Transcript for:
Understanding Cardiomyopathy and Heart Failure

okay so today we're going to be going over cardiomyopathy so it's going to be dilated restrictive and hypertrophic cardiomyopathy this is from the cardiology section for the pants which is about 13 so it's a pretty big chunk of the pants you definitely need to know these things and i just wanted to thank everybody for the comments the likes and if you don't mind subscribing i'm going to be coming out with new content pretty much every week and thank you so much again for the the likes and the comments i really do appreciate it so really quickly before we get into the cardiomyopathies i just wanted to refresh everybody um on heart failure i'm sure you're all probably pretty familiar with this but really quickly let's just go over just in case you've forgotten some of the things so you have systolic and diastolic heart failure so systolic is going to be when the ventricles are going to be thin weakened they're going to be stretched out and they're going to be overly compliant which means that they're going to not pump very well they're not strong they're just kind of baggy and hanging around the muscle is not very strong so you're going to have a decreased ejection fraction so the blood that's pumped out of the heart in each beat is going to be decreased sometimes dramatically decrease so you're not getting enough blood out to the body it's all backing up in these ventricles and then what you hear in auscultation typically with systolic heart failure is something known as an s3 or an s3 gallop and you hear this noise because the atrium is splashing the blood down against these baggy thinned out ventricles and when the blood hits these baggy ventricles you hear something known as an s3 gallop now diastolic is kind of going to be the opposite so the ventricles are okay the muscles work okay they pump fine but they're going to become stiffened or thick and they're not going to relax the way they should and if the ventricles aren't relaxing blood can't fill the ventricles so it's all going to um back up into the atrium the same way because they can't get into the ventricles the ventricles won't open they're stiff they're non-compliant so as far as pumping the ejection fraction is normal the the part is actually pumping okay so ejection fracture will normally be normal in diastolic uh heart failure but again they're not opening the way they should not enough blood is getting into the ventricles and then on diastolic heart failure you'll hear an s4 so the way that i remember that you're gonna hear an s3 on systolic heart failure is um actually i'll go over that in one second let me just a really quick picture so systolic and diastolic or failure this is just a quick diagram so you can see systolic is gonna have these thin stretched out chambers and then diastolic you're gonna have these thickened and sometimes stiffened ventricles so they don't open you can't get as much blood into the ventricle so these are the kind of like the polar opposites of each other and that's what it looks like on a diagram so again what i was saying before so s3 the way i remember s3 is going to be with systolic heart failure again remember like i just went over systolic heart failure is these thin wide open baggy ventricles and you hear an s3 on the systolic heart failure so the way i remember that if you take a look at a 3 and you rest it on its back what does that look like looks like the bottom of a heart and it looks like these wide open baggy ventricles so that's how i remember you hear s3 on systolic heart failure and then if you remember here s3 you know what's the only other one that would be s4 so that's diastolic so s3 big wide open baggy ventricles hurt on systolic heart failure and then you take a look at a three on its side and that's kind of looks like those baggy ventricles to me so now one other thing i wanted to go over really quick is the difference between left and right sided heart failure so left-sided and right-sided heart failure all you have to think about is where's the blood coming from and wherever it's coming from because of the heart failure it's going to be backing up into that area and you'll have symptoms related to that area so left-sided where's the blood coming from where's the blood coming from the left side of the heart it's coming from the lungs via the pulmonary vein so the blood's not pumping out or it can't get into the ventricles it's going to back up and it's going to go back into the lungs so you're going to have dyspnea pulmonary edema congestion and then how do you remember that left-sided heart failure is going to back to the lungs you're going to have long symptoms or pulmonary symptoms remember left-sided left l stands for lungs so right-sided flu is going to back up where it came from where is that that's the ivc and the svc so you're going to have pretty much the rest of the body you're going to have symptoms in peripheral edema in the legs you're going to have jugular venous distension you're going to have hepatic congestion it's all going to back up into the gi system so r the way i remembered it is r stands for rest of the body because it's everywhere else everything coming from the ibc and the spc