Harry Lever, MD
Medical Director, Hypertrophic Cardiomyopathy Clinic, Department of Cardiovascular Medicine, Cleveland Clinic Miller Family Heart & Vascular Institute
Friday, December 7, 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.
General Questions for HCM
BAnthony: My friend has hypertrophic cardiomyopathy. He is 55 years old and has an ejection fraction of 32%. He had a valve replacement and has a pace-maker-defibrillator. What is the overall prognosis for him?
Dr__Lever: It is hard to say without seeing him. This is a good question for his doctor.
stevenAZ: I am a 52 yo male that was recently diagnosed with HCM. My ability to exercise has gone down the tubes. When I exercise, I feel like I can't get enough air. I can't keep up with my friends and I have to slow down. Is it related to the HCM? Is it the meds I am on? Can I hope for improvement?
Dr__Lever: You need to be seen to see what your echocardiogram looks like.
HCMGuy44: I was there last of March/April for a mitral valve replacement.....I'm the guy from Henrietta, NY. Since then I have felt so much better than I was before.....no more shortness of breath...no more leg swelling, actually feel rather normal. At 68 years old and of course still with HCM and taking all the Meds you all prescribed, What can I expect for the future? In other words what will start to wrong and break next down the road? I would appreciate your candid comments. Thanks!
Dr__Lever: I think you should continue to do just fine.
Barbara_P: My husband has had chronic chest pain for years....The last three it has become more frequent and lasting longer even after going to the ED and staying overnight. He was dx. with LVH then years later HCM. He is on cardizem, norvasc, and ranexa. He has a cpap with 2/L bleed in. They just started him on Neurontin Tuesday. He is becoming very discouraged as I am. He also has a pacemaker. All labs come back good. EKG is unchanged (never looked good, ST depression years ago) Should they be treating him with something else for hypertension for the chronic pain? I really don't know where else to turn. He was seen there in Jan of this year in the HCM dept. He sees group in Washington, PA, We really need your help. Sincerely, Barbara.
Dr__Lever: Please call my office so we can talk about this further. 800-223-2273, ext. 46970.
Diagnostic Testing and Follow Up
charlene5526: I have been newly diagnosed with hypertrophic cardiomyopathy. My uncle and grandfather died what I thought was from a heart attack. I don't know. should I get genetic testing done to see if this is something in my family?
Dr__Lever: If you have hypertrophic cardiomyopathy, you should have a genetic test. But that won't tell you what your father had for sure.
hcmpatient: Did you mean 1 echo per year for an untreated HCM case, or for post surgical patients too? Should it be a stress echo?
Dr__Lever: For all HCM patients, in general, you should have an echo and holter once a year. We are not doing stress echo as part of routine follow-up.
hcmpatient: Do you interpret echo results for of out-of-town patients whose home cardiologist conducted the exam?
Dr__Lever: We do - yes.
varnumgl: What exactly is an echo? Is it the test when they do a sonogram and inject you with something to change your heart beat?
Dr__Lever: An echo is a test that allows us to visualize the heart with use of ultrasound. On occasion we use a contrast agent to visualize the heart a bit better. We do use amyl nitrate to look for provocable outflow tract obstruction which may have an impact on heart rate and blood pressure.
marianneK: My dad has HCM. I had an echo but it was normal. Now, 5 years later, I am starting to feel my heart flutter and maybe a little more tired. Wondering if I should see a cardiologist. Can you have a normal echo and then a few years later show HCM by echo?
Dr__Lever: Yes - I think if you are not feeling well you should see a cardiologist. Certainly you can develop a positive echo for hypertrophic cardiomyopathy a few years later.
Cleveland_Clinic_Host: What type of follow up is recommended for someone with HCM?
Dr__Lever: If someone has HCM, we usually see them once a year with an echocardiogram and a holter monitor.
worriedHCM: what are the screening guidelines for children of a parent who has HCM?
