 Okay, so now we are going to continue with hypertrophic cardiomyopathy. Hope you have survived the first part of the lecture. So the second part is basically going to... We are going to be talking about hypertrophic obstructive cardiomyopathy. But before we start with that, we are going to talk about what kind of inheritance we are going to have in a patient that has hypertrophic cardiomyopathy. So as you well know, the most typical... It's autosomic dominant. It's mostly like common... It's the most common genetic cardiomyopathy with a 0.2% of prevalence. And it's the second most common cardiomyopathy after that kind of cardiomyopathy. So when we talk about the type of hypertrophy, it is very characteristic from hypertrophic cardiomyopathy to be asymmetric. The systolic function is almost always preserved or increased, but there is a 2-5% of end-stage hypertrophic cardiomyopathy where the LBEF is less than 50%. Despite say that in the group of the 95%, where they have a preserved systolic function, if you do a longitudinal strain into the ventricle, you are going to see that it's decreased. So they are starting to use longitudinal strain to follow up on those patients. The diastolic function is almost 99.9% of the time impaired, and there is diastolic dysfunction. And the morbid mortality for those patients normally is based on the amount of arrhythmias, cause of the pseudandem, and an overall mortality of less than 1% per year. Okay, so how many types of hypertrophic cardiomyopathy do we have? We have three types. We have the non-obstructive, where the LBOT, peak gradient, and that's the important part from our tichestanosis, that you need to differentiate. In our tichestanosis, we are measuring mean gradients. Here we don't care about the mean gradients, we all care about the peak gradients, okay? So if the peak gradients are below 30 mmHg at rest or provocations, those are considered non-obstructive. The moment that they are equal, or above 30 mmHg, so that's when you call it obstructive, hypertrophic cardiomyopathy, or HACCOM. The latent obstructive at rest is less than 30 mmHg, but with provocations, you actually increase the peak gradients up to 30 mmHg. Okay, so there are four types, not based on obstruction, there are four types based on the type of hypertrophy myocardium that you have. So the type one, which is the most frequent, is sigmoidal, and it's isolated to the basal anterior septal wall. The type two, which is the second most frequent, is the hypertrophy of the basal, but it's going to be not the anterior septal, this time it's going to be the anterior and the inferior septal walls, and there is going to be a sprain of the rest of the walls. The type three, which is 10% of the hypertrophic cardiomyopathy, is an extensive hypertrophy that only spurs the basal inferrolateral wall, and that's very important, because the basal inferrolateral wall is always going to be a spur in hypertrophic cardiomyopathy, and that's a very, like, pathognomic sign of this disease. In the type four, you have an isolated apical, which is only 10%. So this article from Dr. Martijn Bosch and colleagues that was published in 2008 is very well brightened, and it actually illustrates very nicely the four different types of hypertrophic cardiomyopathy that we have. Here we can see the septal, the basal anterior inferior septal, and the type three, which is spurs the basal inferrolateral. So it's important in this type of disease to measure that an apical hypertrophic cardiomyopathy and apical aneurysm can be missed without contrast in the echocardiography. It's not something that we do a bit like that we don't usually do with TE, but then that's the recommendation of the guidelines. You need to use contrast when you have an apical hypertrophic cardiomyopathy and when you have apical aneurysms to actually be able to this in. So what are the types of LVOT obstructions that we can find? So basically we can find subaortic obstruction, which is the 95% of the cases, or we can find a mid-cavitary obstruction, which is only seen in 5% of the cases. There is something that we call dynamic subaortic obstruction, which you can see in 25% of the cases of hypertrophic cardiomyopathy, and do you know what's the most frequent cause of dynamic subaortic obstruction in the LVOT? So you're right. So systolic anterior motion of the anterior mitral leaflet that gets sucked into the LVOT and it actually produces dynamic subaortic obstruction. So mechanisms of hypertrophic cardiomyopathy. So obstruction, as we were mentioning, is present in 30% to 50% of the hypertrophic cardiomyopathy patients. It can be due to obstruction, it can be due to venturi, to systolic anterior motion and in 5% of the cases, sometimes in the anterior lateral papillary muscle interposition. So when you get this kind of LVOT obstruction, sometimes the anterior mitral leaflet is going to be sucked