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Hypertrophic Cardiomyopathy (HCM) involves more than septal thickness. Dr. Nicholas Smedira, Surgical Director of Cleveland Clinic’s Hypertrophic Cardiomyopathy Center describes what happens when a patient has hypertrophic cardiomyopathy. A team approach is used to understand the anatomy and cause of obstruction. Many surgical approaches are available to individualize the care for the HCM patient to address the obstruction or treat the heart when a patient has HCM without obstruction.

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Hypertrophic Obstructive Cardiomyopathy: The Cleveland Clinic Approach to Surgery

Podcast Transcript

Announcer:
Welcome to Love Your Heart. Brought to you by Cleveland Clinic's Sydell and Arnold Miller Family Heart, Vascular and Thoracic Institute. These podcasts will help you learn more about your heart, thoracic and vascular systems, ways to stay healthy and information about diseases and treatment options. Enjoy.

Dr. Nick Smedira:
Hi, I'm Dr. Nicholas Smedira, and I thought I'd take this opportunity to give you an update on the Cleveland Clinic's approach to the treatment of patients with hypertrophic obstructive cardiomyopathy. I have my team here that's doing the video taping and we're all masked and appropriately distanced. So I'll take the mask off or down at least, so it'll be easier for you to see me, and hopefully it'll be easier for you to understand what I'm saying.

Dr. Nick Smedira:
Over the course of, I think about 25 years, we've had a very large clinical experience in treating patients with hypertrophic obstructive cardiomyopathy and developed some novel surgical techniques. And when I see patients, they often have a lot of questions about what is hypertrophic obstructive cardiomyopathy. And there's a lot of confusion around the idea of hypertrophy, hypertrophic, obstruction, and cardiomyopathy. So I thought in this video, I explain our current knowledge of those aspects of the treatment of hypertrophic obstructive cardiomyopathy. Let's deal with the easy part first.

Dr. Nick Smedira:
Cardiomyopathy. What does cardiomyopathy mean? Cardio means heart, myopathy means muscle. And basically from my perspective, means something's going on with the heart muscle and there's dozens and dozens of cardiomyopathies. So it's almost a misnomer for this disorder because in many patients, the heart muscle is actually working perfectly fine. The focus for many, many years has been on the first word hypertrophy and hypertrophy means increased muscle thickness. So patients with hypertrophic cardiomyopathy have for many, many years thought to need and have thickness of the heart muscle. And the area we focus on is the septum. And the septum is the wall that separates the two chambers of the heart, the blue blood and the red blood. So hypertrophic cardiomyopathy. And for us, when we think surgically is about the thickness of the septum and what we have shown here at the Cleveland Clinic over a number of years is, and this is the important part.

Dr. Nick Smedira:
You don't necessarily need to have hypertrophy to have obstruction. And that's, I think that the key thing, and then there's a lot of confusion about what is meant by obstruction because use obstruction and talk about obstruction and a lot of things. When people have a blockage in their coronary artery, we call it an obstruction. When people have problems with valves, there can be obstruction. This obstruction that we're talking about is when there's a problem with the blood getting out of the heart, related to the septum in the mitral valve. And for some reason, they come together when they should be separating and they block or obstruct the blood, getting out of the heart. And then I'll show you a diagram of how this looks like when there is obstruction occurring and when the blood can't get out of the heart, it has to go somewhere.

Dr. Nick Smedira:
So it goes back into the lungs and that's why people will commonly feel short of breath. If enough of it can't get out. And it can't go to your brain. That's when dizziness or passing out what we call syncope occurs. So that the reason that there is obstruction is because of the septum in the mitral valve, get close together when they should be staying far apart. And that's, what's called systolic anterior motion of the mitral valve or SAM. So those are some of the terms you might hear that there's SAM of the valve, where you may have been told that the valve leaks and you have a problem with the mitral valve, but the reality is the mitral valve, 90% of the time is normal. And it's this combination of the muscle in the valve that causes the problem. So from the surgical perspective and making you feel better perspective, we want to get rid of obstruction.

Dr. Nick Smedira:
And so my colleagues and I look for reasons that you have obstruction and one may be the septum is thick, but that's not necessarily something that always has to happen. The other reason is there might be a problem with the mitral valve, or you can have normal valves and normal septum, but over the course of a number of years, especially as we age the heart actually twists on itself. And the pathway for the blood to get out of the heart goes from a straight shot to a right angle turn. So the blood has to turn really sharply to get out. And that generates this obstruction and the importance of all this is that it takes a comprehensive review of the anatomy, either with an echocardiogram or with an MRI or with both to understand the anatomy. And then maybe most importantly, we have to push the heart.

Dr. Nick Smedira:
We have to stress the heart to induce obstruction. Commonly for many, many folks. If they're sitting down at rest, when we look at the heart, there'll be no obstruction. It's beating very calmly. And so many patients have been told there's nothing wrong with their heart. The heart is perfectly normal, which it is. It's a perfectly normal functioning heart, but when it's stressed, stressed meaning it's speeding vigorously, it can generate obstruction because of all this, we've developed a number of techniques that evaluate both the muscle and the valve, and then focus on what we have to do to eliminate the obstruction. We take muscle, when there's excessive muscle, we reduce the length of the leaflet of the mitral valve when it's too long, when the leaflet is by just its nature, set up to be too close to the valve, we pull it away with what we call a reorientation approach and in our hands that can relieve obstruction in over 99% of patients who have obstruction.

Dr. Nick Smedira:
There's another group of patients, much smaller that have no obstruction. They have very thick muscle, which limits the ability of the heart to fill, but they have no obstruction. The blood can get out perfectly well. It just can't get in particularly well. And we have done the similar operation of the myectomy to open up the cavity. So there's more volume in the cavity, even though there's no obstruction. The idea is if you get more in, you can kick more out. And that's for folks that have hypertrophic cardiomyopathy without the obstruction component. Previously, it was thought that there was nothing you could do though for those sorts, because they didn't have obstruction. But we believed in a certain subset of patients that getting rid of that extra thickness increases the capacity of the chamber. And because those hearts are very strong, they kick out all the blood that comes in.

Dr. Nick Smedira:
They just can't get enough in. So it's become more complex than just having thick muscle. We think we need to think about the muscle. We need to think about the valve. We need to think about the anatomy and its orientation and in some patients, all that can be perfectly fine. And there just is not enough space within the chamber. And we continue to, or we consider an operation to help them. Most importantly, it requires a team that has a deep knowledge in the imaging, understanding the pathophysiology, the ability to do stress testing and then to have the surgical team that knows how to manage all these nuances that allows us to really comprehensively treat patients with hypertrophic, obstructive cardiomyopathy and hypertrophic cardiomyopathy.

Announcer:
Thank you for listening. We hope you enjoyed the podcast. We welcome your comments and feedback. Please contact us at heart@ccf.org. Like what you heard, subscribe wherever you get your podcasts or listen at clevelandclinic.org/loveyourheartpodcast.

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