Guest: Dr. Srihari Naidu
Presenter: Neal Howard
Guest Bio: Dr. Srihari Naidu is a nationally-recognized Interventional cardiologist and director of the Hypertrophic Cardiomyopathy Program at Westchester Medical Center, NY.
Segment overview: In this segment, Dr. Srihari Naidu discusses a heart disease called Hypertrophic Cardiomyopathy, or H-C-M.
Health Professional Radio – Hypertrophic Cardiomyopathy
Neal Howard: Hello and welcome to the program, I’m your host Neal Howard thank you for joining us here in Health Professional Radio. You’re trying to be healthier eating right, watching your weight, working out on a regular basis, but there are some genetic conditions that a healthy lifestyle just may not protect you from. Our guest in studio today is Dr. Hari Naidu, nationally-recognized interventional cardiologist and Director of the Hypertrophic Cardiomyopathy Program at Westchester Medical Center in New York. Welcome to the program Dr. Naidu.
Dr. Hari Naidu: Thank you very much for having me here.
N: Thank you for joining us today. What is Hypertrophic Cardiomyopathy or also known as HCM?
H: Sure, so Hypertrophic Cardiomyopathy is a relatively rare form of heart disease, but not totally rare. It’s about 1 in 500 people, so it’s far more common than we previously thought and certainly more common than other diseases that people may have at the tip of their tongue like multiple sclerosis which is 1 in 700 people. Instead of being a disease of the heart arteries such as atherosclerosis or the heart conduction system that requires pacemakers. These are the disease of the muscle itself, the muscle gets abnormally thickened. As it get thickened, generically programmed to do such that happens over the course of patient’s lifetime. Usually in their teenage years and 20’s, 30’s, and 40’s. When it’s actually happening in their teenage and 20’s years, they typically are higher risk of certain types of arrhythmias. If you think about it, every year we have unfortunately a few incidents where athletes about suddenly on the field. Typically basketball or soccer, if you look at their autopsies unfortunately this is the most common cause of death in that population. Now most people make it past that and they don’t have that problem thankfully and then they go on living lives were they just get worsening and worsening heart function and they notice that as fatigue, shortness of breath, palpitations, dizziness, light headedness and sometimes passing out. And this is really the heart muscle getting in the way of itself, it’s not letting the blood circulate out of the heart because the heart is getting too thick, there’s less and less room inside the heart now from blood to circulate. And the physical aspect of the thickening actually gets in the way of valves and gets in the way of the blood, leaving the heart and ultimately it’s kind of hurting itself in that way.
N: Is there anything that can be done to counteract to some of these symptoms? Or is this something that is just going to happen genetically no matter what you do, whether you’re a child, athlete or an adult, or just do going about your daily business?
H: That is a great question. With all the people feeling that they’re not empowered at all here, there is no question. If you have it in your family, you got exact disease, you got the gene from one of your parents and that’s 50-50 inheritance. So if one of your parents has it or 50-50 chance of your each kids being affected. It will grow and it will be pre-programmed to grow to a certain extent. There are things you can do though, there are certain medications that help alleviate the symptoms and we are certainly looking for new medications. For 40 years now, we haven’t any dedicated medication for HCM. But thankfully, we live in an are now where people are being much more precise we call it ‘Precision Medicine’ where companies are now looking at how to target the individual actual problem for disease. One company looking at this is a company called ‘Myocardio’ that has a new drug in development. We’re hoping will be targeted for HCM for the next few years. If those medications don’t work very well, in about 10% of people they go on to some major surgeries which of the type of open heart surgery called ‘Septal Myectomy’. The skin actually shade on the muscle, and then there’s also a minimally invasive form called ‘Alcohol Septal Ablation’ that we do quite a bit here, Westchester Medical Center and we’re well-known for that. Which mimic surgery and tries to develop away or shrink away some of that muscle. But most people really need medications and really the Holy Grail is trying to prevent this from getting to this a level of thickening through medications or more precision medicine.
N: Just to be clear, all of the technology that you’ve been mentioning, the new drugs and things of that nature, we’re talking about ongoing treatments, whether invasive or fully less invasive but there is still no cure. Are any of this development (crosstalk), is their goal a cure from the onset? Or are we still talking about treatment first?
