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Hypertrophic Cardiomyopathy Program


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MDNews - WMC's Hypertrophic Cardiomyopathy Program: World-class Expertise and Comprehensive Care.

Monday, July 1, 2019

Break It Down For Me, Doc: What is Hypertrophic Cardiomyopathy (HCM)?

Posted By: Srihari Naidu
blog post

Quite frankly, most patients are confused by this disease. And, understandably so. Not only is the name a mouthful, but the disease affects so many things that it's hard to explain fully. Truth be told, it's a disease that you have to learn about slowly over time, not just in terms of what it is, but also in terms of how it is affecting you. And the disease may have progressed so slowly that oftentimes you may not even know how sick you are. Can't climb one flight of stairs? Well, sure, maybe it's that you're getting older and gained a few pounds during the holidays, and those things certainly aren't helping, but most likely the HCM is the main culprit here. I often tell patients it's like putting on glasses for the first time. That's when you realize you couldn't see properly without them!

Bottom line is this is most likely a genetic problem for the vast majority of you. But, it can show up anytime in life. Lots of genes are like this. They are there at birth, but when they actually get activated may be influenced by lots of different factors, even in the same family. So, in our HCM center, I've seen patents first diagnosed at age 3, some as teenagers, many in their 20s, most in their 30s to 50s, and quite a few in their 60s to 80s. These older people probably had the disease for decades, actually, and are often misdiagnosed accidentally as other problems. And that's because this disease is confusing to many doctors too! Hopefully over time we will diagnose HCM earlier in life, as by the time people are 60-80 years old, HCM has caused problems in the lungs too, or caused certain arrhythmias, making it that much harder to treat, and very unlikely to reverse fully. That's why empowering people to recognize the symptoms and ask about this disease is important. We want to get to you earlier! HCM is a heart failure problem. I know we don't like to use the term, but that's what it is. The heart simply can't pump as much blood as your body needs. When that happens, the first symptom is plain old fatigue. Usually, early on, this is a problem only on exertion; but over time it gets worse and worse, and eventually becomes congestive heart failure (where the blood backs up, and water builds up throughout the body, but mostly in the lungs) over the next few decades.

But why does it cause heart failure? The heart gets too thick, which makes it stiffer. Even though it can squeeze well, it doesn't let enough blood in to begin with. Think about a thick balloon versus a thin balloon. The thick balloon is harder to fill, right? So, even though it squeezes great, your heart can never let out the normal amount of blood because it didn't have enough blood to work with to begin with. The thicker the heart, generally speaking, the more stiff it is - something we call "diastolic dysfunction".

The majority of HCM patients have a second problem, called obstruction. Unfortunately, roughly 75% of HCM patients have the thickening at precisely the wrong location - right where blood is leaving the heart, a place called the outflow tract. This area is already narrowed, like a tunnel, and all the blood has to leave through it. The thick muscle bulges into this tunnel area, and can actually get completely in the way of blood leaving the heart. This makes the blood pressure drop, and forces blood to stay in the heart, and oftentimes bust through the back door - called the mitral valve, which becomes leaky - and go backwards to the lungs. And that's where the shortness of breath comes in. And, if the muscle gets in the way for longer than 5 seconds or so, there may be too little blood to the brain, causing rapid drops in blood pressure that can make you feel like you're going to pass out.

There are other problems with HCM too. As the blood backs up into the lungs, it stretches the top chamber of the heart called the atrium. This can and does cause an arrhythmia called atrial fibrillation in a large number of patients, which itself is a problem that can cause worsening heart failure and stroke. Stroke happens because the blood can stagnate in that atrium - blood that doesn't move quickly tends to form clots. And, finally, the muscle in HCM in not just thick and stiff, it's also abnormal. If you look under a microscope, the muscle cells don't line up properly, and there's often some scar in there between the muscle fibers too. Together, they can put you at risk for another arrhythmia, called ventricular tachycardia or ventricular fibrillation, and this is the reason some patients, usually young patients, can die suddenly, most notably during or after intense exercise, but also sometimes when they are doing nothing.

Taken together, though, the sudden death aspect is much lower in terms of likelihood than all the heart failure symptoms I mentioned. And, that's an important point. HCM is a form of heart failure mainly, and a form of sudden cardiac death too but at a much lower risk. In general, the sudden death aspects are more important when your'e young, but most patients make it past those years and the heart failure symptoms then become the main problems. Most patients actually live into their 80s, so it's important not to focus too much on the sudden death aspect. And, in fact, it is likely that if you get the heart failure under control, and help the heart work better, the risks of both types of arrhythmias will go down.

Want more good news? We have lots of tricks to make you feel better and live long lives, some of which require medications, but others that can actually get the obstruction to go away and let the heart shrink down to more normal size and function over time. And there are some new medications in the pipeline too. Coming to an HCM center with lots of expertise on the disease, and lots of experience seeing thousands of patients, is really the key. And while these programs are present all over the country, please do your own research. In general you should go to programs with thousands, not just hundreds, of HCM patients, experts who have been treating HCM for over a decade, and programs that do a large number of the two procedures to get rid of the obstruction - called myectomy and alcohol ablatoin - generally felt to be around 25-30 per year. Very few programs do all of this, so it's important to educate yourself and be your own advocate. Ask questions, and get the answers you are happy with. Hope this helped!

About Dr. Srihari Naidu

Dr. Naidu is a nationally recognized interventional cardiologist who is also known for his expertise in the treatment of patients with Hypertrophic Cardiomyopathy, the most common cause of sudden death in athletes and a common cause of heart failure in people of all ages. An author of two textbooks, Hypertrophic Cardiomyopathy and Interventional Heart Failure, Dr. Naidu runs a large multi-disciplinary program in the diagnosis and management of Hypertrophic Cardiomyopathy, has treated over 1000 patients with the disease over the past 15 years, and is especially known for performing the minimally-invasive alcohol septal ablation procedure and teaching the technique throughout the country. Dr. Naidu is a Former Trustee of the Society for Cardiovascular Angiography and Interventions (SCAI), and current Trustee of Brown University, his alma mater. He is the author of over 100 original manuscripts, including co-author on two national guidelines (Stable Ischemic Heart Disease and Hypertrophic Cardiomyopathy), and chair of two consensus statements (including the Cardiac Cath Lab Best Practices document). Dr. Naidu is Board Certified in Internal Medicine, Cardiology and Interventional Cardiology, and runs a busy clinical practice seeing patients in offices in both Long Island and Westchester. For more information about Westchester Medical Center’s HCM Program please visit www.westchestermedicalcenter.org/HCM