Hypertrophic Cardiomyopathy (HCM) (Dr Lever 1 20 12)
Harry Lever, MD
Medical Director, Hypertrophic Cardiomyopathy Clinic, Department of Cardiovascular Medicine, Cleveland Clinic Miller Family Heart & Vascular Institute
Friday, January 20, 2012 - Noon
Hypertrophic cardiomyopathy (HCM) is a complex type of heart disease that affects an estimated 600,000 to 1.5 million Americans, or one in 500 people. It is the most common cause of sudden cardiac death in people under age 30. Dr. Harry Lever, cardiologist and HCM expert from the Miller Family Heart & Vascular Institute answers your questions.
Symptoms of Hypertrophic Cardiomyopathy (HCM)
HCMGuy44: Would a portable Oxygen supply help in the shortness of breath I experience during activity such as walking and simple chores?
Dr__Lever: If you are that short of breath you need to be first thoroughly evaluated to see what is going on.
Duncan1: How much of a role does diastolic dysfunction play in shortness of breath in relation to HCM?
Dr__Lever: It is very important.
mike83: I have a resting gradient of 6 mm. But when I raise my HR, it increases to 144 mm. Recently diagnosed and on Calcium Channel blockers. Was told to keep my HR below 110. This is very hard as I get winded walking down a hall and was very active in running etc before dx. Was told I would need surgery at some point. Why would I not want to have the surgery now in my 30's before it gets worse?
Dr__Lever: If you are short of breath and having symptoms, you should be evaluated for surgery.
hcmalisa: what are the signs of severe heart failure? Is it all based on ejection fraction?
Dr__Lever: No - it is shortness of breath; fluid in the lungs and peripheral swelling.
overthehump_: do symptoms of CHF come and go? or is that like being a little pregnant?
Dr__Lever: The symptoms can come and go. But if you start having symptoms you need to talk to your doctor.
Diagnostic Testing and Hypertrophic Cardiomyopathy (HCM)
mike83: Can you explain and give a range for gradient with a person for HCM?
Dr__Lever: They can range from nothing to over a 100 mm of mercury. When we look at an echo we are looking at how thick the muscle is; the interaction of the mitral valve with the septum; and the orientation of the papillary muscle.
smurf: by "if it is more severe" are you referring to the thickness?
Dr__Lever: It can be thickness and scarring - both.
hcmalisa: if my cardiologist home did an echo 4 yrs ago and didn’t see my HCM. but I have had a murmur off and on since I was a teen... does that mean it was there and they may have missed it.. or is it more likely my heart changed in the past 4 years?
Dr__Lever: It is more likely that your heart changed.
newtothis: How can you check for scarring if you can not do an MRI (because of ICD)
Dr__Lever: There is no way - that is why it is important to do an MRI before you put an ICD in.
mymickey: Having a negative biopsy on the septal wall does not conclude anything?
Dr__Lever: we would need more information to answer your question.
khoffman: Would you please explain the term gradient?
Dr__Lever: That means that there is a pressure difference between the aorta and the left ventricle caused by the mitral valve striking the septum.
For instance if the blood pressure that is measured in your arm is 120 mm Hg and at the time of an echocardiogram we measure a gradient of 100 mm, that means the pressure in your heart is 220 mm Hg.
hcmalisa: is it possible.. to not have a great deal of "thickness" yet have a large gradient....
Dr__Lever: Yes. That is when the mitral valve and papillary muscles are involved. The papillary muscles are the structures that support the mitral valve.
judyld71: my 16 year old has had an icd put in. He has had a cardiac mri before surgery> What will they be looking for when they continue to do an echo?
Dr__Lever: They will be looking for development of obstruction; function of his left ventricle; and particularly at his age - any further thickening of his heart.
jvaldez: What are the problems that you mention related to "The mitral valve can cause problems as can the papillary muscles." How can a HCM patient monitor this, what systems are associated with this? Thanks!
Dr__Lever: It is monitored by doing an echocardiogram and sometimes an MRI scan when needed.
sophiemacie: Is the BNP level generally high for pts with HCM? Why or why not?
Dr__Lever: No it is not usually high. If it becomes high, it is a potential sign there may be problems.
mymickey: What is a BNP?
Dr__Lever: It is a hormone that comes from the heart. When elevated, it is a sign of heart failure.
sophiemacie: Are the results of a MRI beneficial in any way other than checking for scarring? Does an MRI give you any other information?