H: We’re still taking about treatment, there is actually no cure for it right now. If you have the gene, that means it’s in your DNA can’t really get rid of that to the new DNA. But these medications and these treatments can often modify the disease so that it’s not, it’s the heart functioning better. There is some thought that some of the medications if they work well and people, could they possibly prevent the disease from progressing? We’re still away from that, but that will be the kind of the Holy Grail of these disease if we can find medication that can prevent that.
N: When it comes to diagnosis on a DNA level, can you determine if someone is a candidate for this disorder early in life? Or is this something that you have to wait for the symptoms and then determine genetically that they’re pre-disposed?
H: So most people who’ve come to our attention have some symptoms and then that prompts to physical examination, which prompts an ECG, an echocardiogram which is the way we diagnosed it because it can look physically at the heart muscle. That being said, there is increasing amount of genetic testing being done. We have somebody with the very obvious case, we can then send them for genetic testing which is simply a blood sample these days. About 70-75% of the time we can find the gene that’s running in their family. That then allows us to go back to find people in the family who have no significant thickening, but are at risk of it because they carry the genes. We are diagnosing people both ways. One is when they already have symptoms, when we try to get that earlier. And two, is even before that, so that we can then really watch over some more carefully, protect them, make sure they avoid the test or activities that may cause the sudden death, the test with athletics for example and then really follow them overtime to see if they develop some of the heart failure symptoms and try to prevent that.
N: Are there any treatments that greatly diminish the drastic lifestyle change based on the heart’s functioning? Being so diminished if you give the athlete who you’ve identified as being a candidate for this and you start treating him in such a way. Is there anything that can allow him to continue being an athlete for at least some period of time? Or is that a No, once you identified the problem?
H: Yes. Well to answer to your question in theory, there are procedures in the medications and mostly the procedures that can really improve the heart functioning and so patients go back to feeling great. And some of these patients feel like all of a sudden, “I can go back to exercising out of proportion or to … an aggressive level.” The truth is, since all of the muscle has a gene that’s still something that we don’t want patients to do. You start feeling better with the treatments or the medications, they’re still at risk of sudden arrhythmia. Unfortunately, once you have the disease, those types of high levels of activities are out. That doesn’t mean you can’t be fit, it just means you probably can’t play professional baseball or basketball or the equivalent of those types of activities. It does mean that you should stay fit so that your body is as good as shape as possible to tolerate the heart dysfunction. That means catering your exercise routine in conjunction with the cardiologist or an HCM specialist so that you stay fit, but don’t tax your heart too much.
N: We’re talking DNA so that suggests family, now this being a family type of a disease, when it comes to help, and education, and support, what is available for something that is 1 in 500 people such as this disease?
H: Yes. For families the best thing would be this National Advocacy Group. There’s a wonderful one in New Jersey called the ‘Hypertrophic Cardiomyopathy Association’. They’ve been around for over 20 years. The founder is a patient and they can be reached at www.4hcm.org. We do allow and will be happy to take care of any patients here at Westchester even if it’s via email, we have a dedicated HCM email which is HCMProgram@wmchealth.org and we’re happy to answer the questions and just be a resource for people even at their local areas. Finally, one thing that came out last week, actually at the American College of Cardiology annual meeting is this specific iPhone app called the ‘HCMCare’. That’s a free app that can be downloaded, it was put together by some of our friends at Duke University Medical Center with the HCM program there. And they have a lot of resources on there as well as links to all kinds of other educational venues.
N: Great. Well I appreciate you talking with us doctor.
H: My pleasure, I’m happy to.
N: You’ve been listening to Health Professional Radio, I’m your host Neal Howard, in studio with Dr. Hari Naidu. He’s a nationally-recognized interventional cardiologist and we’ve been talking about Hypertrophic Cardiomyopathy and the program that he heads at Westchester Medical Center in New York City. Transcripts and audio of this program are available at hpr.fm and also at healthprofessionalradio.com.au. You can subscribe to this podcast on iTunes, listen in and download at SoundCloud.