Hypertrophic Cardiomyopathy: The Most Interesting Heart Disease

0:00 hello everybody and welcome to a uh another year of grand rounds lectures we uh we're so happy to have everybody with us um as in the past I'll encourage those of you who have cameras with you to keep your video on whenever we can uh I'll turn mine off so I can chew a little bit but otherwise I'll do my best to keep my camera on um thank you all for joining us thank you for your feedback this summer we we heard a lot of interesting feedback about what has worked well and less well in uh in Grand rounds over the last couple of years and so we've been working very hard with the chief residents to really put together a uh tremendous Buffet of um choices of um of speakers for this year and um and

0:47 delighted uh to have all of you join us for that and in particular one of the things one of our goals for this year is to make sure that our alumni are featured and so we are particular ly thrilled to have an Alum Dr yishi shamada here with us today uh many of you remember him and many of you know that he's an assistant professor and the director of research for the Columbia hypertrophic cardiomyopathy Center um after getting his medical degree at the University of Tokyo he was a medicine resident a chief resident here from 08 to 11 and then went to the Harvard system for uh Cardiology fellowship and uh specialized training in hypertrophic cardiomyo aathy and then did an advanced Echo fellowship at Colombia and while

1:34 doing all of that um got his mph from Johns Hopkins he's written 60 peer-reviewed scientific articles which have been cited more than 1400 times he just received a uh ro1 Grand from the NIH I mean I can't say enough nice things we are thrilled to have him answering this important question about whether I guess hypertrophic cardiomyopathy is in fact the most interesting heart disease Dr shamada take it away all right thank you so much for intro your introduction Dr Weissman and hello everyone my name is U shimar and it's true I used to be an internal medicine resident and chief resident at best eal so I'm very thrilled to be back although it's virtual I do feel that I'm

2:20 back um here and today I'm gonna talk about hypertrophic cardiopathy which in my opinion in my opinion is the most interesting heart disease and I'm going to try to convince and you to feel the same way so here are my disclosures and there are three parts in my presentation part one is Introduction of hypertrophic Cathy then part two is the diagnosis and the part three is management of hypertrophy so let me start with a case of a 22 years old male an young athlete basketball player who died after collapsing during a game

3:07 so and as you can see on this newspaper article autopsy revealed um that he actually had hypertrophic cardiopathy as you can see in this case sudden cardiac death can be the first manifestation of HCM this P graph shows causes of sudden cardiac death in young athletes and as you can see from this graph here's HCM and constitute 35% of the causes different causes of sudden cardiac death so uh based on this the HCM is the most common cause of sudden cardiac death in young athlet in the US so this leads to the first take

3:55 home message of the day and I'm saying that this is the most cause of certain C death in young athletes in the US but if you it's actually in the young not only athletes but in the young below 30 years old in the United States and I'm saying in the United States because it's a little bit different in Europe especially like Italy the most common maybe like orogenic RV displasia or something else but in the US it is the most common cause of Southern cardiac death this is a gross pathology of hypertrophic carda ay and here's the left atrium um and here's the mitro valve you can see here and here's the left ventricle which is

4:41 pretty thick and here's the aoic valve and and you can see this left ventricular outlow truck just below the atic valve is pretty narrow um and the intraventricular septum IBS is very very thick uh the upper limit of normal for male is like on Echo is 10 millimeter it's for female it's 9 millimeter so this is like almost looks like 3 cm so it's pretty thick as well as the posterior wall this is also thicken and if you take a closer look there you you see some like white dots here this is um an intertial fibrosis in the sub myocardial subendocardial area

5:31 these pictures show the microscopic findings in normal on the left and patients with HCM on the right The myocardium in the normal subject is aligned in an orderly fashion pretty well in contrast the alignment of The myocardium is bizarre here in hm this is what's called myio myosite disarray in HCM and then microscopically you also see sometimes see intertial fibro process as well now on how many mutations and genes have been discovered to be responsible meaning pathogenic for HCM in the past we used to say that there are more than

6:16 200 mutations in more than a dozen of genes mostly coding for proteins in the sou then we had I had to change the slide because the more mutations were identified and even more mutations have been identified and more at this point I felt that this is kind of something like this like McDonald's start to say served over 1 million and then say over 99 M billion and now say they say billions and billions and then at this point we have over, 1500 mutations in more than a dozen genes that are responsible for hypertrophic copy and we can find mostly uh most of the

