Kenneth Zahka, MD
Kenneth Zahka, MD

Harry Lever, MD
Harry Lever, MD

Friday, August 5, 2016 - 12 Noon

Description

Hypertrophic cardiomyopathy (HCM) is a complex type of heart disease that causes thickening of the heart muscle (especially the ventricles, or lower heart chambers), left ventricular stiffness, mitral valve and cellular changes. Take this opportunity to learn more and have your questions answered by HCM cardiologist Harry Lever, MD and pediatric cardiologist Kenneth Zahka, MD.

More Information


Symptoms

Eugene: I have been diagnosed with idiopathic hypertrophic subaortic stenosis, bradycardia, and hypertensive heart disease without CHF. For over six years, I have been having attacks with dull but high level pain in my left arm, tightness of the chest, and, often, shortness of breath. There is no trigger event for these attacks, which are short duration, and seem to occur randomly. What is a typical treatment plan for these conditions? I am 68, male, 5'11, 190 pounds, non-smoker, moderate alcohol.

Harry_Lever,_MD_: You need to be thoroughly evaluated with a stress echo, MRI and holter monitor. From there a treatment plan would be developed.


Diagnostic Testing and Follow up

AnnaB: I am scheduled for a stress echo in a couple weeks to determine need for surgery for my HCM. What are they looking for?

Harry_Lever,_MD_: We want to see the blood pressure response, heart rate response and if the patient develops an outflow tract gradient (how much obstruction to the blood flow out of the heart they develop).

sinaihospital: I have left ventricular hypertrophy,  what tests do I need for this problem? Doctors I see a cardiologist for this problem. What treatments are for this problem? Doctors, thank you for your advice.

Harry_Lever,_MD_: We first need to find out how severe the HCM is - we start with an echocardiogram and then start from there. You should see a cardiologist for this condition. Treatments would be based on what we see and include medications and surgery when needed.

egglet: If you have HCM - what is the normal follow up with a doctor? What tests should be done during that visit?

Harry_Lever,_MD_: We usually do an echocardiogram and a 24 hour holter monitor once a year.

mariearc2015: My cat scan notes cardiomegaly, my echo notes basal septal hypertrophy, left ventricular diastolic dysfunction grade 1 and dilated aortic root.  Is this the same as an enlarged heart? Thank you for your answer.

Harry_Lever,_MD_: It can be. But - we would need to look at the CT scan and Echo images to tell you for sure.

gd1822: For the past six years I have had multiple diagnostic tests, including echocardiograms, treadmill stress tests, EKGs, and various tests to rule out esophagus and digestive diseases. Lots of time has been wasted pursuing those factors, based on the symptoms of tightness in chest, pain (dull but severe) in left arm and some shortness of breath. I have been diagnosed recently with idiopathic hypertrophic subaortic stenosis, bradycardia, and hypertensive heart disease without CHF. I have sleep apnea. I use a CPAP machine and am taking metoprolol tart 25 mg 2x daily, ranexa 500mg 2x daily, and verapamil 120mg 1x daily. Relief has been moderate, but the attacks still occur, randomly and without a common cause, lasting 10 seconds to several minutes. Thoughts?

Harry_Lever,_MD_: You need a second opinion that would involve reviewing your medical history and test results (including films). We would be happy to see you here or we have an online second opinion service.

gd1822: How do I access the 2nd opinion service?

Harry_Lever,_MD_: MyConsult is the online second opinion service - see my.clevelandclinic.org/online-services/myconsult.


Medications

xdwl: Hi, Dr. Lever. I am a 58-year-old female, HCM post-myectomy in Sep. 2012. I saw you in CCF in 2013. You put me on Metoprolol Succinate 71.25mg/day. I have been doing well. My recent echo: mild-moderate (1+-2+) MR and (1+-2+) AR. LA ID 4.7 cm; LA Volume 38 ml/M2; LV ID (diastole) 5.4 cm; EF 65. No LVOT obstruction. HR 45-55, BP 90/60. NYHA II. I have questions on my treatment and appreciate your advice:
1) Local doctor recommended me to add Enalapril to further protect my heart function. Would you think Enalapril is beneficial for a HCM patient with heart failure (NYHA II)?
2) I was on Spirolactone 20mg/day for a few years. My recent blood potassium 5.5mmol/L (normal limit 5.3mmol/L), Cr 83 umol/L(normal limit 85 umol/L). So I have to stop taking it. Would you advise I should try to resume Spirolactone after my blood potassium drops to normal?
3) What is your comments on the new medication Entresto (Sacubitril and Valsartan) for HCM with heart failure?  Thank you very much!

