Milind Desai, MD
Milind Desai, MD

Thursday, July 13, 2017 | Noon


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. Cardiologist and HCM specialist, Milind Desai, MD answers your questions about HCM.

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Types of Hypertrophic Cardiomyopathy (HCM)

shawna: What is the difference between HCM and HOCM? Are they the same thing?

Milind Desai, MD: HCM is the broader disease process. This is where the heart muscle is thick. About 70% of patients with HCM have obstruction to flow of blood. These would be HOCM, hypertrophic obstructive cardiomyopathy.

72male: What is the difference between HCM and AHCM? Is AHCM inheritable? Does it have the same risk of sudden death in young athletes as HCM? What do we know about genetic testing for the transmission of AHCM to my children and grandchildren? What screening for my three grandchildren ages 1, 13 and 15?

Milind Desai, MD: HCM is the broad disease and apical HCM (I assume that is what you mean by AHCM) is one type of HCM where the predominant thickening of the heart muscle is in the apex of the heart not the base. In terms of genetic testing, the recommendations are the same for HCM across the board. Sudden death - there was previous believe that AHCM patients are at lesser risk. However, we do not believe so and our work up and recommendations are similar across all HCM subtypes. Your grandkids should have an EKG and an echo and possibly a genetic counseling evaluation along with a pediatric cardiology assessment, especially the 13 and 15 year old. For the 1 year old, talk to his pediatrician and perhaps when he is around 5 years old - discuss this with him/her.

Causes of HCM

MsFit123: My sister was just diagnosed with this disorder. The doctor is assuming hers was caused by high blood pressure and sleep apnea. Two questions....can sleep apnea be a cause? ALSO, my sister never had high blood pressure until the last few years....could that still be a cause? What is the most common cause? THANK YOU FOR YOUR HELP.

Milind Desai, MD: How old is your sister? The high blood pressure related pattern is mostly seen in older individuals. Sleep apnea as a cause of HCM is not well established, however they do frequently coexist together. The fact that your sister did not have HBP until a few years ago, suggests this is more likely an inherited disorder. Some patients may have a denovo mutation in them and may be the first person in the family to get this condition without any prior family history.

jayveeop: Is there any connection to Agent Orange?

Milind Desai, MD: No proven link. Don't believe everything you read on google. Most common cause of HCM is genetic.

Tahoe: What effect does hypertension have on Hypertrophic Cardiomyopathy?

Milind Desai, MD: It can make the wall thickening worse. It can potentially add to the symptoms; sort of like adding fuel to the fire.

Genetic Testing

barbara76: I have HCM. My mom has HCM and had a myectomy a couple years ago. Should I have my children gene tested? Also - should I get worse and need treatment, how many myectomies do you do each year at Cleveland Clinic? What is a good number when researching places to have this done?

Milind Desai, MD: You should have your children and yourself undergo genetic counseling. And after discussion with genetic counselors, decide if genetic testing is warranted. We do about 200 myectomies a year which is one of the highest on the planet. The important thing when researching places to have a myectomy done is to avoid low volume centers, for example, centers that do 20-25 a year. If my family member needed a slick operation like this I would go to a big center, not a small or intermediate one. I am not sure if there is a definite number I would attribute to but choosing an experienced team to diagnose, treat and do follow up care is important.

davidsmu1: What genetic and or stem cell research is being done to correct or eradicate HCM?

Milind Desai, MD: We just completed a 2700 patient study sponsored by NIH where we have imaged and drawn blood for genetic analysis for this disease. Hopefully in the next 5 - 7 years we will have more insight into the disease process than we already have. There are multiple many new drug discovery programs underway in the pharmaceutical realm and we are hopeful some of these will bear good results. With regard to stem cells, it is a bit premature and hopefully in the future it will lead us to more exciting options.

HCM Symptoms

gd1822: In addition to HCM symptoms of chest tightness and shortness of breath, I also experience a crushing pain down my left arm, from shoulder to wrist, during the episode. The symptom has been unexplained by doctors. What causes this pain, and is it likely to go away following a myectomy?

Milind Desai, MD: It is not uncommon for HCM patients to have the type of pains you describe. The cause of these pains could be as follows: the combination of thick heart muscle and small coronary blood vessels not supplying enough blood supply to the heart muscle; 2) you may have obstructive disease of your big coronary arteries that may be causing these symptoms. Either way, as part of work-up of a myectomy we would look at obstructive coronary disease and do a bypass. For the thick muscle/small vessel disease, there is belief that removing excess muscle improves these symptoms in many patients.

gd1822: My HCM 'attacks' are random, but often occur when I am walking uphill. Why is that? I also have a crushing short-duration pain down my left arm, in addition to the other symptoms. What's that about?