7:04 time we can find mutations in in familiar cases and early onset sportic cases so early onset nonfamilial cases however the dejection rate decreases to like as low as 20% in late onset sporadic cases there are several limitations in genetic testing um in clinical application of genetic testing data in hypertrophic copy first of all there are as I said there are so many genes with so many different kinds of mutations making it challenging for us to interpret the significance of each mutation second of all and about 50% or more of patients with HCM has no

7:50 identifiable uh mutations and third the genetic clinical correlations are weak so even family members who share the same mutation may have different phenotypes and this is probably because of like modifier genes and environmental factors I had an Aston case of two sets of twins so there were two sets of twin in one family and they shared the exactly the same mutation but one brother did not have any phenotype at all and the other had a lot of like ventrical tardio sou cardic death and it what required an a ICD implantation so this highlights the the weak correlation between the genetic mutation and

8:36 clinical syes lastly genetic testing I wouldn't say is expensive but can be expensive depending on what insurance you have without insurance coverage it cost about a few thousand dollars so how much how much the patient has to pay out of the pocket really depends on what kind of insurance they have moving on to the second part of the the presentation I'll talk about the diagnosis of hypertrophic kopy the diagnosis part is probably more important for you guys as a medicine resident on than the therapeutic or management part because you're are the front line right seeing a lot of

9:22 patients on and so you're you may be the one who makes the diagnosis for the first time in the the patient's life refer to cardiologist or manage those patients in your clinic so I'm going to um put some uh ask some questions for you on later on and then if to answer my question you can just put like your message to chat if you don't feel comfortable messaging to everyone you can just message to only me or you can just unmute yourself and it shout the answer so let's get started first h c m hypertrophy cardiopathy is characterized by left ventricular hypertrophy without other conditions

10:07 that can cause the same degree of hypertrophy so LV hypertrophy out of proportion to like other loading conditions such as blood pressure sometimes it could cause right ventricular hypertrophy as well the prevalence is 0.2 to 0.5% in other words one in 200 to 500 people are affected by HCM imagine that during your residency or maybe fellowship or during your career you see thousands of patients right and if 200 to one out of 200 to 500 people are affected in by ACM then you can probably see a only at least a few during your residency so just make

10:53 sure uh to get some features from my presentation and then make sure do not you not miss those patients with ACM because they are very very underdiagnosed sometimes there are sporadic cases however HCM is often familiar with autosomal dominant pattern of inheritance it can lead to diastolic dysfunction and heart failure ischemia even without coronary Ary disease arithm such as atrial fibrillation ventricular tardia and ventricular viation and at uh and mitro valve abnormalities it can cause left ventricular Alpha tract obstruction or

11:40 lvot obstruction in some patients and these cases are called hypertrophic obstructive cardiomyopathy or hokam a lot of people call HCM as hokam because it's easier to pronounce hokam but hokam is actually reserved for obstructive form of HCM not uh not just HCM then why do patients with obstructive HCM get symptoms the key component is Sam or systolic anterior motion of the mitro valve leaflet when you have asymmetric septo hypertrophy displayed here then the LV outlow tract written

12:27 here gets a TR creates and creates faster blood blood flow through the lvot in that case the anterior leaflet of the mitrol get dragged anteriorly which is Sam right the syic anterior motion of the mitro leaflet and further Narrows the lvot left ventricular outflow tract if the anterior leaflet gets dragged further then the coaptation between the anterior and posterior leaflets gets lost and this results in mitro regation as displayed here so because of these uh patients can

13:13 get disia and other symptoms of symptoms of heart failure mainly due to diastolic dysfunction plus minus mitro ration they can also get Ana even without significant coronary out disease because the myio cardium is very very thick and oxygen oxygen supply gets impaired in the subendocardial area they can also feel palpitations from arithm they may complain of Li headedness dizziness pre Syncopy or Syncopy and these are Mo most often due to LV Outlaw tract obstruction however they may be due to ventricular tachic cardia the most feared symptom

13:59 of hypertrophic cardiopathy is sudden cardiac death and as I said it this can be the first manifestation of the disease on the physical exam you may feel brisk crowded upstroke and a forceful LV impulse there may be S4 you sometimes hear murmur in obstructive form of ACM this is due to obstru LV outload truck obstruction mro regation or maybe both um murmur due to lvot obstruction gets louder with Bava maneuver and this is because the lb becomes smaller due to

