 Okay, good afternoon everybody, and I'm going to record this lecture of Ventricular diseases and Cardiomyopathy that I gave To all of you I said yesterday So now I'm going to record it and you can have it So Ventricular disease This lecture is based on two guidelines The first guideline is old is 2011 right line by American Society of Echo and by American Society of Blue-Clock, Algymousia and MRI And that's a combination for multi-modality Cardiomyopathy imaging of patients is hypertrophy of Cardiomyopathy And the second guideline that is a recent guideline was published in 2020 That's a guideline of American Society of Echo and American and Society of Cardiovascular Anesthesiologies And Society of Thoracic Surgeons so the cardiac surgeon are inside this guideline as well and They talk about decision-making in the world for different surgical procedure between Cardiac anesthesiologist Echo cardiographer that is anesthesiologist again or maybe cardiologist and cardiac surgeon And this is a guideline that I recommend all of you to read it very well for exam So Cardiomyopathy We have three types of Cardiomyopathy, hypertrophy of Cardiomyopathy Restricted Cardiomyopathy and Dilated Cardiomyopathy. In hypertrophy Cardiomyopathy, there is asymmetric hypertrophy on one part of the heart most of them septum, but it can be apex or mid cavity as well Usually their systolic function is normal at the beginning except they come very late Restricted Cardiomyopathy is a Cardiomyopathy that again has mainly Diastolic dysfunction, maybe systolic function is normal at the beginning But they don't have any asymmetric hypertrophy. They might have hypertrophy, but this is symmetric and Dilated Cardiomyopathy as you see the LV is more dilated and systolic function is Depressed and maybe at the end diastolic dysfunction So there's two type of classification of Cardiomyopathy, one by WHO and one by American Heart Association WHO classification Define the Cardiomyopathy as a disease of the myocardium associated with Cardiomyopathy dysfunction And they declassified the Cardiomyopathy as a dilated Diastolic Erythrogenic This is the RV Cardiomyopathy mainly because RV dysplasia They don't have usually Surgical treatment. They don't come to the war and Some unclassified Cardiomyopathy that again most of them they don't come to war except for Elvath or for transplant American Heart Association expert panel definition for Cardiomyopathy Again define the Cardiomyopathy as a disease that is mainly in the myocardium It can be because of the heart itself or can be because of the systemic disease And based on American Heart Association Cardiomyopathy can be primary or secondary Primary like hypertomocardium is a part of the primary Cardiomyopathy and It can be mixed as well. It can be acquired as well Like inflammatory myocarditis. That's one of the main cause of Cardiomyopathy That usually goes to dilated Cardiomyopathy like a COVID can give a myocarditis or even Shagas is a type of dilated Cardiomyopathy because of the again involvement of the myocardium a Stress-induced that's the Takotsubu Cardiomyopathy. So all of them are primary Secondary it means this another disease and created the Cardiomyopathy like amyloidosis like a storage disease like a toxicity like a hyper eosinophilic syndrome inflammation like sarcoidosis and endocrine disease and some other type of disease What about management of the patient this hypertrophy hypertrophic obstructive Cardiomyopathy? That's the one that we will see them in the war and we as a ecocardiographer We have to know how to diagnose them and how to manage them hypertrophic obstructive Cardiomyopathy is characterized by left ventricular outflow tract obstruction Due to asymmetric septal hypertrophy and systolic anterior motion of the mitral valve Patients who fail medical management and have ongoing symptoms of dyspnea Chest pain or syncope can be managed either by myectomy surgical or alcohol ablation That alcohol ablation is mainly European procedure and we don't do too many here But I came from Europe to North America. So North America treatment is mainly surgical treatment In addition some center offer concomitant mitral valve surgery For primary treatment of LVU obstruction, especially for cases with no significant septal hypertrophy They might have just mitral valve replacement and or mitral clip to treat them are surgical rejection of muscle from the sub aortic region To enlarge the LVU T results in relief of Sam of the mitral valve and its resultant mitral degurgitation Surgical myectomy is the gold standard for refractory LVU T obstruction at centers with dedicated hypertrophy obstructive Cardiomyopathy surgeon So we should have a dedicated surgeon for myectomy many center We don't have a dedicated surgeon for myectomy. All cardiac surgeon They do myectomy and most of the time is inadequate myectomy In dedicated hypertrophy obstructive Cardiomyopathy center Mortality is typically less than 1% for isolated myectomy and in our center even is