Milind Desai, MD: The symptoms what you call attacks are very likely due to obstruction due to flow of blood which can be dynamic. By that I mean, it can happen in an inconsistent manner under different circumstances such as climbing a hill, or working out on a hot muggy day or bending over, etc. We answered the question on chest pain and arm pain earlier - please refer to that Q and A.

Arrhythmias and HCM

Terryv: Hello, I have midcavity and apical HCM with a 2 cm aneurysm in the apex. My gradient was 60 at rest, 80 provoked. Dr. raised my Verapamil from 360 to 480 mg daily plus anticoagulant. My exercise test was excellent. I had one NSVT of 158 for 9 seconds on 2-day monitor. Three-week monitor showed rare PVCs only. Please share your thoughts. Surgery or ICD? I'm 65 years old, female, and high anxiety! Thank you.

Milind Desai, MD: I would consider, based on what you described, an electrophysiology evaluation for possible ICD. Your exercise capacity does not justify jumping into surgery just yet.

Duncan53: I recently had some short but rapid runs of NSVT as reported by ICD interrogation. The rate was 220-250 bpm at 6-14 beats in separate episodes. I was told these runs of NSVT held "no clinical relevance." What does that comment mean in light of HCM and a significant family history of SCD?

Milind Desai, MD: If the rate was 220 - 250 bpm that is fast and worrisome. If this rate persists for a little longer, you can get shocked. I would discuss this in detail with your cardiologist and potentially adjust your medications and if need be, seek an opinion from an electrophysiologist.

Implantable Defibrillator (ICD) and HCM

Gretchen7: Hi - I have HCM and had the Boston Scientific S-ICD implanted on 12/2. Two questions: are you seeing a lot of inappropriate shocks in the HCM community? We can have an issue with T-wave over sensing. Also: I am having a very difficult time getting access to my S-ICD data and reports. The data is stored at Boston Scientific. Why am I encountering so much resistance and why don't device companies make our cardiac data available to us? There is no Boston Scientific patient portal for me to login and get my data. Can you help us get access to our own data?

Milind Desai, MD: There have been episodes with defective leads in the past resulting in such issues. The other problem may be like you suspect, t-wave over sensing. Big picture, I would talk to an experienced electrophysiologist who can evaluate your ICD and perhaps consider adjusting thresholds and amplitudes so you do not get inappropriate shocks. In terms of getting data, I would have your experienced electrophysiologist fight the fight for you.

HCM Care

Nikkimc: I am from New Zealand, my (late) father and brother had HOCM, my sister has HOCM and had a myectomy. We all have ICDs. My HCM is severe in thickness 3cm and a lot of scar tissue. I like to think our cardiologists are up with the latest but know they don’t deal with many HCM patients. I was diagnosed at 30 and am now 46, reasonably active and slim but for last seven months my symptoms have increased affecting my daily life, increased heart rate with slight movement 120bpm or up to 190 when walking stairs or slight hill, dizzy and faint, tired, etc. Doctors thought I was now obstructed and have completed a new echo and stress test and can't see any obstruction which confused them. I am wondering if they are missing something due to not being experts in this field and wonder how I can go about getting another opinion from you maybe, based on my reports I could supply? My brother died last year at 47 which was partially because they didn't have the knowledge as I don't want to end up the same. Thanks.

Milind Desai, MD: You definitely need to be comprehensively evaluated at an experienced center of excellence, like the Cleveland Clinic. We would be happy to evaluate you to establish the most appropriate plan of action going forward. We have advanced imaging capabilities which would aid in optimal evaluation.

Beachguy: I am an HCM patient at Brigham and Women's in Boston for seven years now. How do I go about getting your doctors to review my medical history and records? Do your doctors review the original test results? Who pays for it? Must I also be present? Thank you.

Milind Desai, MD: We have two options: the best option is you come here, we take a look at records (including your images/films) and may require repeating testing if necessary. The other option is our econsult program which also would incur a charge. My opinion is a face-to-face evaluation is a better option. We would be happy to see you if you decide to come here.

Medical Management of HCM

bella: Are there any new medications that are used to treat HCM that help with the fatigue and symptoms of HCM.

Milind Desai, MD: There are things in pipeline - new drugs being developed - which hope to improve symptoms and fatigue. The most common drugs we use currently are beta blockers; one sub-group of calcium channel blockers and disopyramide.

xdwl: Hello, Doctor, my question is about the new medication for heart failure -- Entresto (Sacubitril/Valsartan). I heard it is good for patients with systolic heart failure. I would like to know whether it is also recommended for heart failure patients with preserved ejection fraction (HFpEF), e.g. HCM? What is the indication for taking this medication for a HCM patient? Thank you very much!

Milind Desai, MD: No - it is not indicated for HCM patients at the present time.