14:46 decreased preload and the LV outlow track abstraction gets worse so here's your take home message number two there there are only two cardiac conditions whose murmur increases with f Sala maneuver one is as I said obstructive form of hypertrophic kopy and the other is mitrol prolapse this is kind of high yield in the USM and also Medical Board and also actually Cardiology for all right let's talk about EKGs so EKG in patients with HCM can show essentially anything and everything uh this is kind of more typical EKG of

15:34 hypertropic chopy showing left Inc hypertrophy with strain pattern see SD depressions down sloping SD depressions strand pattern um this one is showing Qs pattern in V1 you see no R right it's q and s and V2 and V3 me just shrink my screen yeah so this if you are shown this then you would think that oh this patient may have had a prior Mar cardum infact in the septo and anterior distribution but and we know clinically

16:20 that he he did not because we did Cath and he had a pristine coronary artery so this is pseudo infar pattern in this particular case it's a pseudo anole micro infrction pattern so HCM could do this to theg this is another example of patient with uh hypertrophic Cathy you can see Q waves in the inferior leades right to three avf and then kind of tall AR wave in V1 V2 so as you know this is in theory infer pattern and what uh the infarction of what area causes tall r on in V1 V2

17:11 it's posterior infar right so this is pseudo inferior and posterior Mi pattern caused by uh hypertrophic copy again this patient does not have history of Mi or does not have any coronary a dise this is or HCM doing this to the EKG next one has Qs pattern in the anterior or sepal lead B1 V2 and then also Qs pattern in the inferior lead so this EKG is uh showing double infer pattern and typal and uh and

17:56 inferior and the second next one it looks a little bit scary because those t- waves look pretty peaked right so this is typical for the hyperacute T in stemi before s elevation is if you catch the catch them in the hyperacute face you see this very tall peaked looks like it's pretty sharp and uh it's going to be painful to sit on on t-wave but this is their this patient's Baseline EKG and this is done the changes are caused by hypertrophic cardos but not esia so HCM could do this to the kgs and this patient has on apical and

18:46 lateral t-wave inversions on even without the presence of esia and this one is t uh a good one for the pocketbook uh one you you remember from today so this show this is showing pretty deep very deep t-w inv versions to3 avf but uh more importantly they like V4 V6 maybe V4 V5 this is called giant negative t-wave uh can anyone guess what sub type of Hy hypertropic kopy this is you can send a chat to me or if you feel

19:32 comfortable everyone or you can unmute yourself and Shout the answer what sub type of hyperic copy is this EKG typical for giant negative T waves in in apical Le

20:26 all right yeah the answer is the apical sub dipe of hypertrophic copy it's a giant negative T waves those are typical for epical form of H all right so this one looks like the patient is having STD elevation Mi here and there uh but this is their Baseline EKG this patient is not having chest pain or cardiac esea um but this HCM that's causing these change STD changes so our practice or advice to the patient to is to give a copy of EKG to the patient so they can carry them carry the EKG in their wallet

21:13 otherwise what happens if this patient comes to the Ed with chest pain they're going to cast him right yeah they they those people sometimes get emerging card cast for atal or non cardiac chest pain just because this uh Bas their Baseline EKG has St elevations so there we're gonna ask we ask the patients to keep the EKG in their wallet this is a rather atypical unusual EKG for a patient with hyperopic copy can someone guess why this EKG is unusual for hypertrophic kaposy

22:03 maybe voltage is like looks voltage looks normal yeah yeah you got to write yes so it's like including voltage that that's very normal right there's no STD changes and yeah things look normal there's no LBH or strain pattern so yeah it's rare for patients with ACM to have completely normal EKG it's very rare they usually have some AKG abnormalities so echo cardiogram is the first and the most let me just play it again most important modality to

22:48 diagnose HCM as you can see here this patient with HCM has pretty thick intraventricular septum here as well as a thick posterior wall and there is a little bit Sam in during CIS the tip of the mitro liid get dragged anteriorly as you can see here dragged to anteriorly during this okay there are several other conditions that can cause LVH and we always have to think about other conditions that can mimic HCM hypert copy the differentials for HCM hypertrophic cardiopathy include

23:34 pressure overload due to hypertension aoic stenosis subotic membrane and the other differential include athlete heart so physiologic conditioning against H strainer training other differentials include fabri disease and other storage diseases and cardiac amiloidosis shown here are examples of pathological findings from the three HCM mimickers um Can someone guess what this is on the left upper side this is showing a light pink highing extracellular deposits displacing the cardiac myoides