Dr__Lever: If a parent has HCM and is gene test positive, the children should also be gene tested. If the parent is positive and the children are negative, no further screening is required. If the parent has HCM and is gene negative, the children need to be followed with echo until age of 21; every 12 - 18 months. After that, every 5 years.
willy03: My sister has HCM. She had a myectomy but the doctor was not satisfied with the outcome. She then had a defibrillator pacemaker put in. She still does not feel well. She lives in a small town and went to the city for the surgery but not sure that it was the best. Do you think there is more that can be done? She is on medications, still feels poor, short of breath.
Dr__Lever: We would be happy to see your sister. She needs a second opinion.
Fulton1052: Can you explain the surgery to treat hypertrophic cardiomyopathy. My doctor wants me to have a valve replacement and another surgical procedure in my heart to treat it. Does that sound right to you? Is that what is done at Cleveland Clinic?
Dr__Lever: In hypertrophic cardiomyopathy, the surgery may involve removing a part of the muscle called the septum. This is called septal myectomy. Sometimes we need to repair the mitral valve or the papillary muscles that support the mitral valve. Today, we rarely replace the mitral valve. We would be more than happy to see you to look at your echocardiograms. We try at all costs to not replace the mitral valve.
hcmpatient: What is the latest news on the different forms of surgery to correct HCM?
Dr__Lever: We do different types of surgery depending on the anatomy. We can remove a piece of muscle from the septum (septal myectomy); we can repair the mitral valve; we can repair the papillary muscles. We have seen good results for HCM patients in over 200 cases last year at Cleveland Clinic.
Markus: I had a septal myectomy with aortic valve repair at CC in 1999. I have had annual echocardiograms since returning home. Since my return home the Cleveland Clinic has sent me release forms for my medical tests which I released. My question is who reads these and if something significant was seen, would someone call me.
Dr__Lever: Can you please contact the resource nurses so we can look into this further. We need to know more about where your are sending them. - please call 866-289-6911 or email us at firstname.lastname@example.org so we can talk to you.
Cindi-VA: If you have a myectomy and 5 years later your gradients are up again? Do they do another myectomy or is transplant the next step? What are the indications for transplant?
Dr__Lever: If you have outflow tract obstruction again, another myectomy can be considered. We would ordinarily not do a transplant as the next step. You need to be evaluated for the best course of treatment.
hcmpatient: Is ablation now more common than open heart surgery? What about robotics?
Dr__Lever: Alcohol ablation has been done more frequently than surgery but we feel it has been done too often. It should only be done on carefully selected cases. The anatomy has to be just right for an alcohol septal ablation. We are not doing robotic septal myectomy at this time.
Arrhythmias and HCM
SalfromWA: How common is sudden death from HCM in older people. I am 65 years old and have been treated for some time. I started having abnormal beats and my doctor wants to put in an ICD. I am a little nervous about this but will do it if it is necessary.
Dr__Lever: We look for scarring on an MRI scan to help us decide if someone should have an ICD. A little bit of scar makes us worry somewhat less. Long term monitoring with a monitor is also monitor for at least a month to see what the ongoing rhythm disturbances are. We are not putting ICDs in all patients with HCM. I would strongly suggest you be seen in a Hypertrophic Cardiomyopathy Center for further guidance.
William: If one has HCM and atrial fibrillation, does that change the options for treating the atrial fibrillation. For example is the success of ablation less?
Dr__Lever: It is true that the atrial fibrillation ablation procedures are probably less successful in patients with HCM. It also depends on if you have provocable or resting outflow tract obstruction. If obstruction either at rest or provocation is present then surgery to repair the obstruction can be helpful in reducing occurrence of afib and at the same we make some modifications in the left atrium to prevent atrial fibrillation.
kimJM: Are irregular beats common in hypertrophic cardiomyopathy? I have a lot of pvcs. would a pacemaker help?
Dr__Lever: Premature beats are common but a pacemaker by itself would not help. Sometimes they can be treated with beta blockers.
DougH: Hello Dr. Lever, I was wondering about how often verapamil is used in treating HCM. One of my local cardiologists stated that verapamil was not used any longer for HCM. I don't think that is correct and I was interested in your comments about that. Thank you.