Harry_Lever,_MD_: Enalapril can make people with HCM lightheaded. I am not fond of this drug.
2) Would strongly advise further evaluation prior to starting on spirolactone.
3) I would have to evaluate your echos to make a determination on whether to use that.

Perhaps another visit would be helpful to evaluate you and answer your questions.

GretchenR: Hi - what medications are available to cure cardiac fibrosis? Do you offer stem cell therapy to reduce cardiac scars? Do pulmonary anti-fibrotics have any effect on cardiac fibrosis (prifedidone or nintedanib)?

Harry_Lever,_MD_: Unfortunately there is nothing we can do to cure cardiac fibrosis. We do not offer stem cell therapy here to reduce cardiac scar. The drugs you mention are for pulmonary fibrosis.

GretchenR: Are there any medications available to shrink cardiac cells?

Harry_Lever,_MD_: Not that we know of.

GretchenR: What anti-depression medications can I take in conjunction with Metropolol?

Harry_Lever,_MD_: It is best to discuss this with your prescribing physician.


Diet: Food, Alcohol

nhlfan: Post septal myectomy (one year+), what are the guidelines for alcohol consumption? I still take a daily beta blocker.

Harry_Lever,_MD_: You can take the beta blocker. I tell my patients not to drink alcohol.

kansas77: Are there any diet restrictions for people with HCM - foods you should avoid? Or for that matter, foods that we should definitely eat to be healthier? What about alcohol?

Harry_Lever,_MD_: I really do not like alcohol. There is an increased incidence of heart rhythm disturbances particularly atrial fibrillation. There is no particular foods to avoid. Usually, unless there is heart failure, I do not restrict salt. You do need to know to drink lots of fluids in very hot weather. I have told some people to increase salt within reason in very hot weather to retain fluids - unless their BNP is very high.


Screening, Genetics and HCM

Sondra: when should you consider genetic testing in HCM?

Kenneth_Zahka,_MD: This is an excellent question, and one which largely depends on the individual details of the patient. We find that a positive genetic test is helpful in confirming the diagnosis in a patient with early signs of HCM. Occasionally, the type of mutation is also helpful in predicting the future course of the disease. A negative test unfortunately does not exclude HCM. For many families, the most important use of genetic testing to help screen family members for the gene before it becomes evident on the echocardiogram or MRI. It is important that genetic testing be coordinated by the cardiovascular genetics team. The interpretation of the results requires a detailed knowledge of both HCM and genetics.

Barbara22: At what age should screening/testing begin in children of a parent with HCM?

Kenneth_Zahka,_MD: Excellent question. The guidelines suggest that late childhood is the usual time. I will screen children earlier for symptoms, for parental concerns or if the family history suggests an early onset of disease.

nhlfan: Can HCM be acquired or is it always a genetic condition?

Kenneth_Zahka,_MD: Most HCM in the young is genetic, either inherited from one or both parents or as a new mutation. In the elderly, HCM can be a reaction to long-standing high blood pressure and thus be "acquired". This is not likely passed onto the offspring. We occasionally see young people who have excessive thickening related to significant weight training.

GJL56: Can obesity in a young child "activate" the HCM genes to cause the thickening in the heart or rather is the thickening likely to occur in any case (presenting vs. recessive)?

Kenneth_Zahka,_MD: Obesity does not seem to be more common in our pediatric HCM population at the time of diagnosis. We have seen obesity become a problem in the teenagers who have become more sedentary due to activity restrictions. We continue to try to find the right balance to ensure that the teenagers stay active but do not push them too far.


HCM and Congenital Heart Conditions

Logansmom: My son is seven and has the official HCM diagnosis. Has also has a VSD, PFO and PDA. He was diagnosed with Wolff Parkinson White syndrome at three months of age. He was on digoxin for over two years but is not on any medication right now. We have been seeing a cardiologist since I was pregnant with him. Any info you could offer would be appreciated and comforting...If my son has the HCM diagnosis at age seven, should I be getting some type of testing? My father has heart issues and a pacemaker since he was 49.

Kenneth_Zahka,_MD: Further details would be very important. WPW may be a separate issue or can be seen in HCM especially in some of the storage diseases where the muscle is thickened due to storage of substances within the heart muscle rather than abnormal muscle cells themselves. We also occasionally see congenital defects in children with HCM. We'd be happy to see him here at Cleveland Clinic for a second opinion, this process you can start online or you can call for an appointment at 216.445.5015. See the answer above for the MyConsult link.