Dr__Lever: We are looking at the papillary muscles and alignment of the mitral valve.
Medical Management of HCM
maineobg1939: Are there any objective, measurable parameters for determining the effectiveness of treatment for HCM? Or, stated in another way, does any treatment of HCM affect the rate of progression of cardiac muscle hypertrophy? I have been treated for HCM for 14 months and have seen no significant change in Sx (slowly increasing DOE over 15 years). I am 72 Y/O, have some Sx of postural hypotension and less well-controlled moderate hypertension since HCTZ was D/C 'd and I was placed on metroprolol. I wonder whether I have any benefit from treatment. I have minimal interventricular septal thickening, minimal symmetrical ventricular wall thickening, mitral valve prolapse with significant regurgitation only on exercise. Am I really getting any benefit from RX?
Dr__Lever: There are varying types of HCM. Some have more valvular disease than muscular disease. We would be happy to see you to evaluate you further.
mike83: What if any new treatments/medications can you tell us are being tested to improve or correct HCM? Do you see treatment changing drastically in the next 5-10 years?
Dr__Lever: There are no new medications that I no of at this time. I do not see treatment changing in the next 5 - 10 years. Surgical techniques may change but no new drugs on the horizon right now.
Duncan1: Is Diltiazem an effective medication for treating HCM?
Dr__Lever: Yes - I use it a lot.
mark720: Is dilitazem better than toprol for hocm patients
mike83: Diltiazem is a generic. My pharmacy always will give me a generic if available. Is this an acceptable generic?
Dr__Lever: It depends. First - if you are feeling well on one preparation, you should not change the manufacturer. For drugs like diltiazem that are sustained release, I prefer the name brand.
The key is if you are feeling well on the drug you are on - to stay on that manufacturer.
jvaldez: After being released from the Cleveland Clinic for my septal myectomy, I was told to take a multi-vitamin with iron and low dose aspirin. I was never given an end date so I've been taking them since (more than a year and a half). Is this recommended?
Dr__Lever: Stop the iron and call my office to check your records about the aspirin.
Surgical Treatment of HCM
mike83: If there is a high probability for correction through a myectomy, why would you not want to perform the surgery for someone in their 30's? This is assuming symptoms will only get worse.
Dr__Lever: We don't like to operate if there are no symptoms; secondly the symptoms do not always get worse; and third, there are always risks of an untoward event with surgery, even though the chances are small.
khoffman: What are the indications used to determine when a myectomy should be performed?
Dr__Lever: You need a resting or provokable gradient greater than 50 mm Hg; loss of consciousness in a patient who has a resting or provokable gradient; or significant shortness of breathe with a gradient. We don't operate on people who do not have a gradient.
mark720: can the heart muscle regrow after a myectomy or ablation?
Dr__Lever: In adults, it usually does not. It is possible in young children - therefore there is a chance that they may need a second operation.
overthehump_: Is it usual to have CHF after HOCM surgery? I tried to take a zumba class and only lasted 2 1/2 minutes
Dr__Lever: It depends on how sick you were before surgery; and if you were severely deconditioned, you may not be able to do much. That is somewhat unusual, and if you are feeling symptoms you need to be seen.
queen53: I had septal myectomy in june 20 2011 I am doing well, have pacemaker My question is now what? What will happen? I know I am not cured.
Dr__Lever: I anticipate you will do fine. You need to be followed as anyone needs to with HCM
jvaldez: Besides septal thickness, what other issues with the heart that can cause issues with a person with HCM (septal myectomy already performed)?
Dr__Lever: The mitral valve can cause problems as can the papillary muscles.
hcmalisa: when should someone think of transplant as an option?
Dr__Lever: when there are signs of severe heart failure.
mike83: what are complications from a myectomy?
Dr__Lever: You can have infection; stroke; heart rhythm disturbance; complete heart block which requires a permanent pacemaker; post pericardiotomy syndrome, which is an inflammation of the sac around the heart and lungs which are treated with non-steroidal anti-inflammatory medications.
mymickey: Can one hope for a normal life span after a myectomy, metaprolol, exercise and an ICD?
LindaS: What is the recommendation for annual evaluations post myectomy? What tests do you recommend? Can it be done less frequently than annually?