24:28 yeah the answer is ameloid cardiac ameloid how about this one showing no cating granuloma on the right upper so this is sarcoid cardiac sarcoid and the last one showing cardiomyo side hypertrophy and perinuclear vacu this is an example of fabric disease the other differential a heart it is sometimes difficult to distinguish between HCM and athlete heart here are uh echo cardiograms in a peral Long aess View from an elite athlete Olympic roller and a patient with hypertrophic

25:17 copy so the interventricular septum uh thickness was similar in each and this may create that diagnostic dilemma let me show you an example of such diagnostic difficulty to highlight the magnitude of the problem so Eddie Curry was a high school Center pretty tall and had a was a player of the high school player of the player of the year and turned Pro in 2001 and a great record 16 points per per game five rebounds per game great numbers and then went into free agent hoping for a 60 to 70 million

26:02 contract in 2005 however he had a ventricular ta cardia event and missed the 13 games of the of the season and experts got together on did on Echo Echo was equivocal they couldn't tell if that's genetic hypertrophic copy or just physiologic conditioning due to uh this basketball training athlet heart so three experts got together one said that he should get genetic testing he said nope and then Chicago Bulls uh that he used to belong at that time did not

26:50 allow him to play anymore without without getting the recommended genetic testing so he was about to finish his career there but one team took him without genetic testing and allowed him to play does anyone know which team took him think you're in New York y Team Six got it you got it right yes New York Nick took him so yeah Nick took him and a him to play and eventually he completed his career without any VT V or sudden

27:37 cardiac death event so this highlights the magnitude of how much the diagnosis of HCM on and fear of sudden cardiac death could affect one person's career as an athlete think six tenzine Lauren and K thank you all right so HCM is classified into two categories uh based on hemodynamics this distinction is important not because of academic purposes but because it does change our management as I will discuss later if you have significant lvot struction

28:23 defined as a great lvot gradient above 30 m Mill Ary at rest or with provocation this is classified as obstructive hm or hooka you can provoke lvot obstruction using several different techniques including exercise and valava maneuver if you cannot provoke LV obstruction then it is categorized as nonobstructive HCM now this is an example of pressure tracing in the LV and femoral artery representing the intra aoic blood pressure during Vala maneuver in a patient with obstructive form of hypertrophic kopy the yellow line above

29:11 here says LV is uh shows LV pressure and the orange line or tracing below says fa shows pressure in the artery as you can see here there is almost no pressure gradient meaning no no lvot obstruction between LV and aota before V of maneuver however significant pressure gradient arises during and after Vol of maneuver as you can see here which gradually goes down to the Baseline level during the release phase of V now the important point of this figure or this tracing is this what is the

29:58 lowest systolic blood pressure in the artery in this patient so here the systolic press blood pressure goes down and this is probably the lowest point and the scale is that this is zero this is 100 so it's like 20 40 60 80 so what is the lowest PR like prop pressure on this speed can anyone tell that looks like 38 yeah yeah it's like below 40 right it's like about 38 yeah so systolic blood pressure being 38 what happened to the patient they may syncopize right with this blood

30:44 pressure so that the point and leads to the take-home message number three so when you're performing vola maneuver you have to place the patient in a Supine position because if they are in a sitting position or standing position and if you Deva then they may actually synop okay and when making a diagnosis of HCM is challenging cardiac MRI magnetic resonance imaging can be very helpful it can measure LV wall thickness more accurately than Echo it can visualize the AP better than echo cardiogram and

31:30 it can MRI can assess for LV fibrosis by using l g linium enhancement this is an anecdotal case of a patient who got EKG and echo cardiogram for family screening purposes so he was asymtomatic and and Echo looked pretty normal the echo wall thickness of 0.8 which is uh pretty normal range and the kg was also essentially normal however we detected that this R progression it's kind of overlapping a little bit but R progression was reversed between V2 and V3 in normal it should you should have a progression of

32:17 R in V1 V2 V3 but there is a reverse R progression and there's no other abnormality on EKG or Echo but we got an MRI just in case because just because of this reverse or progression and that MRI showed wall thickness wow of 21 millim about 15 is the diagnostic criteria for hypertropic paropy so it's way above the diagnostic criteria so he's got the diagnosis established diagnosis of HCM so this case tells us that it is important to obtain C MRI if there's any even slightest concern for HCM based on