HCM and Heart Failure

Hmarsh: Please explain what a patient should do once HCM results in congestive heart failure. What is the prognosis once the CHF is diagnosed and what can be done to prolong lifespan and the quality of life in such a patient (age 72). My myectomy was performed in 2004 at Cleveland Clinic by Dr. Smedira.

Harry_Lever,_MD_: I would highly suggest a re-evaluation in our clinic prior to providing you advice and a treatment plan.


HCM and Arrhythmia

Katherineall: If you have hypertrophic non-obstructive card and chronic a-fib at 73, should I have an ablation or wait until shortness of breath becomes major issue. I feel good, just tired and only take Toprol?

Harry_Lever,_MD_: It depends on how well the heart rate is controlled. If you feel well - I would watch. If you are having shortness of breath, I would think about being evaluated for an ablation.

Logansmom: Are there any similarities between Wolff Parkinson White syndrome and Hypertrophic Cardiomyopathy?

Harry_Lever,_MD_: You can have WPW without HCM - it is basically a rhythm disturbance and you can have HCM with WPW. It can occur separately or together.


ICD and HCM

CharleneK: If I have an ICD and now will be going into surgery for myectomy next month - will I still need the ICD? Do they ever remove it?

Harry_Lever,_MD_: Usually they do not remove the ICD once it is implanted. It depends on the reason the ICD was implanted - if you will need it in the future.

Adnerb: Hello. Does the thickness of the septum determine when an ICD should be considered and if so, what thickness is considered to be appropriate for ICD for HCM. Thank you.

Harry_Lever,_MD_: Yes - when the septal thickness is above 30 mm, we become concerned that the patient needs an ICD. But we often do an MRI scan to see how much scarring they have and it is the amount of scarring that helps determines the need for ICD. We also do cardiac monitoring to determine the heart rhythm.

GretchenR: Regarding cardiac scarring and need for ICD: do you look at the placement/location of cardiac fibrosis when determining ICD recommendation? Or are recommendations made only on total percentages?

Harry_Lever,_MD_: It is usually made on total percentage.

Adnerb: My son died of HCM (age 41) six years ago. An MRI shows that I have HCM and genetic testing picked up the mutation. Do these facts increase my need for an ICD?

Harry_Lever,_MD_: This history definitely would lead to an evaluation of an ICD - but it does not mean necessarily that you will require an ICD. There are many scenarios and factors that go into this determination. Testing and evaluation will lead to a decision.

donna: What are the indications to have an ICD placed if you have HCM?

Harry_Lever,_MD_: Indications are: proven cardiac arrest, non-sustained VT with HR greater than 160-170 bpm with more than 10 VT beats in a run, a thick heart with greater than 15% scar coupled with the rhythm, syncope that required resuscitation, and sustained VT on a holter monitor.


Myectomy

Robert: What is the general healing times for after myectomy? What can I expect to feel like after?

Harry_Lever,_MD_: It takes about six weeks until the pain is better from the incision, and healing of the incision. But the heart performance improves quickly and patients tend to feel better in about a week.

vincentl: If you have a septal myectomy - can the thickness grow back?

Harry_Lever,_MD_: Usually it does not grow back. Unless - you are a young child. Not in adults.

Nick: What determines if a patient is a candidate for myectomy vs. ablation for HCM?

Harry_Lever,_MD_: We tend to do alcohol septal ablation for patients who are not candidates for surgical myectomy. The outcomes for patients who have surgical myectomy are generally better than those who have had alcohol ablation. In order to do an alcohol septal ablation, the anatomy has to be perfect. That is the artery that we will ablate has to supply the area of the heart where there is outflow obstruction and it cannot be too big or it will cause too large a heart attack; or too small and it won’t do anything. If the alcohol ablation does not work, there is a significant risk that if the patient needs to go on to surgery, they will need a permanent pacemaker. Why? The heart has two electrical bundles - the right and left bundle. The right bundle is damaged with alcohol ablation and the left bundle is damaged with surgery. So if both bundles are damaged there is likelihood that you will need a pacemaker.

mamabear: my 6-month-old granddaughter has obstructive hypertrophic cardiomyopathy. She does not seem to have symptoms but her doctors want to do a myectomy on her. Is that too young? Would that be the case or should we get a second opinion? I am so worried?