into the LVOT and this is going to generate a posteriorly directed mitral regurgitation jet that can be moderate or severe and is going to produce turbulent flow in the LVOT, which instead of having the normal color between red and blue, you're going to have an intermittent color as yellow is with pixels all around the LVOT showing the flow acceleration in this area. There is always a systolic dysfunction and there are patients that have a tendency to have subendocardial ischemia because of the thickening of the ventricle and that predisposes them to have arrhythmias with ventricular tachycardia being one of the worst and they normally require the insertion of an AICB. Okay, so what can we do with TE? So from the American Society of Echo guidelines published in 2011, we are going to give you what are the tools that we can use in Hong Kong medicine TE. So the recommendation from the American Society of Echo is that ecographies, the non-invasive method of choice for the evaluation of functional normalities in Hong Kong. The recommendation continues with Doppler, Colorful Doppler and M-Mole. We will talk when to use those and the criterion for actually defining hypertrophic cardiomyopathy is going to be the ventricular hypertrophy which is usually going to be asymmetric. They require a wild thickness greater than 15 mm or no, well, not increase or any wild thickness but having a positive mood and gene for the hypertrophic cardiomyopathy. So in the table one, the guidelines reflect what do we need to assess. So you need to assess that there is hypertrophy, that you should measure the dimensions of the ventricle and the wild thickness, the septal, the posterior and the maximum. You should assess the systolic function, you should assess the right ventricle hypertrophy, the left atrium volume. This is going to be a problem in TE because the left atrium volume should be indexed and the best that correlates with the apical BU in the TTE on TE is going to be your fourth chamber and you are going to foreshorten in the left atrium. You need to assess the diastology on the left ventricle, the pulmonary artery systolic pressure, if there is or not dynamic obstruction, if there is mitral regurgitation. So there is many, many things that we need to do when we assess those patients and it's not only to measure the thickness of the patient. So as per definition in the American Society of the FECCO, anything higher than 11 millimeters in the left ventricle is hypertrophy, anything higher than 5 millimeters in the right ventricle is hypertrophy. So to assess the left ventricle walls, we put the same... I wanted to actually put again the same image. So in the fourth chamber view on the left side of the screen, the walls that we can see is the inferior septal and in the right side it's the anterior lateral. And why? Because when we cut the heart, the heart decided to be tilted and that's where at zero degrees angle should be giving you. As soon as we go to the two chamber view which is at 90 degrees, the wall that is in contact with the left lateral appendix is the anterior, the wall that is in contact with the coronary senors is the inferior. And as soon as we increase the plane to an aortic valve long axis, the wall that is in contact with the right ventricle is going to be the anterior septal and the wall that is distal to it is going to be the inferior lateral. So this inferior lateral at the basal segment is the only wall that is always a spur in hypertrophic cardiomyopathy. Okay, so examples, type one, sigmoidal. So you are expected to see in the anterior septal wall. Okay, and it's the most frequent one as you can see here. And it's limited to it. So if you go to the fourth chamber and you have it in the anterior septal, you are not supposed to see it there, okay? So type two. So the type two, we are going to have at the basal level of the anterior septal and the inferior septal wall. So you are going to be able to see it, not only in the long axis view of, not only in the long axis view of the, in the long axis view of the aortic valve, you are going to be able to see this hypertrophy in the fourth chamber view, okay? And this is the second most common. Third type. So this one is going to be affected an extensive hypertrophy in the whole heart. And the only thing that is spurring is the inferrolateral wall, which we are going to be able to see in the aortic valve long axis view. And the fourth type, which is only 10% too, is when the apical. This apical can be missed. Again, remember, if we don't give contrast, the same thing as apical aneurysms, okay? So just be aware of that. So evaluation of hypertrophic cardiomyopathy requires to determine if there is some with dynamics of active obstructions where we can see up to 25% of those patients, okay? And as you can see here, as soon as the mitral valve closes the mitral leaflets even touches the septum and