Dr__Lever: That is not true. It is still used. It is not my drug of choice. I tend to use beta blockers unless the patient also has asthma.
BrooksK: What are the typical side effects of beta blockers? Are there any "new and improved" beta blockers? (I'm currently on Nadolol).
Dr__Lever: Usually they make you fatigued and on occasion can make you feel dizzy. I frequently use Toprol but it should be the brand rather than generic as I have found the generic form of that drug does not work as well.
BrooksK: What should the target blood pressure and pulse be for one with HCM? What is the typical progression of drugs to control BP if the beta blocker is not effective? What pulse is too low?
Dr__Lever: If there is left ventricular outflow tract (LVOT) obstruction, I don't control the BP as tightly and would be satisfied with 135-140/85-90. If the beta blockers are not working, I use a drug called clonidine or an old drug called aldomet. I try to stay away from drugs called ace inhibitors or drugs called angiotensin receptor blockers as they cause vaso dilatation and can worsen outflow tract obstruction. If there is no LVOT obstruction at rest or provocation, then those drugs (ace inhibitors or drugs called angiotensin receptor blockers) can be cautiously used.
RoseanneS: Is vigorous and sometimes competitive exercise approved for adults and children who are phenotype negative, genotype positive? I refer to running, Frisbee, soccer but not basketball in a family cohort ranging in age from infancy to age 49. Father, grandfather is phenotype and genotype positive.
Dr__Lever: The guidelines say that you can exercise but I would avoid weight training because it is potentially possible that weight lifting could turn on the gene for HCM, though that is not proven.
BrooksK: I'm not sure what caused my HCM, but my echo seems to show a slightly less thick septum and improved mitral valve function since changing blood pressure medications. Are other improvements possible through diet (i.e., does a "heart healthy diet" do anything for HCM)? Can exercise reduce HCM? Best exercise?
Dr__Lever: There is no data that healthy diet has any affect on HCM. Exercise does not reduce HCM. The best type of exercise is walking, swimming, golf if you walk and bowling. Stay on your medications.
frankL2510: I have HCM, diagnosed this past year. I am a very active male, cycle, hike, ocean sports. Can I go scuba diving? What about difficulty with altitude?
Dr__Lever: I would prefer not for scuba diving. If you were going to do it, you should certainly not do it alone. Some people do have more shortness of breath with high altitude. It is a matter of trying it and seeing what happens.
JimmytL1772: I have seen athletes with hypertrophic cardiomyopathy die on court or field. Is it possible that intense sports can cause this? Or is it undiagnosed to begin with. Just curious.
Dr__Lever: We think that vigorous sports are related to sudden death in patients with HCM and that in general, HCM is under-diagnosed. It is not really known for sure that sports can cause HCM.
Hearty1: Are there specific HCM centers?
Dr__Lever: You can be seen here in our Hypertrophic Cardiomyopathy Center; Mayo Clinic; Minneapolis Heart Institute; Roosevelt St. Lukes in NY or Tufts NE Medical Center has an HCM center.
HamBirder: Are there any recommended HCM centers in the Central Gulf Coast area?
Dr__Lever: We know of Mayo Clinic; Minneapolis Heart Institute; Roosevelt St. Lukes in NY or Tufts NE Medical Center has an HCM center. You can contact the HCMA to see if they have a recommendation in your area.
MapleLeaf: Are there any promising research studies for HCM? Are they using stem cells or any medications to stop worsening?
Dr__Lever: Not using stem cells so far for HCM. There are no new studies going on for HCM at this time that I am aware of.
marg: Does amyloidosis cause hypertrophic cardiomyopathy? What is hypertrophic apical variant cardiomyopathy?