which is pretty much everything except for um you know so you'll see the lungs on the left side but the rest of the body is going to be right-sided heart failure so that's why you remember those two and the symptoms you'll have and then just a really quick diagram to kind of just reinforce what i just said so right side it's a really simplified obviously you'll have an spc and svc separately but right sided if it's backing up here from the ventricle into the atrium it's going to go back to the rest of the body left side if it's backing up at the ventricle into the atrium it's going to go back to the lungs via the pulmonary vein so really quick review of heart failure so let's move into dilated cardiomyopathy and go over that so dilated cardiomyopathy is going to be when you have these dilated um baggy ventricles which is going to lead to impaired contraction so your systolic function is going to suffer these ventricles are dilated exactly like it says they're stretched out if you think of like a piece of gum when you stretch it out it gets really thin or plato it's the same thing that's happening to these ventricles and because of that become really weakened and you're going to have impaired contraction now it's the most common type of cardiomyopathy 90 sometimes as high as 95 percent of all cases of cardiomyopathy are dilated so this is the one that's really important because it's the most common the one that you'll most commonly see in real life ventricles again like i said are baggy stretched out and weak that's what this looks like so normal heart here you can see the normal diameter of the ventricles and dilated cardiomyopathy it's stretched out it's wide open and you can see the ventricles are very thin so the muscle is going to be weak that blood's not going to pump out the way it should it's going to get backed up here and then of course back up it to the rest of the body depending on the side that's most prevalent sometimes both sides now as far as etiologies your most common etiology is going to be idiopathic it's about 50 percent of the cases most common cause and there's a few other ones you need to know as well these are pretty important because i do remember these coming up pretty frequently so alcohol and cocaine use so substance abuse can cause dialytic cardiomyopathy doxorubicin which is a chemotherapy agent is another important one and it's funny because it really causes very few cases of dilated cardiomyopathy probably like less than one percent but it comes up very often on your exam so just know that one um it's one of the ones you probably won't see in real life but you'll definitely see on your exams infections specifically viral like oxy virus is another common cause of dialytic cardiomyopathy and then finally vitamin b1 deficiency can also cause dialytic cardiomyopathy so these are there's a lot to know but i really think you should know them and the way that i came up with this is i'm actually pretty proud of this mnemonic but um so dilated cardiomyopathy is uh the only cardiomyopathy that starts with the d so if you think of dilated cardiomyopathy think of a d and think of your sixties of dialytic cardiomyopathy as far as your causes so the first one drinking alcohol second one to know or don't know which is going to be your idiopathic your third one deficiency as in vitamin b1 deficiency your fourth doxorubicin your chemotherapy agent your fifth drugs as in cocaine and then finally disease specifically viral disease so those are your sixties of dialytic cardiomyopathy and that'll help you remember the um the etiologies remember idiopathic or dino is going to be your most common cause now moving on to the the manifestations these patients are going to be in systolic heart failure so you're going to have symptoms of heart failure and both left and right sided heart failure so just like we went over before if it's left sided they're going to have pulmonary symptoms so dyspnea cough wheezing if it's right-sided pedomegaly jugular venous distension peripheral edema all of the symptoms on the right side so the legs may have all that edema the pitting edema they may have the jugular venous ascension which you'll see in the neck you'll certainly be doing that in your oscis jvd and you'll just see symptoms in the rest of the body so nothing really specific just symptoms of heart failure in general both right and left side one thing that you will also see on a physical exam or here on a physical exam is going to be your s3 gallop and that's pretty important for you to know that's uh one of the ones that is uh when you see an s3 you definitely should be thinking of systolic maybe you see other symptoms alongside it you know that's a pretty hallmark finding for dialytic cardiomyopathy so remember s3 gallop as well remember again that's you're splashing down on this the blood splashing down on this compliant overly compliant ventricle so those are going to be your findings on exam now as far as diagnosis you're going to see in all the cardiomyopathies we're going to pretty much diagnose the same way that's going to be with echocardiogram now echocardiogram is for dilated cardiomyopathy you're going to see three main things here so first thing obviously you're going to see