less than 1% Is like a 0.5% is as you said is excellent long-term survival Surgical myectomy of the septum is called moro operation because the first time in 1961 I think was done by andro moro. He was the chief of this surgery of NIH He did the first myectomy and later one of the day he was sitting with Eugene Bromvold our famous cardiologist That is a textbook of cardiology and he asked more what can you listen to my heart? Because I have some palpitation and we're all listen to his heart and said, oh, you have hypertrophy your self So you have to go for surgical treatment and he refused that and he had a sudden this and died So this is a paper that was written about a moro and his faith in the disease by Barry Maron Barry Maron is from mayor Kenny. He and his son are the two main person Specialized in hypertrophy cardiomyopathy cardiologists in the world most of the thing that we know about hypertrophy but he is written by Barry Maron and his friend, Dr. Weigel that was our cardiologist here Dr. Weigel passed away about four or five years ago. He's the autopsy heart of the Moro himself and this is from his daughter. His daughter had a hypertrophy and passed away as well This is genetic disease This is a very nice paper from Dr. Tony Ralph Edward from our center was published in analysis of catheterastic surgery in 2017 About result of isolated septal myctomy in Toronto General Hospital So in his paper this paper He presented 291 patient done from 2012 to 2016 So he's doing about 80 case per year Insta 150 of them. They had only isolated my to know my travel or other things in that 150 None of his patient had a Sam significant in post op echo five percent they had a MR great on the mild No VST very good result Five percent complete every block and only one this one this in hundred fifty patient And Tony wrote a very nice paper game in analysis of catheter of surgery in terms of the technique how we do the make to me and This is some of the image from that so he opened a precardiom They all taught to me The way that he is doing every day you can watch it in the war as well So he is start from below the right corner cast this right corner cast This is a non corner cast and this is left corner cast surgeon is standing here So he is start from below the right corner cast and goes Towards the left main coronary and the depths of this myectomy is about one centimeter So we tell him is it two centimeter or two point two centimeter He has an 11 blade this 11 blade that will cut on the 11 millimeter So he will do like a 10 11 millimeter depths and he goes down The the lengths that we told him to be up echo we say for example Is four centimeter from the right corner cast still is sick and they will go for something Usually he will go beyond the some septal contact And then he will do more And he take all of this muscle and at the end he will do a little bit below the right corner as well And when the muscle came aim out He should be able to see the papyri muscle So the good make to me is the make to me that the one the surgeon look at it from your thought to me Inside the LV can see the papyri muscle. This is a cut The up and this is a post-op Muscle is out and we gave them the same map on almost by echo I'm closing the orthotomy and he has a very nice movie there as well So I recommend you if you go to the site of UHM library and find that paper You can see the movie as well I couldn't copy the movie and bring it for you in the power print presentation But it's a couple of minutes. I see nine minutes movie is a very nice movie and Tony himself is talking and if you see that movie you can understand exactly what he is doing in the war and He concluded that my egg to me is the gold standard for treatment of LVT obstruction Excellent result can be obtained at centers with the indicated hyperthoracidomyopathy program like our center Careful attention throughout the procedure preoperative Interoperative and post-operative setting can ensure no complication and no Mortality and very good long-term result This is one of our old paper. I am part of that before 2000 in that time when he was a still resident and Victor Bill William Head of the cardiac surgery of six in the hospital. He was doing the my egg to me and I Did the interrupt key for him about 300 cases in eight years that I was here Bill William actually was the resident of must start the famous must start operation So Bill William retired for five years ago And in that time he was training Tony and now Tony is doing so we showed that There's a good relation between relief of the MR and the my egg to me So in our video was 300, but we put 104 patient in this paper published in Jack 2000 and We showed in 93 patient that they had MR secondary to Sam they improved another 10 patient they had primary mites of our disease as well and that was our conclusion in that time So what is our role in terms of the echo? First of all, we have three type of myectomy based on the guideline The first might to me is the way of myectomy