Dr__Lever: Amyloid can look like HCM and can even cause outflow tract obstruction - but it is a completely different disease. It has a characteristic appearance on MRI scan as well as myocardial biopsy. Hypertrophic apical variant cardiomyopathy is a form of HCM that causes thickening of the tip or apex of the heart.
lmaier: My father died at 61 with HCM and his father at 51 suddenly. This year after doing a senior yoga class I had chest pains and breathing problems and very weak I am 61 yo female also with MS. My echo in April showed EF30, Over the Summer I took Vitamin D 50,000 IU for 13 wks for my MS and in the Sept my echo EF was 52. I was told I have Non Ischemic Dilated Cardiomyopathy. Can the vitamin make a difference? The only thing I was told is to watch my breathing.
Dr__Lever: I am not aware that vitamin D can make a difference in ejection fraction.
lmaier: Can you clarify the difference between Non Ischemic Dilated Cardiomyopathy and Hypertrophic Cardiomyopathy.
Dr__Lever: HCM is a situation in which the walls of the heart is usually thick and the cavity is small and non ischemic dilated cardiomyopathy, the walls tend to be thin and the cavity is dilated.
chuck: I have been very active; weight lifter my whole life. feeling short of breath and went to the doctor. My doctor said I have a hypertrophic heart. Is that the same as hypertrophic cardiomyopathy? I am now in the process of undergoing more tests.
Dr__Lever: It may be the same but we would need to look at the echo to be certain of what the diagnosis is.
EllenR: My daughter is 18 yo w Dix last year if myocarditis. Her EF was 45 then and doctors say it is now 30. They say she either has myocarditis again or possible cardiomyopathy. Testing is this month. She also has irregular rhythms and is asymptomatic. Her lifestyle has not changed during this FCC and she continues to live an active college life and excels in activities and studies. With an EF if 30 what should we be doing for her in terms of continued lifestyle? She is only on a beta blocker now.
Dr__Lever: She needs to be carefully followed. I would recommend her to see one of our heart failure doctors (they treat cardiomyopathy).
Hearty1: Is it possible to reduce growth of scar tissue with exercise- diagnosed with possible myocarditis or cardiomyopathy and am 18.
Dr__Lever: No - it is not possible with exercise.
Hearty1: Daughter is asymptomatic with possible dx of myocarditis and or HCM- having pet and MRI - EF is 30% what is prognosis for an 18 YO who continues to be active and exercise?
Dr__Lever: You have to know the diagnosis first - is it myocarditis or hypertrophic cardiomyopathy. The second point would be to see a specialist who can follow your daughter closely.
Hearty1: Can EF be reduced due to scar tissue forming or a new inflammation.
Dr__Lever: Yes that is true.
Hearty1: They are not sure if it’s the return of myocarditis or possible HCM- testing this month with PET and MRI but she continues to insist on vigorous exercise as she is asymptomatic. She has also gained significant weight in last 2 weeks, and I wonder if it is fluid related.
Dr__Lever: She really needs to rely on her doctor and if you are not comfortable with the doctor, seek another opinion. Her questions are best answered by her doctor. If she has worsening or additional symptoms, she should notify the doctor. It is most important to confirm what her diagnosis is.
Hearty1: What about siblings? Is myocarditis and or HCM (again not sure of dx yet) something that should be checked in 16 YO sibling?
Dr__Lever: If it is thought to be myocarditis, it is usually not contagious and it is not genetic, I would not check the 16 year old unless there are symptoms - or unless it is HCM.
Hearty1: Is it typical to be asymptomatic with an EF 0f 30%?
Dr__Lever: No; this is not typical but it can occur.
Hearty1: Can EF be related to scar tissue or new inflammation and could it go from a 45 to 30 in a 6 month period?
Dr__Lever: If it is myocarditis it can go to 45 to 30 in 6 months if there is active inflammation.
Hearty1: What about vigorous exercise in gym?
Dr__Lever: With an ejection fraction of 30% they should not be doing vigorous exercise and for sure not weight lifting.
Hearty1: What can I expect an MRI and pet to show in terms of dx?
Dr__Lever: When pulse is too low - I don't like it below 40-45. MRI scan with myocarditis may show scar. Similar on a PET scan.