33:03 the AKG EKG or Echo the other test that can be useful in equivocal cases is genetic testing we can be sure that the patient has HCM if genetic testing shows a pathogenic mutation I.E comes comes back positive therefore genetic testing can be useful to rule in HCM but it does not rule out HCM meaning that negative genetic testing does not rule out HCM the genetic testing panel for HCM also includes genes for a certain type of amalo dois Danon disease and a Fab disease so

33:51 we can sometimes rule in or rule out these HCM mimickers based on genetic testing all right moving on to the part three of the presentation but if anyone has any questions so far I can take your questions one or two questions now you can chat or shout or if you don't have any question question I'm going to move on all right let's move on so I'm going to talk about the management of an hypertrophic

34:39 cardiomyopathy whenever we see a patient with hypertrophic Cardy in our Clinic we always hit these four points there are activity restrictions screening of family members prevention of sudden cardiac death and control of symptoms I will start with discussing the activity restrictions in general patients with hyperopic cardiopathy are advised against strainers disease uh strainers exercise burst exercise competitive sports or contact sports like basketball and ice hockey American football and isometric exercise for

35:26 example free heavy weightlifting and then they're also recommended to keep themselves very well hydrated when they're doing exercise or sweating for a long period of time the second point that we always cover when seeing patients with HCM is family screening strategies the American Heart Association and an American College of Cardiology recommend phenotypic screening with sorry going back yeah phenotypic screening with echo cardiogram and electrocardiogram to be repeated every three to five years in adults and for children and adolescents uh they

36:13 recommend screening with EKG and Echo every one to three years depending on their risk the other strategy other than phenotypic screening meaning Echo and and EKG uh to perform family screening is the genotypic screening so there essentially there are two strategies for family screening inm one is phenotypic and the other is genotypic for genot genotypic screening first of all we test the proband the proband is the person or the patient who already has a diagnosis of HCM and if we are lucky enough to find the genetic

36:59 mutation that's um responsible for this proband HCM then we can test this specific genetic mutation in the family members each first degree family member has a 50% chance of inheriting inheriting this disease causing mutation and if family members have negative genetic testing then they are confirmed to be free of the risk of the developing the disease for the rest of the life therefore such family members do not have to undergo phenotypic screening with ech electrocardiogram and echo cardiogram every five years one thing we have to be very

37:46 careful about when recommending a genetic testing for the family member is that genetic testing results May influence insurability if the test com back positive there's a federal law named Gina or Gina act or genetic information non-discrimination act which prohibits insurance companies to deny Insurance based on genetic testing for employment and health insurance however the law is silent regarding life disability or long-term care insur insurance so we have to talk about this issue with family members before sending the specimens for genetic

38:35 testing the third point that we always review in our HCM Clinic is prevention of sudden cardiac deaths because as I stated in that that at the beginning of this presentation HCM is the most common cause of certain cardiac death in the young and this is one of the examples of a young athlete who was dead on the field while playing soccer as you know icds or implantable cardiov defibrillators can prevent sudden cardiac death due to ventricular T cardiio fi uh ventricular fibrillation however icds are also associated with complications such as infection and bleeding uh the need for repetitive

39:23 battery changes and reduction in a qualitative life because of fear of inappropriate shocks then how can we determine exactly which patient should receive icds and we should not shown here is the data comparing the event weight between icds placed for primary prevention and that for secondary prevention as you can see in this graph the event rate for patients who received icds for the secondary prevention teary prevention meaning they had an event and then received icds to prevent another event um the event rate is pretty high it's reaching like 50% of

40:10 receiving appropriate intervention or ICD shocks eight years after implantation therefore the guideline recommend ICD implantation for patients with prior Cardiac Arrest or sustain VT in patients with HCM this is kind of easy answer because they they do need aicd they already died once they we have to prevent another death sudden KC death the problem or dilemma sorry let me go back the Dilemma uh is the primary prevention primary prevention means that prevention for those who never had such an event of like V vac sustain vac or sat cardiac death because as you can see on this in

40:56 this graph the event rate is relatively low it's about 4% per year then how can we determine exactly which patient should receive icds and which should not for the primary prevention that's a difficult difficult part so far we have identified five major risk factors for sudden cardiac death in patients with hyperic cardiopathy the first is the family history of sat cardiac the second is family uh sorry personal history of unexplained Syncopy over the last five years the third is marked meaning more than 30 millimeter left ventricular hypertrophy the fourth