that morrow did it that was very limited might The second type of myectomy is called extended my to me. That's the way that Tony is doing in our war okay the third one that is in the guideline it says myectomy plus perication of the anterometer leaflet and plus release of the property muscle So this came in the guideline guideline of 2020 I Asked Tony this morning. Actually in the war Do you agree with everything is written in the guideline? He said no, he doesn't agree and he even doesn't know that center That center is a small center in New York is called St. Luke a Roosevelt Hospital is a hospital belong to Columbia University It's not the main hospital of Columbia University They are doing like a 10 case per year So we are doing 80 per year so Everything that they said came to the guideline. We don't know maybe The surgeon that is in his operative here. He was sitting as a surgeon in the guideline But anyway, I think you have to read this paper and learn a little bit because the question Will come from the guideline so in that guideline they said Anytime until my to leave it is more than three centimeter MR is more than two plus is this historic until emotion and there's no romantic disease You have to do leaflet perication, but we are not doing at all I asked Tony this morning and he said he did only one leaflet application in last 10 years So he's not doing that way. So the way that the Tony did do the surgery is a very effective We see it and may you can they do this way as well But the way that the guideline is discussed is mainly in that hospital in New York So in that guideline they showed that About 62 percent of all of their patients they had a publication of the anterior mitral leaflet and some of them they had a mitral valve replacement and Again, they suggested RPR repair R means rejection of the septum P means publication of the anterior mitral leaflet R means release of the anterior papillary muscle and as I said None of these are done by Tony. Tony will do only the first one a good rejection This is the some image that how we do Sceptile rejection how we do the papillary muscle release and Pelication of the valve So in period of assessment by echo we have to see the septum very well And we have to assess the mitral valve and timing of the MR and We see exactly what is the cause for LVT obstruction For the septum we have to measure the septal sickness In the guideline it says you can measure in four chamber view But usually in our center we use a long axis view under 20 to measure the septal sickness But septal sickness in that view is a little bit oblique So always it's better to look at the transverse echo of the patient all of them They have a transverse echo in our center for myectomy So you should the best measurement is the measurement of the sickness by transversing and You can use multiplanar 3d as well for better Measurement is one of the example of the LVT obstruction and the MR after the Sam This is a guideline way guideline said measure the septal sickness in the four chamber view we measured in long axis view and Guidelines said measure the lengths of the anterior mitral leaflet if it's more than 30 millimeter or 30 centimeter We should do application and we don't do it But but you learn everything that is written in the guideline because if there's a question question will come based on the guideline so the pre-op Measurement and that's a post-op septum In terms of the mitral valve always you should see That MR is all secondary to Sam or Some MR before Sam will come if the MR is start before Sam that is primary mitral valve disease Always look at the ruptured corda. Always look at the Mac especially So about 10% of all hypertomical hemopathy Their MR maybe is a primary mitral valve disease plus post some MR and sometimes this primary MR is Obscured by the post Sam MR And it will show up post up and we had the case like two three weeks ago We didn't see any independent MR pretty up and post up when he did the rejection the MR showed up So in that time surgeon especially Dr. Tony because he's not very familiar with Mitral valve repair So if Tyrone is not around He prefer not to do that case in that day if mitral valve needs repair. So it's better Mainly our echo lab they should tell him a Useful measurement for mitral valve include the length of the leaflet as we talked it Lv2 obstruction we should measure the gradient see exactly what is the obstruction is dynamic or not Maybe there's some fixed obstruction as well congenital the classic Obstruction is a dagger shape Doppler and you have to differentiate it from MR This is the obstruction of the RVT is a dagger shape and this is just MR So don't mix this one. This is a late systolic gradient This MR jet is Doppler from our way from the beginning is a parabolic shape and This is a dagger shape and usually the MR velocity is Higher than this This is mainly be maximum four meter or five meter MR velocity usually six seven or eight meter even So this is the table in the guideline I