ventricular dilation as the name states second thing that you're going to see is thin stretched out ventricular walls so much thinner than they normally should be and then finally you're going to have a decreased ejection fraction because remember diluted ventricles stretched out very weak they're not pumping out the blood the muscles are definitely diminished so you're going to have decreased ejection fraction less blood coming out than you should sometimes as low as in the 30 percent 40 range normal being of course 50 60 ejection fraction now this is what it looks like on an echo we kind of shaded it here so you get a better idea sometimes they're not very easy to read if you're not familiar with them so this is a normal size of a ventricle here and then you can see on this side this is this wide open big ventricle thinned out walls and just really dilated so that's what it would look like on an echo now you can also do an x-ray it's not going to be very specific because a lot of things can cause some of the symptoms you'll see or some of the x-ray findings but you'll see cardiomegaly plural effusion and then an x right here you can see this why this huge heart it's almost taking up the entire chest and then plural effusion so on a chest x-ray you should see these pointy costophrenic angles they should come to a nice point here but on this side you can see it's blunted that's kind of it's uh tapered off here that's because this is all fluid filling up here so it doesn't have that nice um tapered off appearance it's kind of blunted here on that side so that's the pleural effusion and of course this is your cardiomegaly this enlarged heart which is quite large on this person and you can also do an ekg as well again not very specific but you're going to see arrhythmias sinus tachycardia and those are your ways to diagnose but your main thing the main thing you need to know is your echo and of course remember what you're going to see on echo so you'll be able to diagnose it at least for the exam and then you know in real life as well so treatment you're going to treat these patients like you would any other patient with systolic heart failure so how do you treat systolic heart failure well there's four main classes or medications that you need to know that reduce mortality and heart failure so what are those ace inhibitors is going to be your number one medication class for heart failure so that's going to be your prills your lacina pro catapro et cetera second line really is going to be your beta blockers um hydrolyzing plus nitrates specifically isosorbidity is normally the one used with hydrolyzing and then spironolactone now i put a little asterisks here because you need to know all four of these classes or these medications are proven to decrease mortality in patients with heart failure because there's a lot of meds you can use for heart failure but not all of them decrease mortality but these are the four that you need to know now there are some newer classes of meds i'm not going to go over them now because i don't think it's going to be on your boards they are fairly new but there's one called intresto which is similar to ace inhibitors it's a neprilacin inhibitor and it works really well it can actually reverse some of the causes of heart failure but it's pretty new i don't think it's going to be on there and then also sglt2 inhibitors if you remember from diabetes those are some of the diabetic medications that flush the sugar out through the kidneys these also work for heart failure and they work really well but again these are newer classes probably not going to be on the boards but you should know that for real life because one day you're going to be practicing medicine so those are something to know as well as a side note but these are the four that you really need to know and then also go over the ones they use for symptom control so these do not decrease mortality but they make your patient feel a lot better so they are important so loop diuretics furosemide it's going to pull all the fluid off help these patients feel a little bit better get the swelling off their legs and then digoxin increases the um the contractility of the heart so it helps it beat better and that's important because remember you have this weakened heart muscle these weakened ventricles so digestion digoxin helps the contractility of the heart so these are going to help them feel better but it won't decrease mortality so make sure you you're able to differentiate the two and then finally in patients with really low ejection fraction normally 35 or less um history of cardiac arrest v fib these patients are going to need an icd so implantable cardiac defibrillator so this is really going to be your last line when these patients are really getting sick and you need something a little bit more invasive to help them now of all of these what i think you need to know is those four classes those four medications um so how do you remember those because those are the ones you really need to know that they're going to ask you how do you remember that it's bash the heart to make it beat harder so just think of your heart's not beating hard you just kind of like bash it and say wake up start beating harder so bash