41:42 actually these two were added in the uh renewal of the HTM guideline in 2020 uh so the one uh the fourth major risk factor is apical aneurysm and the fifth is LV ejection fraction equal to or lower than 50% because usually hypertrophic copy patients have normal or like upper limit of normal even or even hyper dnamic LV function and higher lvf so it's very very abnormal for patients with hyperopic cardiopathy to have abnormally low ejection LV ejection fraction and if the patient has one of

42:28 these five major risk factors or one or more then the guidelin said that ICD implantation is reasonable if they do not have any of these five major risk factors then we assess the these two minor risk factors or predictors of sudden cardiac death which are extensive late G linium enhancement on cardiac MRI which represents clinically represents its myocardial fibrosis in the left ventricle and the other is nonsustained ventricle tardia on Halter monitoring so if they have one or one of these two then the guidelin say that an ICD

43:16 implantation may be considered so here's the summary of ICD implant implantation strategy in HCM if the patient already had an event such as sudden cardiac death V fever sustain VT then yeah it's class one indication they need aicd so you're talking about secondary prevention here if the patient did not have such an event then you're talking about the primary prevention then you're going to turn to those five major risk factors and if they have any one of those or more then ICD implantation is reasonable if they do not have any of the five risk factors but one of the these two minor risk

44:04 factors then an aicd may be considered so that's the algorithm for aicd implantation the last the fourth point that we always discuss at our Clinic is control of symptoms to review uh to review the P physiology of symptoms in obstructive form of HCM the key component key point is this asymmetric sepal hypertrophy and this leads to all the consequences such as lvot gradient Sam and my uh and Mr therefore the target of therapy including medical and invasive in patients with symptomatic

44:51 obstructive HCM is to poison the hypertrophy de septum this this is why negative inotropic agents such as beta blocker and calcium chel blocker be become so important in the management of obstructive HCM the first option is always beta blocker and if the patient has a contraindication for beta blocker or cannot tolerate beta blocker because of side effects or heart rate being too slow will either substitute with what add Verapamil the third option for obstructive HCM pH pharmacologic therapy is disopyramide disopyramide have been

45:38 developed for arthas and has been around around for decades it's kind of it's a very old medication how however it fell out of favor of cardiac electrophysiologists for a while before it was rediscovered to have favorable effects in patient with obstructive hypertropic K and the favorable effects are due to its negative inotropic effects therefore opens up the lvot obstruction and the basa constrictive properties therefore maintaining the block pressure and avoiding synop it's been shown that disopyramide can decrease the lvot gradient now if we have tried all of these

46:25 medications and and if the patient still feel REM feel symptomatic then we have two invasive options for septo reduction therapy one is septo myectomy this is an open heart surgery and what's happening in the O during septo myotomy is that the surgeon places an incision on the uh on the aota through the aota and looks down on the LV through the aoic valve then the surgeon takes out a scal bell and cuts out a chunk of the hypertrophy basil by cardium to create a canel so that the blood can go through

47:13 without obstruction wait okay the other option invasive option for sepal reduction therapy for symptomatic relief of obstructive hypertrophic copy is alcohol sepal ablation um surgical myotomy has been around since 9058 whereas this alcohol SE ablation technology is irely new and it has been developed since 1994 and I'm going to probably go the actual procedure starts with

48:00 selecting the appropriate septo perator branch and obstructing including the branch with the balloon catheter this is done to prevent the alcohol from going back to the LED which would create a massive mardio infection in in the entire my uh anterior wall so after confirming that there's no backflow we inject let me move it yeah we inject an intra intravascular Echo contast and take a look at the area with echocardiography like this to predict which area will be undergoing necrosis then we'll inject 100% pure alcohol into the sepor artery

48:48 and we usually see some effect immediately by a reduction in the lvot gradient this is an example of homodynamic uh monitoring before and after alcohol HP opulation you can see that there is a huge gradient between LV and aota which disappeared immediately after the alcohol sep ablation into the septo parator artery for the sake of the time I'm going to just skip the results or the real email that uh we received

49:35 um and there are three the most important thing after SE ablation is to assess for pacemaker because the most common complication after alcohol SE ablation is conduction defects requiring a permanent Pacemaker and this is because the conduction system or the AV node is very very close to the S perator Branch as you can see in this figure so this take uh brings us to the takes on message two uh number four the most common complication after sep ablation uh is conduction abnormality requiring permanent pacemaker this algorithm is a summary of