will not repeat it again because I said most of this what you should do in the pre-op What do you you should use it and what image modality and what is your limitation and Especially our fellow they see it. We do it every day in the war But you have to review all of these tables and Question will come inside this table post-op Like every post-op you should see the adequacy of the surgical procedure and you should detect the complication For cardio for hybrid of cardiomyopathy, I will tell you most of these cases almost 99% of these cases They had a good echo period up in our echo lab because we have a dedicated a Card a hybrid of cardiomyopathy clinic in our echo lab and in our cardiology is one day per week I think one day the Torkovsky will sit my teacher and one day And who will sit so they have a good assessment. So don't worry about the pre-op assessment Look the post-op. This is our role in the war First of all, we have to see that the surgical rejection was adequate took enough and To see the gradient The guideline will recommend that we should challenge the LV boys the vitamin 10 micro per kilo per minute Increase the heart rate at least 20 and see if the velocity goes above 3 meter per second We have to ask the surgeon go back on home and fix it We don't do it in our war, but you learn this because it might come in the exam so post-op for hypertrophic cardiomyopathy guideline we said we should do a Do vitamin challenge with 10 micro per kilo per minute some center they do a PVC So you can ask the surgeon just top the heart create the PVC to see gradient will go up or not We used to do PVC and now these days we don't do it and we don't do this to be to me challenge We measure the septal sickness how much is left and we reevaluate the mitral valve post-op This is like a pre-op Turbulence at the LVT and the MR and more disappeared post-op Complication a couple of complication one is inadequate Rejection that very rarely happens in the Tony's hand always rejection is enough And second complication is VST That you will see a flow from LV to RV So you should not make a mistake of Sceptile perforator flow as a VST because VST flow is systolic and septal perforator is mainly diastolic VST flow is very high velocity Sceptile perforator flow is low velocity is below one meter VST flow usually three meter four meter and Sometimes the patient might develop AI as well. Don't take it as a VST So in terms of complication as we suggested before VST is one of the important complication As we showed it didn't happen in our series But in some centers, they might have one person to person VST always you have to distinguish the VST from Coral flow or septal perforator flow that surgeon will cut it and Some new AI so VST is systolic flow. The velocity is high usually more than two three meter per second Coral flow is diastolic flow and velocity is low is less than one meter. So that's a way to differentiate these two together This is all table from the guideline that what we should check during the Post-procedural assessment and I'm not going to repeat it again. I already talked about that and In terms of the mitral valve again You have to be careful that the mitral valve has the integrity is not caught There is any residual MR or not differentiated from new anterior MR sometimes MR there is a new anterior MR That will not show pre-op and you might see it only in post-op so Sgt. Might tell you how you didn't tell me for pre-op But sometimes is covered by the some MR and will show up only in posterior in post Mike to me. So surgeon has to be ready. If something is necessary for mitral valve go back again And sometimes you might have a eutrogenic MS as well This experience of meachalic they have the largest experience in the world They published the result of three thousand myectomy in a matter of like a 17 years So they do like a like a hundred fifty case per year We do like a 70 case per year So probably we are second center in the North America after meachalic In there myectomy three thousand Again five six percent of them. They had a mitral valve repair replacement and 115 of them they had myectomy from apical approach this senior surgeon of Mayo Victor Schaaf He's the president. He's the head of the cardiac surgeon there. He will do this Epical approach as well Here Tony is not doing I remember when I was fellow here With today we did one two from apical approach myectomy Some of the case example These are all case of our center. We're done last year I wanted to show you some of our new cases then this year and recent months for this Thursday, but now Victor Dallas Duncan I think he was showing hopefully he showed you some of this This is one of the case that we did last year. You see the Sam very well and You see the septal sickness how we measure it You see the systolic notch in the aortic and mode. That was the only way To diagnose the hypertofocardomyopathy when I was a resident in that time 2d was not there So that is a turbulence at the LVOT and the MR So this early