stands for beta blockers ace inhibitors spironolactone and hydrolyzing plus your nitrate of course so remember that that's really the ones you need to know for the uh for real life really and for your board so bash the heart to make it beat harder those are the ones with proven mortality benefit in patients with heart failure okay so what do you need to know i think you need to know that this is a systolic dysfunction that leads to a weakened dilated ventricles you need to know your 6ds the etiologies that i went over because those all may come up you need to know s3 is a hallmark finding on physical exam and then you need to know your bash for treatment that we just went over so those are the things that i really feel like you need to know if you forget everything else remember those four things now moving on to restrictive cardiomyopathy so this is kind of going to be the opposite so restrictive cardiomyopathy is caused generally by an infiltrative disease so whether it's amyloidosis hemochromatosis that leads to the stiffening of the ventricle so the ventricles pump okay but they're going to become stiff and when they become stiff they're not going to relax properly and if they don't relax and they don't open up on diastole they're not going to be able to be filled with blood so you're going to have this diastolic dysfunction so they're going to beat okay but they're going to become really stiff they're not going to open up on diastole and that means you're not going to get enough blood in sometimes you can wind up backing up into the rest of the body so that's restrictive cardiomyopathy and this is what it looks like here so here's your normal heart and then restrict if you have this thickening sometimes thickening not always more of a stiffening i'm sorry so not necessarily thickened but more of a stiffening of these of these heart it can become slightly thickened though so that that is important to know but it's mostly just a stiffening of these of these ventricles that allow them to not relax or so you're gonna have problems with your diet or your diastole now it's rare it's only about one percent of the cases of cardiomyopathy compared to dilated which was about 90 to 95 so this one's not going to be very common in real life certainly doesn't mean you don't need to know for the boards but you need to know they probably won't see it very often in real life as far as etiologies your most common cause is going to be amyloidosis and then other infiltrative diseases are also really common so hemochromatosis sarcoidosis now let me explain really quickly why you see these specific etiologies and restrictive cardiomyopathy so what happens is for instance with amyloidosis if you've gone over this before you'll remember amyloidosis is abnormal deposits of a protein called amyloids so they can deposit in the heart they can deposit in the kidneys they can positive a bunch of organs but what happens when they deposit in the heart these amyloid proteins stiffen the ventricles and it's the same thing in hemochromatosis and sarcoidosis so sarcoidosis you're going to have these granuloma deposits that's going to make the heart rock hard hemochromatosis you're going to have these iron deposits that's also going to make the the ventricles really hard so generally what you need to understand is all of these infiltrated diseases all these um diseases that basically deposit all of these abnormal things in the heart are going to lead to it hardening and getting stiff and not being able to relax so that's the main idea here there's some other causes as well not as common but um radiation metastatic disease chemotherapy and again because a lot of these like the radiation and everything can cause scar tissue and um and fibrosis in the heart so a lot of the radiation chemo and those types of things so similar idea but they're just not as common now that's going to be your most common etiologies amyloidosis and your infiltrative diseases the way i remember that is going to be amy i'm sorry not amy amy has restrictive cardiomyopathy so amy has restricted cardiomyopathy what that stands for is amy stands for amyloidosis so first three letters in amyloidosis is amy so amy has and then has stands for hemochromatosis amyloidosis a second time and that helps you remember that amyloidosis is your most common cause overall because it comes up twice in this mnemonic and then finally the s stands for sarcoidosis so amy has restrictive cardiomyopathy amy stands for amyloidosis and then the has stands for hemochromatosis amyloidosis a second type i'm sorry and then sarcoidosis so that's going to be the way you remember the etiologies for that now moving on to the clinical manifestations so these patients are going to have heart failure but it's going to be diastolic heart failure now it's going to be more common for them to have right sided heart failure symptoms so again remember what's right sided right side will left eyes as well but right side you're going to have the hepatomegaly jugular venous distension peripheral edema and then kusmal sign also which we'll go over in a minute which is pretty important for this and then left-sided failure of course is dyspnea cough wheezing but right side is what you're going to see