occludes the LBUT. And I think it's a really good example on what happens. And if you can see, the artery wall is opening and it's flickering. At the same time that it's open because it's open and close, it's open and close due to the dynamics of our aortic obstruction. Okay, so measurements. What are the measurements that are required in those patients? So first of all, frame before the mitral valve closure. Okay, so what are the three measurements that we want to do? You need to make a four. That's the way to remember. So if you try to look up there. So the first measurement that you can do is the distance between the artery valve annulus and the impact lesion. You call impact lesion at a lesion that the anterior mitral leaflet does when it touches the septum. Okay, and that's one of the description. That distance is very important for the surgeon because he's going to perform the myectomy there. So he needs to know how far to go when he's doing the myectomy. Second really important maxim is how thick is the ventricle because the cardiac surgeon needs to know how deep he can go when he's actually performing the myectomy. Okay, and the maximum thickness is going to be measured from the impact lesion to the maximum thickness of the septum. And the last measurement from the artery valve annulus you are going to go to the apical stand of the septum bulge. Those are the three measurements that you want to know. So C-sept distance. So what's C-sept distance? So the C-sept distance was developed for mitral valve repairs. So they came up with this idea as we were measuring the regular measurements you need to go to NC study. The frame before the artery valve closes so then you want to measure the distance which is between the co-optation point and your septum. So if this distance is below 2.75 centimeters that implies an anterior displacement of the quantation so you need to have some after repair. And that's something very typical that we can see in a hypertrophic heart. So what we do is we measure that before going on pump unless that has not been validated for hypertrophic obstructive cardiomyopathy and we let the surgeon know because what we want to do is after the surgeon we want to know what's the space and what's the risk for this patient to develop some. So if you can actually do that you can actually do that that will give him an idea if he needs to do something about the anterior vital valve lipid before and not only a myectomy to try to avoid some after the myectomy is being performed intraperatively. This article was described by Dr. Ma with colleagues in 2015 in anesthesia and analgesia. So another classical design for hypertrophic for how patients is the use of M-mode through the leaflets of the aortic valve where we can see the leaflet flatter or notch in as a sign of early closure when you have subaortic dynamic construction on the LVOT. Here is an example of color flow Doppler and the typical pattern of flow acceleration in the LVOT with this turbulent flow with a combination of colors due to the flow acceleration and this posteriorly directed vital valve jet due to sauna. So LVOT's function as we mentioned before is normally going to be normal or increased but despite that is because you have a super thick muscle that is able to pump a lot. But when we say that we need to be aware that a very specific characteristic of hypertrophic cardiomyopathy is to have a reduction in the global in the global longitudinal longitudinal strain. So there are three types of strain we have radial, longitudinal or circumferential and the one that we are assessing in TE is the longitudinal which is the capacity of the LVOT to actually shrink and the more the better and this is being affected the longitudinal strains despite normally in hypertrophic cardiomyopathy patients. So diastology so the diastology is almost always in pair the recommendation as per the guidelines is to do the to assess the A wave of the metrolene flow remember that when we are assessing that you need to actually put your pulse rate Doppler at the level of the annulus of the mitral valve and what it's been seen is in this pathology you have a short mitral A so you do pulse rate Doppler through the pulmonary veins you have an increased amplitude in the AR of the pulmonary veins that those are two very typical signs for the hyperdiastology in hypertrophic cardiomyopathy. If you do if you use tissue Doppler if you use tissue Doppler what you are going to be able to actually assess is the E prime on the septal wall and the E prime on the medial wall and you are going to be able to get the parameters based on the new guidelines as you can see here you need to have two of more parameters so you need to do your septal E which is going to be less than 7 cm per second your lateral E prime that is the one that we were measuring here in this example which should be below 10 to be abnormal