Kenneth_Zahka,_MD: You should get a second opinion. Even with our experience with myectomy, we virtually always wait for symptoms. Obviously symptoms can be difficult to define. In this setting, it is also particularly important that surgery be done at a HCM center.


Transplant

breakinthemix: What percentage of HOCM patients will eventually require transplant? I have been advised that due to the progression of my condition over the last 10 years, transplant is a real possibility in the future.

Harry_Lever,_MD_: It is less than 5 percent of HCM patients that require a transplant.


Exercise and HCM

Claudia: What kind of exercise would be safe for a child with HCM? My son has an ICD and would like to participate in some type of sports.  Dr. Zahka - what are your thoughts on sports participation? Who can evaluate him to tell us/school what is safe.

Kenneth_Zahka,_MD: We generally recommend aerobic/cardio type activity initially guided by heart rate monitoring with the maximum heart rate selected from the results of exercise testing. An ICD does not necessarily prevent this type of exercise, but the details of why the ICD was placed would be essential for knowing what type of exercise was appropriate.

nhlfan: What is the maximum heart rate in exercise for a 64-year-old male, post myectomy?

Harry_Lever,_MD_: Usually the maximum heart rate for a 64-year-old male post myectomy is 110 - 120 bpm.

Rocco: I had a myectomy six months ago. I am back to doing activities and mostly running or on an elliptical. I am wondering what the restrictions are at this point on weights. Can I use bands? Is there still a weight restriction for lifting now that I had the myectomy? If so - does it matter if you are lifting with your legs vs. your arms?

Harry_Lever,_MD_: No heavy weights - do not go above 20 - 30 pounds. Same with legs vs. arms. I am not sure about bands.

Kenneth_Zahka,_MD: I tell patients that you can do, in general, 3 sets of 15 reps without any breath holding. This generally keeps the blood pressure at an appropriate level - and - allows the individual person to adjust the effort based on their own bodies. Checking blood pressure after or during exercise would be a good indicator as well. Holding breath or straining during exercise is not advised.


Athletic Screening and HCM

Jbennett: Since high blood pressure can contribute and this condition is common in athletes and people under 30 that can cause death, are there any screening athletes go through to detect this early on?

Kenneth_Zahka,_MD: The screening of athletes remains a controversial and very important topic. Routine screening based on family history and heart examination is universally accepted. The addition of an electrocardiogram may have some further value. We do not generally favor "screening" echocardiograms in the absence of other findings. If an athlete has symptoms, a concerning family history, an abnormal exam or ECG we recommend a formal comprehensive HCM assessment with a complete echocardiogram.


Other HCM Questions

pauleis: Any treatments to reverse HOCM?

Harry_Lever,_MD_: We do not know of any treatments to reverse Hypertrophic Cardiomyopathy.

chuckfrompa:  Any new research into treatments for HCM that we should know about?

Harry_Lever,_MD_: We are currently involved in a study that is looking at a new approach to medication management to prevent further thickening or scarring. This is in the very early stages and should be approached with a clinical team involved in this study.


Apical Variant Hypertrophic Cardiomyopathy

wihistory: I have apical variant hypertrophic cardiomyopathy. I also have a pacemaker for my atrial fibrillation. I have severe problems with edema and shortness of breath. I currently take 240mg of lasix a day and 25mg of eplerenone a day. They recently took me off of hydralazine and put me on verapamil which has given me more side effects. Can you suggest any other medications or procedures that might help my heart? I am basically home bound at this time. Thank you.

Harry_Lever,_MD_: We truly need to see you as apical variant hypertrophic cardiomyopathy is a complex condition. Treatment would be specific to you.


Other Heart Questions

Nausheen: Hello sir. My name is Nausheen. I need your help sir, please help me. Sir, my cousin was having heart attack last night and today he is admitted in the hospital, but the doctor said that one of the veins of his heart is closed and he is not in the condition for operation. Blood also came out from his mouth. Sir, please help me so that I'll help them. He is in critical condition.

Harry_Lever,_MD_: I am sorry to hear about your cousin. We would need to review the studies that have been done. We do have evaluations that can be done through the internet.

This information is provided by Cleveland Clinic as a convenience service only and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. Please remember that this information, in the absence of a visit with a health care professional, must be considered as an educational service only and is not designed to replace a physician's independent judgment about the appropriateness or risks of a procedure for a given patient. The views and opinions expressed by an individual in this forum are not necessarily the views of the Cleveland Clinic institution or other Cleveland Clinic physicians.

Reviewed: 08/16