more commonly in restrictive cardiomyopathy so these are the things you should look for in restrictive cardiomyopathy and let's go over cool small sign in case you're not familiar with it so acoustimal sign is is uh increased jugular venous pressure with inspiration now that's the opposite of what normally happens in the body so when you take in a deep breath the lungs expand obviously and you produce this negative intrathoracic pressure which decreases the pressure in the in the chest and what happens when you decrease that pressure all of the blood rushes back to the heart and it fills the right atrium goes into the right ventricle and um so you're going to have all this filling and what happens then is the internal jugular vein which all of that blood was full with is going to rush back to the heart and you're going to have this decreased jugular venous pressure but in a patient with restrictive cardiomyopathy as the blood is rushing back because of this decreased or this negative intrathoracic pressure it's rushing back to the heart but what happens is because the ventricle on the right side is not opening up it's actually backing up into the right atrium and then eventually because all the blood is rushing back there it's going to back up right back into the internal jugular vein and you're going to have this increased jugular venous distension and that's what um cool small sign is and that's what that's actually going to look like you're going to have this increased jugular venous pressure with inspiration which is the opposite of what should be happening in a normal patient so it's it's all backing up into the um the jugular internal jugular vein so that's what that looks like on a patient and that's why you measure your jvd on on exam for these patients so that's what you're going to see and that's a pretty common sign in restrictive cardiomyopathy so overall again remember heart failure symptoms more common to see right-sided heart failure symptoms and patients with restricted cardiomyopathy now diagnosis you're going to do again your echo just like we did with all of them and echo is going to show initially probably a normal maybe slightly thickened ventricles and then you're also going to have diastolic dysfunction so you're going to have decreased filling of the ventricles your ejection fraction will be normal as i went over before it's a little bit misleading because you're still not getting a lot of blood out but because this is a fraction it's a percentage of how much is going in how much is coming out less is going into the ventricle so even though less overall is going out this is a fraction so again wastion over the exam is going to be uh typically a normal ejection fraction but um you will have this diastolic dysfunction and the important thing that you need to know is that you're going to have dilation of the atrium so atrial dilation and why does that happen well the atrium is pumping blood into the ventricles but it's doing so against resistance these ventricles are not relaxing so what happens is when they push against resistance they're eventually going to have this backed up pressure and they're going to start to dilate so that's one of the most common things you'll see on an echo of restrictive cardiomyopathy so these big wide open atrial atrium so dilation you'll see on echo now the most definitive way to actually diagnose restrictive cardiomyopathy is um with an endomyocardial biopsy so that's actually going in taking a piece of the heart and doing a biopsy on it it's not commonly done because it's obviously much more invasive and you can kind of diagnose from the the echo but if you do it and you do happen to find your most common cause which is going to be amyloidosis what you're going to see is this apple green birefringence on staining it's called congo red staining and why do i mention something so ridiculous well it's because it does come up in the exams i've seen it in questions so if you ever see a biopsy positive for apple green birefringence think amyloidosis and again amyloidosis isn't just in the heart so you may see in the kidneys other places as well so remember that you ever see apple green biopharyngeans you should be thinking amyloidosis this is what it actually looks like so this is just the exam but this is just what it looks like under microscope but this is actually the staining with it's called congo red and you can see this apple green birefringence is what it's called here so remember that because it definitely may come up it's one of those weird things that they like to ask so endometrial endomyocardial biopsy is going to be your definitive diagnosis but generally you're just really going to do an echo now as far as treatment the main thing that you need to address here is the underlying cause so is it amyloidosis is it hemochromatosis you're going to do iron chelation whatever the cause is treat the underlying cause and generally this will improve um you can treat them with diastolic car failure meds so beta blockers calcium channel blockers furosemide but really what they're looking for here the answer is going to be to treat the underlying cause and then everything else will improve after that so that's really the main thing you