Dr__Lever: We do a 48 hour holter monitor and echo once a year. It is important to check the heart rhythm once a year.
Hoppy: I saw you in November, hoping to unmask an obstruction, but we didn't find one. What can be done, for someone with HCM, without obstruction? With high left heart pressures,(pcwp 30), and an enlarged atria, and pulmonary hypertension, is there anything that can be done, to keep the left heart pressures, from destroying the right heart, assuming that the maxium amount of drugs, that the body can handle, are already being taken ?
Dr__Lever: Please call me to discuss your case further.
Arrhythmias (Abnormal Heart Beats) and Hypertrophic Cardiomyopathy (HCM)
Jenny: What are the chances of getting an arrhythmia if you have HCM? And is the risk greater when a person is in the high risk category for sudden death from HCM?
Dr__Lever: It depends on the severity of the HCM. If it is more severe then the risks increase, particularly if there is scarring on the muscle.
That is what that means that you are in the high risk category.
sophiemacie: If a person with HCM has a first degree relative die of SCD, does that qualify a pt. for having an ICD? If the first degree relative had a cardiac arrest and is alive because of an AED, does that carry the same necessity for having an ICD?
Dr__Lever: It definitely qualifies the patient to be evaluated for an ICD. If a person has a SCD event, they should be evaluated for an ICD.
Duncan1: Is left bundle branch block a concern after myectomy surgery?
flyannapolis: I had a short A-Fib duration in Sept. of 2010 My Doc put me on Warfarin Jan 2011, I have had no other A-fib. Should I still be on it?
Dr__Lever: We need to have more information about your heart. You should discuss this with your doctor or we would be happy to see you at the Cleveland Clinic.
hcmalisa: Hello from the HCMA... could Dr. Lever please address the need for risk assessment for SCA to be revisited in those with HCM.. and at what intervals...thank you!
Dr__Lever: Patients need to be seen if they are between the ages of 11 and 21 once a year; and need to have a 48 hour holter monitor once a year. This should be included along with an EKG and history and physical exam. After age 21, probably, depending on how they are doing, at least every 2 years.
smurf: Is there any data around the instances of having A-fib among those without obstruction versus with obstruction?
Dr__Lever: I do not think there is a good study related to this question.
HCMGuy44: I have has HCM for 42 yrs.. Now since I'm 67 I have developed A Fib about 10 yrs ago and my mitral valve is really in bad shape. ....ejection rate around 40% ay last check. I have an appointment with you in February but thought I could get a comment now......... My cardiologist says "something" has to be done soon. Would you comment briefly on whether anything can be fixed? Thanks
Dr__Lever: I will need to see you and take a look. I need to see your echocardiogram and evaluate you. If you want to send an echo before your appointment, I would be happy to take a look.
mark720: does a icd detect a v-tach and stop it
mike83: What causes you to develop an A-fib with this disease? Do HCM patients never develop an a-fib?
Dr__Lever: what causes development of afib is enlargement of the left atrium because of stiffening of the left ventricle and mitral regurgitation. Yes - some HCM patients never develop afib
mymickey: If a MRI did not show scarring but I had a short episode of tachycardia would you implant an IED?
Dr__Lever: ICD - It depends on how fast, how frequent and how long the episode lasted.
Activity, Exercise and Hypertrophic Cardiomyopathy (HCM)
savtaro: My husband and his sister (both in their 70's) have HCM. Most of our grandchildren and all of our children have been genetically tested. Many are positive but clinically negative. Many are athletes (mostly running and Frisbee). Although all have regularly echocardiographs and are seen by specialists (two adult daughters, New York), I worry about the exercising. Can you offer advice as to whether they should be refraining from sprint running and Frisbee? They range in age from children through adults in their upper 40's.
Dr__Lever: The Frisbee is ok and the running is ok but I would avoid weight training.
judyld71: my 16 son was diagnosed with hcm 3 years ago. He recently has had an icd put in. In your opinion do you think it is alright for him to lift weights? Could this cause his heart to be thicker?
Dr__Lever: No - he should not lift weights.
sophiemacie: What is your opinion of a person (asymptomatic) with HCM going to a third world country for missions work where there is obviously no medical facilities within hours?