systolic MR just after QRS this will stay after myectomy But the MR secondary to some is late system a bit sisterly to late sisterly that will go away This is a gradient Who stopped this is site of the myectomy? Very nice extended myectomy very mild some does not problem And you see there's no turbulence at the LVOT. That's a septal perforator Sceptile perforator flow is a diastolic flow. If you see the septal perforator is good It means the surgeon did a good myectomy If you don't see any septal perforator probably surgeon didn't do any good myectomy. Maybe it's very superficial So here you see there's no turbulence at the LVOT and they mean PK is only seven or eight case number two is a known case of again of Petrov you see very Like a mix of matrix mitral valve This is a valve that maybe perlication is good, but again this one Tony did it without perlication of the valve did only good myectomy and result was very so you see the SAM and And the measure that's MR after the SAM after the SAM Is it early MR that will stay This is a late MR. It will go after myectomy Very good if you function hypertrophy That's a gradient pre-op In the pot you see the SAM is not there. There's no LVOT obstruction No MR So result is Very good The case number three is a little bit Challenging because this case came from british columbia And didn't have any echo in our center. Usually patients that they are from our center Because we have a special clinic for hypertrophic cardiomyopathy. I see every every Thursday And that is after the name of the tovaygel is called the tovaygel clinic of Happen to cut the MR potty and dr. Kouski my teacher is running that one But this patient came from british columbia. So didn't have any echo here. That's the reason that we were in trouble in the war When we did the pre-op echo we saw no SAM We see some septal hypertrophy, but there's no SAM and the valve has some restriction looks like this aromatic valve See there is MR is posterior directed, but it's not after SAM Or is Mixing with the MR after SAM Is AI any time you saw two valves three valves involved usually they are aromatic Okay So this mixing of the SAM MR and primary mitral valve disease MR valve is a little stronger Anyway, I I warned dr. me that You you are doing my egg to me, but I don't think will be any effective for MR I'm patient will be the same automatic again so Yeah, he said I didn't know I didn't tell dr. David so I will do the my egg and hopefully we do the mitral some Translator So this is a AI is there And you see the MR is there. So there's no difference for patient So the result was not good because this patient was not assessed pre-op before coming to the war very well And This patient was taken to this ICU You after my to me in ICU arrested the day after I think Was resuscitated. What was this? Or but still the MR was there. So there's a couple of questions here at the end of the session the question number one based on american heart association classification Which of the following cardinopathy is a primary cardinopathy? A stress induced is a primary Cardic amyloidosis is not primary Endomyocardial fibrosis or laflare disease is not a primary Is because of the hyper eosinophilic syndrome And homochromosatosis is not primary. So number a is the answer All of the following are a complication of surgical myectomy except VST is a complication. Also, we don't see it in our center completely block is a complication Pulmonary regurgitation is not a complication because we are not opening the right side. So answer is C Question number three, which of the following is correct? Mortality of the myectomy is three percent two percent is not is less than one percent In group of patients with senior symptoms chance of VST is higher is not at least in the hand of Tony Chance of complete every block is less than alcohol septal ablation. This is correct Chance of residual severe some is five percent. No severe some. We don't see it usually if you see Some you have to go back and fix it. So the answer is C Which of the following treatment in hypertrophy? What is less effective? Myectomy is effective alcohol is effective medication is effective pacemaker is not effective. So B is the answer And number five question All of the following statement about alcohol septal ablation in hypertrophy are correct except It is less invasive compared to the surgical myectomy. This is correct Chance of every block is 10 percent. This is correct in elderly patient with comorbidity is a better choice. It is correct Relief of gradient at this LVOT is faster than surgical. No In surgical relief is fast. You see it in the war In alcohol it takes six months to see a good reduction of the LVOT obstruction So these are the answers And I think we reached to the end. So as I said You can use this lecture Even for the people that they They are going to leave the center after a couple of days a week You can have it and you can be an ambassador of myectomy in your center Thank you very much for your attention