need to know for that now what do you need to know um you need to know this is diastolic dysfunction so it's a filling defect these ventricles are hard they're not opening they're very stiff they're non-compliant and you need to know the most common is amyloidosis remember amy has restrictive cardiomyopathy and then remember the treatment is generally just to treat the underlying cause so that's restrictive cardiomyopathy not a lot to know there now let's move into the last one but i would say probably the the most popular of all of these to be asked and it's hypertrophic cardiomyopathy so hypertrophic cardiomyopathy is a genetic disorder it's an autosomal dominant genetic disorder and you have these mutations in the sarcomere genes and what the sarcomere genes are responsible for is the contractile portion of the heart so you have a problem with the contractile portion of the heart and it's going to lead to this hypertrophy of the left ventricle and more specifically the septum and when you have this hypertrophy of the septum you're going to lead to it's going to lead to diastolic dysfunction because you're going to block your outflow to the the aorta and that's what you develop in hypertrophic cardiomyopathy you have this outflow obstruction so the septum is going to have this hypertrophy the left ventricle and it's going to block the outflow to the to the aorta now as far as clinical manifestations there's a wide range and really quickly let me just show you what a diagram looks like here so this is going to be your normal heart blood's going to flow from the left ventricle right into the aorta here through the aortic valve now in patients with hypertrophic cardiomyopathy you have this thickened ventricular septum the left ventricle is going to thicken as well so when blood is trying to get out here because you have this big puffed out hypertrophied septum here it's going to block the outflow now this is in every patient some patients actually don't have an outflow obstruction and they're asymptomatic they don't have problems but it is slightly enlarged here and then some patients have full-blown hypertrophy septum and these patients can actually have this can actually cause cardiac failure and death from ventricular fibrillation and things like that which we'll go over shortly so that's what that looks like and that's why you have these symptoms now as far as clinical manifestation again like i said depends on how severe the outflow obstruction is so there's a wide range it can be anywhere from asymptomatic to sudden cardiac death so some patients again like maybe asymptomatic dyspnea will be your most common symptom just due to the lack of blood flow to the rest of the body so um you know hypoxia angina is another common one you're not getting enough blood flow fatigue pre-syncope or syncope if they're exerting themselves or dehydrated and then sudden cardiac death is a possibility if they do have exertion dehydration which i'll go over the reason for some of those things in a minute now so those are some of the clinical manifestations again wide range of symptoms that you may see on physical exam this is really important i would say probably this is the most important thing that you need to know so physical exam you're going to have this harsh systolic murmur that's best heard at the left sternal border so you really need to know this one and it's not very specific just a systolic murmur harsh systolic murmur the way you differentiate this from other ones is with some of your your tests that you'll do some of the the exam things that you'll do with the patient so in a normal murmur most other murmurs aortic stenosis and some of the other ones when you have decreased venous return less blood coming to the heart normally the murmur's going to decrease because you have less blood going to the heart you have less blood pumping through this stenotic valve or less regurgitation because there's less blood flow but in patients with hypertrophic cardiomyopathy if you have decreased venous return like when a patient stands on the blood rushes from the heart or of all solvent maneuver this is actually going to increase the intensity of the murmur and the the same with the opposite so if you have increased venous return so squatting leg raise blood rushing back to the heart blood's coming back into the heart the murmur is actually going to decrease which again is the opposite of most other murmurs and that's how you differentiate this from other ones so more blood coming to the heart decreased intensity of the murmur which is opposite of aortic stenosis and a lot of the other murmurs and then decreased venous return less blood coming to the heart it's actually going to make the murmur worse and again the other ones are going to be the opposite less blood normally coming to the heart less intensity of the murmur and um so again this is the opposite of other murmurs and you're also going to hear s4 and auscultation so the reason i wanted to go over is because when you have hypertrophic cardiomyopathy as i showed you before you have this obstruction and if you think of if you can kind of think of that diagram i had before um let's pull it up here again so you have the heart here the normal heart but