Chapters Transcript Video Case Study in Hypertrophic Cardiomyopathy Uh for those who don't know me. Uh I'm, I mean, I'm a Harford cardiologist at sa um associate professor at EV MS um working together. So these are my disclosures. I'm not gonna be discussing anything off labor uh or investigational use of any drugs or devices. So I'm starting with uh a uh case of mine which is a patient who presented to one of our center um sister facilities. Uh He's 23 year old guy previously healthy and uh he works in a car shop actually and he was having worsening shortness of breath and chest pains for the past two years. And uh this pain became so bad that he came to the emergency room. So they did an EKG on him on presentation that they had like LVH and uh ST depressions L VA voltage criteria with some report abnormalities. Everybody gets troponin. His troponin were positive. So 23 year old male with troponin positive, uh they want to do some work up for his scheme and knowing that the pre test probability for having obstructive disease is low. So they did AC T A on him. Uh CT Angio and um as you can see here on the CT scan um on the left side, left panel, the coronary were patent. But what is more impressive is on the left panel, he has a pretty thick myocardium, thick heart. And uh that definitely uh raised some concerns uh for our session today, which is hypertrophic cardiomyopathy. So att E was ordered and uh basically, this was his echocardiogram uh on presentation uh the emergency room. And as you can see here as a pretty thick myocardium LV was pretty severely hypertrophied. And for those images here and uh who's also look at echoes and echo texts uh in the uh meeting, we see also that uh there is kind of systolic interior motion of the mitral valve and cly the uh mi mitra valve leaflet is touching the LV, septal wall and he has some uh regurgitation, which is a mitral regurgitation. So all of these, basically, as you can see here, uh it's still a picture where we see um the mitra regurge and a trust, you can see the gradient like four V squared and um do the calculations. Uh the pressure grade in the LVOT area is significantly high and when they ask him to strain and do a valsalva and you can see her significantly increase in his uh pressure. So, uh this gentleman has significant LVOT kind of obstruction and which is a grade in between the left ventricle um aortic valve. So, what we think about as a uh team is that somebody with thick heart with some uh gradient across his um outflow track, we think about hypertrophic cardiomyopathy. So, what to do next in this individual. He was discharged home and he was referred to the Hartford Clinic. So, uh where we basically have a multi dissimilar team uh from cardiologists uh in our clinic in Norfolk, we have the dietician social worker and financial uh coordinator. So it's a multisystem approach and we work closely with uh different subspecialties uh including EP and structure heart closely and surgery as well. So he was referred to our clinic and I saw him in clinic and we took a detailed history for from him and there's no known family history of sudden cardiac death or hypertrophic cardiomyopathy. Um So what we did, we started him on a beta blocker and then eventually put him verapamil, which is nondihydropyridine. Um And then we ordered also genetic testing which uh is important, especially people with HCM or newly diagnosed cardiomyopathies, uh event monitor and cardiac MRI and we'll see some of the results of these stuff. So this is uh the work up um that is uh published recently at um uh Jack Journal, which is Journal of American College of Cardiology, published, published in June of this year. I don't know if it's clear and um which basically details uh the work up for patients with suspected HCM um or with family history of hypertrophic cardiomyopathy and the most important thing is taking detailed history and of any family history of HCM, do the exams and uh do diagnostic work up including A KG and uh echocardiogram among others. And then you go through the different um pathways being a phenotype positive, which means um you know, you have um the disease, the gene for the disease and you have the uh thickness of the heart and sometimes the phenotype negative uh which is you have the mutation, but you don't have the uh disease process. So it's a different pathway. Um just wanna introduce it now and I think my colleagues will go through it later on and this is um how the treatment um that is recommended according to the AC CH A uh guidelines. Again, this is just published a couple of months ago at the Journal of American College of Cardiology where it shows that uh it's so important to first identify patients and knowing whether these patients have obstructive symptoms, which means that they have some significant gradient across the valve and they have the symptoms with it and if they don't have it, so we're gonna go with uh the obstructive physiology on the right. And again, for most of these patients, we try to avoid uh as much as possible peripheral vasodilators. Be it nitro endure, for example, or hydrALAZINE or dihydropteroate and nifedipine. Um So we try to avoid, avoid them. And um we need to start these patients usually on a beta blocker or calcium channel blockers, but the calcium channel blockers are usually non dihydrocodeine such as verra or dilTIAZem. So, um, as you see here, if the symptoms persist, uh, despite