Dr__Lever: I wouldn't do it.
jillrn: If children had the HCM gene but all echoes are fine...could they still play competitive sports
Dr__Lever: As long as it does not require weight training - but many sports require weight training.
mymickey: I had a myectomy a year ago at Cleveland and you were my great cardiologist. I am doing cardio exercise but can I do weight training up to 10lbs of dumb bells?
Dr__Lever: Up to 10 pounds - yes.
mrimer22: What is reason you can run but not lift weights?
Dr__Lever: Because lifting weights raises the blood pressure much more.
flyannapolis: Hi, Nick Smedira did my Myectomy 3/2004 and I now have an ICD and had a bovine aortic valve replaced by Dr. at Emory, Atlanta 11/2010 I feel great and am walking an hour 4 to 5 days a week and fly-fish by wading in a flowing river once a week. Should I be doing more or less?
Dr__Lever: Keep doing what you are doing.
judyld71: my 16 year old son has never passed out and he has a icd due to slight scarring. you still recommend not to lift weights.
sophiemacie: Why is it important for people with HCM to stay well hydrated?
Dr__Lever: Because if you get dehydrated the size of the left ventricular cavity decreases, that moves the mitral valve closer to the septum which increases your chance for more obstruction.
queen53: WHAT about weights that are done with the legs, lying on a machine and pushing with legs?
Dr__Lever: Any of these exercises will raise the blood pressure. Therefore, NO.
smurf: You warn against lifting weights because it raises blood pressure. Should you avoid lifting something heavy once and putting a strain on the body that single time, or just avoid weight training (as in repetition of lifting weights).
Dr__Lever: It is not lifting - it is the repetitive weight lifting. If you look at someone's biceps muscle, if you do an occasional lift - it does not get big. But - if you do repetitive weight lifting your biceps become larger. We do not want this to occur with your heart.
smurf: and what does raising the blood pressure matter so much?
Dr__Lever: It can potentially make the heart muscle thicken
HCMGuy44: Seems there are many questions relating to lifting weights and HCM. Would this include lifting period.....boxes, and bags of groceries, shoveling snow etc. should be avoided??
Dr__Lever: No - see question above.
CLSTAR: I'm in late stage of heart failure. Do I need to avoid all lifting of heavy items?
Dr__Lever: Don't lift heavy objects and do not shovel snow.
jvaldez: I heard shoveling snow can be very bad for someone with a heart condition (high rate of heart attack when shoveling).
Dr__Lever: I would rather you do not shovel snow.
Genetic Testing and HCM
newtothis: How do you tell your child they have the hcm gene without devastating him/her
Dr__Lever: That is a very complex question. We have a team that works with our patients, including genetic counselors and psychologists. We would be happy to discuss this with you more.
jillrn: even if they had the gene ..is it possible that hcm never develops?
hcmalisa: Does the gene I have indicate how my HCM will progress?
Dr__Lever: No - it does not.
mymickey: Why did the IED not prevent Ben Breedlove from dying? I thought that this was the only measure to prevent cardiac arrest, not the myectomy
Dr__Lever: We do not have enough information. Please go to 4hcma.org for more information.
smurf: Are you familiar with Ben Breedlove's case? If he had an ICD, why did he still die?
Dr__Lever: We don't know the details of this case so we cannot speak to it. This is extraordinarily unusual.
Cleveland_Clinic_Host: There have been a lot of questions about Ben Breedlove, please visit HCMA association site www.4hcm.org for more information.
Research and HCM
smurf: What institution is doing the most research on HCM?
Dr__Lever: The top treating HCM are Cleveland Clinic; Mayo Clinic; Tufts in Boston; Roosevelt St. Lukes in New York; University of Michigan; and the Minneapolis Heart Institute
smurf: I asked who is doing the most research on HCM. you answered who is treating the most. does treating = research? are there any specific research studies going on that you can tell us about?
Dr__Lever: Most of these centers are doing research. We forgot Brigham and Women’s. They are doing a study on one drug to see if the development of thickening of the heart muscle can be stopped. You can always look at www.clinicaltrials.gov to see research studies.
flyannapolis: Thank you, Dr. Lever!
maineobg1939: thanks for a very informative chat
Duncan1: Thank you Dr. Lever.
mymickey: Thank you for saving me. I think I will be ok thanks to you
HCMGuy44: Thank you Doctor Lever. See you in February.
hcmalisa: Thank you it was a great chat.. you can encourage participants to continue talking to each other on the HCMA message board at www.4hcm.org
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