this is the one we're looking at the hypertrophic cardiomyopathy so you see this obstruction here now if you fill this heart up with blood what's going to happen here is the blood is going to push this out a little bit push this out a little bit and what's going to happen when you fill this with blood and these both push out to the sides this obstruction is going to decrease so you have more blood in the heart the murmur is going to get better now you have less blood there decreased venous return you're going to have less blood so this is going to kind of collapse on itself and this obstruction will get worse so that's the reason why less blood to the heart is actually going to increase the murmur you're going to have more obstruction here and then more blood coming back to the heart increased venous return stretch this out the obstruction will lessen and the abs and the murmur will actually improve and that's also the reason why which i'll go over later is why patients who become dehydrated or take medications that decreases your venous return or decreases the preload will actually have increased the severity of the symptoms and increase in the obstruction so again like i said before also s4 and auscultation which is not as important but you do need to know you'll have that on a physical exam as well as far as diagnosis surprise surprise we're going to do an echo now you're echoing these patients you're going to have a diagnostic criteria left ventricular wall of 15 millimeters or more or in a patient with history of hypertrophic cardiomyopathy of family history you only need to have 13 millimeters of hypertrophy left ventricle or the septum uh more it's actually the the left ventricular wall that you're going to be looking so either 15 or more in a patient without history or 13 or more in a patient with family history of hypertrophic cardiomyopathy on echo to diagnose you can also do an ekg it's very sensitive about 90 percent of patients with hypertrophic cardiomyopathy will have an abnormal ekg but it's not very specific so sensitive but not specific you'll have some repo changes left axis deviation and left ventricular hypertrophy so it's good to do ekg for screening especially if you're doing like a sports physical on young children who are going to be going out and playing sports for the first time to screen but to actually make the diagnosis you're going to need to do an echo um so treatment treatment for hypertrophic cardiomyopathy the first thing that you always want to give these patients is going to be a beta blocker why do you want to give them a beta blocker well as we went over before you have this obstruction and you want more blood to come to the heart you want that heart to fill up to decrease that obstruction you want more blood to come into the heart so you can actually pump more out so how do you do that while you do that let's actually throw the second one on there too so you do that with either beta blockers or calcium channel blockers beta blockers are preferred just due to their side of side effect profile and things like that but the reason why you give beta blockers or non-dihydropyridine calcium channel blockers is because these both they slow the heart rate and if you have a heart that's beating a hundred times a minute how much blood you think is going to get into the heart not a lot it's pumping really fast so if you slow down the heart rate you have more time in diastole more time for the blood from the atrium to get into the ventricles and pump out more time to release that obstruction to fill up the heart and lessen that obstruction that's why you want to use these um these medications like beta blockers and calcium channel blockers now beta blockers you use and then calcium channel blockers you're only going to use your non-dihydropyridine so non-dihydropyridine medications are going to have an effect on the av node and the sa node as well they're going to slow av no conduction which slows the heart rate the dihydropyridine which are going to be your amlodipine and those meds are not going to slow the heart rate they don't have an effect on the av node so only calcium channel blockers non-dihydropyridine and the way that i remember the non-dihydropyridine which is going to be diltiazem and varapamil and i've never forgotten this one this one's um and you'll always see it's going to be either diltiazem or varapam these are the most commonly used nondihydropyridines i highly doubt you'll see anything but these the way i remember it is non-dihydropyridine again affect the av no conduction they slow the heart rate so they decrease velocity of the heart by decreasing av no conduction decreased velocity dv diltiazem varapamil there's your non-dihydropyridine calcium channel blockers there you go you won't forget which ones those are so if you have a bunch of they say which medication would you use there's no beta blockers on there they just list calcium channel blockers they have amlodipine varapamil and then some other dihydropyridines you think of decreased velocity and then look for diltiazem of verapamil that's how you remember because you'll see other calcium channel blockers and other questions as well and you'll never forget that one so now if patients