases any diastolic dysfunction we shouldn't see any of that but we may see some right heart strain and dysfunction so what do we see no valvular heart diseases is normal EF normal diastolic function but the increased right heart pressures are present supporting right heart strain telling us again there's no type 2 cause and then again there's no obvious type 1 cause because we've ruled out HIV portal pulmonary hypertensions Scleroderma and lupus could it be like again an idiopathic or heritable cause it could be let's go to the next one so if it's not type 2 because we ruled that out with an obvious EKG no acute MI no kind of like heart congestive heart failure that's present there as well no valvular heart a disease or abnormalities to support a type 2 cause is it a Type 3 so look for any kind of obstructive or restrictive pulmonary diseases check your pfts if we check the pfts the patient has a normal FEC a normal total lung capacity but they have a low dlco that could support a pulmonary vascular cause but it doesn't support in any way shape or form a specifically an obstructive lung disease if they have a normal FEC a normal total lung capacity this does not support an obstructive this does not support any type of you know in general a restrictive lung disease so as you can see here if we check the PF disease and we go through this process here nothing is really popping up as an obvious cause especially with the lungs next thing is we gotta roll out if it's not that could it also be anything obvious in the chest so do they have any kind of other weird lung abnormalities that I'm missing so make sure that there's nothing acute there's no pneumonia no pneumothorax no nothing weird here that I'm missing and I don't see anything when I get a chest CT guess what I do see holy crap that right side of the heart is huge and my pulmonary artery is bigger than I can even imagine this thing's gargantuous so there's dilation or pruning of the large pulmonary artery here so definitely something again supporting High pulmonary artery pressures and supporting a lot of like pulmonary vascular resistance as well so again more supportive of a pulmonary artery hypertension that has no type 2 type 3 cause at this point let's go to a VQ scan this will help me also I could do a ctpa but VQ scans have actually been shown to be more beneficial because I want to rule out a Type 4 cost is there any kind of chronic pulmonary emblem lie that are present that are causing ventilation perfusion deficits here because that also can cause increased pulmonary vascular resistance and I do a VQ scan and if I did a ctpa I would show no evidence of any pulmonary emboli with ventilation perfusion deficits the other thing I could do is I could also you know make sure that I rule out any other kind of causes here that there's nothing else weird within the you know heart and I could cat them so I could stick a Swan's gonze catheter a right heart Catherine thread it up into the pulmonary artery and then in inflate a balloon to calculate the pulmonary capillary wedge pressure and to calculate the pulmonary artery pressures now generally whether it's type 2 type 3 Type 4 type 5 type 1 all of them are going to have mean pulmonary arterial pressures that are high because they all can cause high pulmonary artery pressures any one of the types of pulmonary hypertension so the pressure is going to be high if the mean pulmonary artery pressure is greater than 25 we already know that they have pulmonary hypertension but the big thing is use that time also to rule out a left heart cause so again make sure that there's no other type 2 cause here and check the pulmonary capillary wedge pressure if the pulmonary capillary wedge pressure is less than 18 it tells you that the left heart is not the problem so again we've ruled out with Echo catheterization and EKG type 2 costs we viewed pfts chest x-ray CT scan to rule out lung causes type 3 causes we've utilized VQ scans and if we needed to a CT pulmonary angiogram to rule out Type 4 causes and we could also use a chest x-ray and a CT scan of the chest to rule out things like type 5 causes in other words is their sarcoidosis is their mediastinal mass is there a big granuloma that's compressing onto the pulmonary vessels and causing increased resistance so we could also rule out type 5 causes and so we've gone through here and we've even gone through the type 1 causes is it not idiopathic in other words is it HIV polar pulmonary hypertension is it some type of problem with a lupus vasculinity of some type or anything like that and we've ruled all those things out so this really supports an idiopathic pulmonary artery hypertension the next thing I need to be able to determine though is is it a Vaso reactive type 1 pulmonary artery hypertension so I give them a calcium channel blocker like nifedipine when I give them nifedipine what I expect is is it to dilate the pulmonary arterial system and reduce the pulmonary artery pressure and if I reduce the pulmonary artery pressure I should see evidence of that and so that's what I need to do I need to give them nifedipine and then what I got to do is I got to determine did it actually help if the mean pulmonary artery pressure does drop more than 10 millimeters of mercury that's a positive sign and on top of that if the total value so let's say that the norm the number was 60. and it drops more than 10 maybe it drops down to 30. is the number less than 40 and did it actually have no change or increase in right ventricular cardiac output in other words did it drop the pressure and potentially improve the right ventricular outflow if it dropped the pressure and it dropped the absolute pressure more to less than 40. in other words took the pressure dropped it more than 10. then the absolute pressure that we have in the pulmonary circulation is less than 40 and if the pressure in the pulmonary arteries are lower did it improve the right ventricular cardiac output if it did this shows a Vaso reactive pulmonary artery hypertension that would benefit from a calcium channel blocker so we would continue nifedipine if that was the case if they failed the vasoreactive test then we would use other types of analogues so nitric oxide analogues prostocyclin analogues endothel and receptor antagonist types of drugs so processicular agonists we would use things like illoprost epaproxenol tripoprosanol and if it's pde inhibitors these are going to be things like sildenafil to delophyll and if it's endothel and antagonist this would be like bosentan and R ambrozantan okay so these were the drugs that I would use if it's not a Vaso reactive type now here's the last thing that I want you to do I want you to just completely quickly recap and like match the appropriate pulmonary artery hypertension to the potential etiology so if I said idiopathic or acquired so for example Scleroderma HIV lupus it was some type of hepatopulmonary syndrome or anything like that what would you say it's type 1. if I told you it was from pulmonary emboli so it's some type of like chronic pulmonary emboli or VTE process then you would say type three no that's type four type four type three is the lung that's lung problems if it's from pulmonary vascular compression from outside in the mediastinum like a granuloma a tomb or a mediastinal mass of some kind and it's compressing on the pulmonary vessels and increasing the pressure proximal to that that would be a type 5. if it's left heart disease like heart failure valvular heart disease that's type 2 and then again if it's any lung disease like COPD asthma interstitial lung disease of any type that's type three okay so idiopathic or acquired type 1 left heart disease type 2 lung disease type 3 pulmonary emboli are kind of like chronic kind of blockage of the pulmonary vessels would be Type 4 and then again pulmonary compression pulmonary artery compression from the mediastinum or outside structures type 5. all right my friends that covers pulmonary hypertension I hope it made sense I hope that you guys enjoyed it and as always until next time foreign [Music] [Music]

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Length: 59min 31sec (3571 seconds)

Published: Fri Apr 28 2023