and then you need to do the peak EE and remember when you are doing the peak EE at the level of the annulus should be measured at the mitral valve tips and compare it with the E prime ratio and if it's more than 14 is abnormal you should do the volume of the left atrium indexed if it's more than 34 mm per meters to the square it's abnormal and the TR velocity more than 2.8 m per second so that's how we assess diastology so long access view in a patient with Hong Kong that's the classical thing that we expect to have is this nice septum the anterior mitral valve that is getting in the middle of the LVOT this severely posteriorly directed MRJET turbulence LVOT flow the flickering the R2 valves all the signs that we have been describing so far so an important part that I think we need to actually explore a little bit more is when you have a subarctic LVOT obstruction you need to differentiate between dynamic and fix the dynamic is more or less what we are going to find in a Hong Kong patient the fix is the classical one that we are going to find what we have a subarctic membrane the subarctic membrane of fixed subarctic LVOT obstruction is going to give you a continuous with doctor shape form similar to the arctic stenosis curve the important one that is the one that we are assessing here is the dynamic subarbular so the classical signs for this Doppler profile is a dagger or a shark tooth shape which is characteristic for having a mid to late systole, a late peak LVOT signal which is what the yellow arrow is actually showing here in the bigger shape Doppler profile when we assess fixed subarbular obstruction which is the one that you will see with a subarctic membrane there is an early peak LVOT signal and you can see the presence of AI too which is typical with this kind of pathologies treatment for hypertrophic cardiomyopathy there is the description of the alcoholic septal ablation through septal perforators resynchronization but the one that actually is important for us is the fact that the patient will need a myectomy so the indications for myectomy are going to be based on echocardiography so peak reading of more than 30 at rest or more than 50 with provocation myotomy is myectomy is going to be performed and you are going to remove from 5 to 10 grams of septum and risk of complications almost 60% of LVOT only 3% of complete heart block and 1% of PSD or aortic insufficiency the mortality is up to 2% so 3D echo can perfectly be used to assess those patients too we were able to estimate the septum and we were able to get more accurate measurements of the multiplane reconstruction to the surgeon that we 2D so the classical view that we are going to get is like that so we wrote it said in those images and what we are going to get is this view which is the classical surgical surgeon view or amphis view with the mitral valve below and the aortic valve on top to the right and then on top of the aorta the pulmonic valve so the surgeon is going to put the retractor to the right coronary casp and the interventricular septum is going to be on the right side of the screen and that's where he actually performs the myectomy so once the myectomy is performed so what should be should be addressing so the first that you need to address is is there any residual LVOT obstruction if there is any residual or mitral regurgitation due to that is there any VSD is there residual AI so those are the questions that we mainly want to ask so as per the guidelines again that's what we got so in a patient with surgical myectomy so the recommendation is the presence of some surgical obstruction, mitral regurgitation and ventricular septum's defects so if you pay attention to this image we go over here so you can certainly see stop the image there okay so you see the aortic valve is closed so if you pay a little bit of attention it goes away and then it sits to the and then again there it is and you see in the diastole with the mitral valve open so this, so what do you guys think that this is due to so this is a septal perforator so that's very typical after a myectomy that doesn't mean that the patient has a VSD that's actually the artery from the coronary arteries that comes and they use to actually use the septal colablation and it's always in diastole and not in cystole as you will be expecting a possible VSD from coming from there okay in a VSD so post myectomy a successful septal myectomy is considered when there is no significant rest in LVOT gradient so as per the papers LVOT gradient less than 16 millimeters per mercury is considered appropriate and nonsignificant we normally again can use the deep transgastric or the transgastric long axis recommended from my point of view the deep transgastric has a more better possibility to align the plane than the transgastric long axis congratulations you made it so please if you have any questions email me and all the best