are refractory to refractory to pharmacologic therapy there are a few more invasive options and those are going to be either a septal myomectomy which is quite simply you're going to cut out that porsche portion of hypertrophied septum that's all that is you're just cutting it out and you're going to lessen the obstruction by doing that and the other option is something called an alcohol septal ablation which is actually when they take um it's about a 96 ethanol and they um they do it through a catheter procedure the catheter goes all the way down right into the septum and then they release this alcohol into the septum now the alcohol is actually toxic to the heart um it kills the the um the cells of the um the myocardium it causes them to shrink and and die and because of that because of the death of these um these myocardial cells um it's actually going to cause the the hypertrophy the part of the heart to actually shrink and decrease the obstruction so a lot of times it can be refractory it needs to be repeated but it is less invasive and it is another option for patients refractory to medication so basically you're spraying ethanol onto the septum of the heart killing the heart cells and decreasing this obstruction by decreasing or killing off the um the um the cells of the heart here so that's pretty much all that is so those are your options for for treatment now one last thing that i wanted to go over is in hypertrophy cardiomyopathy exacerbation of the obstruction is seen in both increased contractility of the heart and dehydration so because of that what do you want to avoid in these patients you want to avoid a few different medications you do not want to give these patients digoxin why don't you want to give digoxin well digoxin increases contractility of the heart that's going to make the obstruction worse why do you want to avoid nitrates and diuretics both of those increase or i'm sorry decrease preload so it decreases the blood coming back to the heart which again remember is going to close off that left ventricle it's going to make the obstruction worse so that's why you don't use nitrates and diuretics in the same way that dehydration is also going to decrease preload and also going to make the obstruction worse and that's the same reason avoid dehydration and exertion exertion is going to cause increased contractility of the heart dehydration decrease your preload same way the nitrates and diuretics work so anything that's going to decrease the amount of blood coming back to the heart or increase the contractility is bad so no digoxin no nitrates no diuretics and these patients really should not be exerting themselves and they should not become dehydrated those are the things you really want to avoid in children with or adults with hypertrophic cardiomyopathy so those are the main things that you need to know that you need to avoid these patients now what do you need to know you need to know this is an autosomal dominant dominant genetic disorder that leads to mutations in the sarcomere genes you need to know it's a harsh systolic murmur that's best hurt the left sternal border and remember increases with decreased venous return less blood coming back to the heart makes the murmur worse and decreases with increased venous return so it gets better when you have more blood coming back to the heart and then finally remember your first line pharmacologic agent is going to be your beta blockers so that's what you really need to know with hypertrophic cardiomyopathy and let's take it home with uh test your knowledge we'll do five quick questions to see what you remember from what we just learned so what is the most common type of cardiomyopathy that is going to be dilated cardiomyopathy over 90 percent of the cases what are the four known classes or medications known to reduce mortality risk in patients with heart failure i really hope you remember this one because i worked hard on mnemonic and that is going to be your bash meds so beta blockers ace inhibitors spironolactone and hydralazine with a nitrate third question what is the most common cause of restrictive cardiomyopathy again you'll hurt my feelings if you don't know this one because i think that mnemonic was pretty pretty good so that's going to be amyloidosis remember amy has restrictive cardiomyopathy so amyloidosis most common cause of restrictive cardiomyopathy what is coo small sign so remember cool small sign from the right-sided heart failure it's going to be an increase in jugular venous pressure with inspiration so opposite of what a normal patient would have and then finally what is first-line pharmacologic agent for treatment of hypertrophic cardiomyopathy we just went over that one and that's going to be beta blockers so those are your questions hopefully you got those all right and i hope that helped um it was a little bit longer than i would have liked it to be but i think it's pretty important information and i really wanted to make you sure you understood everything so thank you so much for listening thank you for all the likes the the new subscriptions um to the channel i really do appreciate everything in the comments and um good luck on your pants your panerai your eors and good luck in pa school