50:20 the symptomatic Management in patients with hypertrophic copy as I stated the first option beta blocker if we fail or if the patient cannot tolerate beta blocker then the second option verl or DM if symptoms persist we can try disopyramide if we fail medical therapy then we're going to be talking about the uh invasive options such as um Surgical myectomy and alcohol septi oblation and this is a the final slide uh what we have have reviewed so far was the treatment options for obstructive form of hypertrophic card in contrast therapeutic options for

51:06 nonobstructive cardom hypertrophic cardiopathy are fairly limited we can still use beta blockers and calcium chel blockers however diamide is not indicated in patients with non-obstructive hypertropic kopy in addition there if there's no OB no obstruction then invasive therapeutic options such as Surgical myotomy and alcohol sep ablation does not work so they're not available so I hope I have successfully conveyed this take home message to you um and I hope I have convinced you today

51:52 that HCM is the most interesting heart disease thank you for your attention thanks Dr Shaman I really appreciate it we'll have to give the other cardiac diseases a chance to argue for their uh moment in the Sun so we have a couple of minutes for questions before we start I just wanted to remind everybody of hayato mata's email from Saturday morning which highlighted the new system that that Sinai has put in place this year for getting CME credits uh I will put the directions in the chat but it's for most people it's going to be useful to look at the original email and um and look at the attachments there

52:38 basically you have to set up a cloud CME account which probably most of you have already text your cell phone number to the account and then between an hour before Grand rounds and an hour after Grand rounds text the secret code which today is 4926 to that number so that you get instantaneous credit so I'll let everybody do that um I'll also let people either put their questions in the chat or unmute themselves um and ask questions maybe uh maybe while we're doing that I can ask uh I don't think we talked much about screening athletes asymptomatic athletes with no family history um for cardiomyopathy what's

53:24 what's the current thinking on that yeah it's actually the one of the most controversial areas in the management or screening of for hypertrophy copy and actually the guideline recommendations are different between European and American guidelines this kind of highlights the scarcity of data and agreement in like in the worldwide standard level but typically so they they do so for the competitive athletes like college level or professional level typically the screening starts with physical examine history taking like the personal history and family history and also EKGs so the these are pretty uh standard worldwide and they have to

54:11 undergo these screening strategies before participating in a competitive Sports at a professional level the controversy is if everyone needs to undergo echo cardiogram because it's as you know time consuming you're talking about I don't know how many like thousands or like maybe millions of people uh across the years would have to uh go into professional level athletic activities and if we have to they have to screen uh everyone in eard then it's going to be a huge um require hose time resources budget and and power uh Human Resources so that that point of

54:57 controversial but we typically start with uh history taking physical exam looking looking for to for mmers and uh and usually EKG and start from there and there's nobody advocating for genetic testing for screening right at this point it's uh not a prime time um it's one could be expensive it's getting cheaper but could be expensive and two the interpretation is it's pretty difficult sometimes if if you have one of those like what we call V or V variants with uh uh variant with un unknown significance unknown significance so

55:46 yeah yep thank you Dr sh um so I have a question um what we should we do if we get a patient like we get an admission for a patient with a known like hulum presented with like um acut DEC compensated heart failure we are like taught uh to like judiciously use diuretic and avoid after load reduction as much as possible but how would you like like um deal with that like yeah it's a great point and uh sorry I didn't have a chance to visit that point but essentially uh almost all anti-hypertensive or cardiac medications are relatively contraindicated in hypertrophic cardos like the hydro uh

56:33 the after reducting agents like calcium CH blockers except for those D and Dam are relatively contraindicated a orb spacon Etc or relatively contraindicated and the diuretics so yeah you got the answer right the the key is to carefully if you you have to use diuretics it's okay to use so if you believe that this patient is volume overloaded then is we do sometimes use diuretics but we have to be very very careful not to volume deplete the intervascular uh volume because if we do then it's going to worth an albitic gradient um so yeah so the the answer

57:19 you have to just be careful use in using diuretics and avoid uh uh after a all reductor as much as possible thank you so much all right it's 1258 I think this is probably a good time uh to quit thank you so so much Dr shamata for joining us always have you back here uh we'll hope to see you again and we'll hope we'll see everybody else next Monday at 12 as our grand round series continues thanks every my pleasure thank you so much