medications, you can consider other options as we're gonna be seeing later, um, through this, uh, presentation. And if the patients do not have the obstructive symptoms, uh, the physiology, uh, you also look at the ejection fraction if it's more than 50% or less than 50%. If it's less than 50% you see if these patients qualify for I CD and you wanna make sure you optimize them and look at other causes that could lead to drop an ejection fraction and make sure they are on guideline directed medical therapy as well too. So um this other images here for him, as you can see here, these are the Tragal echocardiogram images. Uh You see um how thick his myocardium um is, it's a pretty severe hypertrophy septal wall thickness is significant and you can see also the systolic interior motion uh where uh where also some um ma regurgitation was directed. And um this is the MRI uh which thanks Doctor Veer for pulling this MRI image for us. So this is the, you can see the asymmetric hyper of the left ventricle um which you can, I don't have the um pointer here, but you can see the wall, it's on the uh the middle. It's pretty uh thick. And also one of the important things of the MRI is not just to determining the structure, but also to look at what we call if they have uh hyper enhancement or scar formation. Because the higher the scar formation, the higher the risk of sudden cardiac death. And there is where the electrophysiology team comes in. So you can see some patchy involvement, those uh white stuff uh and the myocardium and these signify um fibrosis and the larger the number, the higher the chance of sudden cardiac death. So, um again, with this individual, basically, um had around 23% of uh his myocardium with scarring, which is pretty significant for sudden cardiac death. Uh We did genetic testing and genetic testing came back positive for the mycin uh heavy chain uh which is uh C which is typical Patno patno uh mutation for patients with HCM. Um again, solidifying the diagnosis, but again, not everybody with HCM have positive genetic testing. So, if your genetic testing is negative with HCM does not mean you don't have the disease. Um So his genetic testing was positive and the event monitor, luckily he didn't have uh ventricular tachycardia or significant ventricular dysrhythmias because if they have that it is usually high risk for sudden cardiac death. Um But again, knowing his uh ventricular wall thickness of 3.4 it's by itself one of the criteria for kind of uh primary recommendation for I CD. If it's more than three centimeters if uh there's a family history of sudden cardiac death, if the patients have syncope uh or ventricular dysrhythmias, uh or they have significant fibrosis, um they are definitely high indication for uh primary prevention I CD. So, uh the patients remain symptomatic despite being on a beta blocker and uh verapamil. So, what is next? So if you wanna go through that, uh basically uh guideline recommendation, they can try some mycin binding in uh inhibitors um or add di di all or then you have to consider other options, which is um um interventional ones, which is uh working closely with against structure, heart team like alcohol seal ablation or Myectomy basically. And knowing how severe this uh patients uh ventricle wall thickness was, it's like again in 3.4 centimeters which is significant. Um And um we met and we spoke with uh doctor um at that time, Doctor Summers, Doctor Corcino, Doctor Hedley and Patel and uh we decided the best option for this uh mutual patient was to proceed with Myectomy. And after his myectomy, the plan was to proceed with I CD as a primary prevention as well. So we proceeded with Myectomy and um this was kind of the intra op T for the patient. Um but we did the TT E prior to discharge. And as you can see here, the mitra valve regurgitation has improved and the wall thickness has gotten better. But uh on the next image I will be showing the CT scan which is a pre and post because he had a uh post uh ablation CT, the pre is on the right left side. And as you can see here, the um the outflow track is pretty narrow. Uh The septal wall is pretty thick and you can see the mitral valve opening and that's in diastole where the leaflets are open. And on the right side, you see this gap that was done um during myectomy. Uh and there is less, significantly less gradient and I was in the operating room with Doctor Cortina at that time when he did the procedure. And um we decided not to do further because of the mitral valve. We tried to spare the patients from a mitral valve intervention knowing how young he is. Um So the plan was to, we did a myectomy and he did well and he was discharged and he's, he's back to work and doing great and no chest pain and shortness of breath. Um So this is a case that I just wanna share with you um from the surgical standpoint, how we can manage these patients. And sometimes we're seeing um younger and younger patients presenting with different physiologist and path of like diseases, be it HCM amyloid and whatnot. So somebody coming in to the emergency room with chest pains or shortness of breath. Um It's not usually most of the time anxiety or whatever it is. It's just we have to think sometimes beyond and do the necessary work up. Published November 14, 2024 Created by Related Presenters Amin Yehya, M.D. Sentara Advanced Heart Failure Center View full profile
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