Akatlynn : I had no symptoms until diagnosed with HCM two years ago. Was prescribed metoprolol 100mg per day. Fatigue along with my heart feeling like it's a sore object sometimes started occurring after my first dose of this drug. My BP has always been normal and pulse excellent. Now BP is sometimes low and pulse now typically in 50s or dropping to 45 - 50. Could the meds be causing these symptoms?

Milind Desai, MD: Yes - potentially. Perhaps you should consider lowering the metoprolol dose after discussion with your cardiologist.

Akatlynn : Thank you for responding. You suggested perhaps my cardiologist would consider lowering my metoprolol dosage because my BP and heart rate are sometimes low. When I discussed this with him, his preference is for me to take as much as I can stand. I feel horrible especially during the night. I may come to Cleveland Clinic at some point. Can I call to discuss further?

Milind Desai, MD: I would be happy to discuss. Please call us and set up an appointment we would be happy to see you for a second opinion.

Beachguy: Can diet and exercise reverse the enlargement caused by HCM?

Milind Desai, MD: No - in general the answer is no - however, as one gets older, if the diet and exercise are suboptimal, they can develop high blood pressure that can make things worse.

psloan: What all does aggressive cardiopulmonary rehab entail and how often would this occur?

Milind Desai, MD: This would involve going to a facility that does cardiac rehabilitation where they initially assess you and then prescribe a tailored exercise program for you, usually this entails under a controlled environment, such as a medical facility.

Duncan53: I cannot tolerate beta blockers. What other medications could be used post-myectomy for blood pressure and rate control. Thank you for hosting this webchat!

Milind Desai, MD: Calcium channel blockers are like diltiazem or verapamil would be a decent option. In my experience, diltiazem is well tolerated.

gd1822: The drugs I am taking: metoprolol, Norpace, and losartan, have had minimal effect on my HCM symptoms. If I choose not to have surgery, what is the likely prognosis and disease progression?

Milind Desai, MD: This is a difficult question to answer without knowing your data in detail, especially whether you have obstruction or not. It would require evaluation at an experienced center.


xdwl: Hello, Doctor, I am a patient with HCM, had myectomy in 2012. My recent echo shows no LVOT obstruction, but with mild-moderate (1+-2+) MR and (1+-2+) AR. LA Diameter 4.7 cm; LA Volume 38 ml/M2; LV ID (diastole) 5.4 cm; EF 65. HR 45-55, BP 90/60. NYHA II. I have been doing well. I am on Metoprolol Succinate 71.25mg/day (1 and half tablet), Spirolactone 20mg/day. But I cannot take Enalapril. Even a small dose of Enalapril (2.5mg/day), it can cause my creatine elevation and hyperkalemia to 5.6. I really appreciate your advice, whether Enalapril is critical for a HCM post myectomy? Any other replacement? Thank you very much!

Milind Desai, MD: Enalapril is not critical post myectomy. I believe in the philosophy of "first, do no harm."

Beachguy: How many surgical procedures do you perform to correct HCM? What is success rate? How severe does the condition need to be to recommend surgery? Does anyone ever get better without surgery? I have Sleep Apnea and HCM, as does my sister. How strong is this link between Sleep Apnea and HCM? Does everyone with HCM develop Atrial Fibrillation? I have had two Cardioversions so far {both successful} many are considered too many? How risky is the Cardioversion process?

Milind Desai, MD: We do close to 200 myectomies per year - which is amongst the highest in the world. Success rate: mortality rate is 0% or very close to 0%. Relief of symptoms and obstruction is very close to 100% as well as long lasting symptom relief. Severity and surgery recommendation is more art than science - and you would need to be evaluated comprehensively at an experienced center. There are some medications that can help your symptoms for a while, but very often disease progresses and you may end up needing surgery. Not everyone develops afib - but it is fairly commonly observed in patients with HCM. It is difficult to manage in HCM patients and the success rate of standard procedures to cure afib is lower in HCM patients.

hcmpatient: What is the latest on HCM recurrence after myectomy surgery (approx. 12 years ago)? I recall that part of my removed tissue was retained for a related recurrence study. Any results from that study? Thanks.

Milind Desai, MD: In patients with a well done myectomy, tissue does not grow back. We have ongoing studies with tissue samples which is helping us understand the pathophysiology of this disease. In fact, I published a paper a few years ago, where we used research from tissue such as yours. So thank you.

raymondJ: At what point does one consider a myectomy for HCM. I have decreased my activities to the point where I cannot do many of the sports I enjoy doing. After a myectomy is it possible to go back to sports such as tennis or pick-up basketball that i used to do - even last year. At this point, I cannot do any of these and it is very depressing.

Milind Desai, MD: You may not be far from myectomy. However, before definitely recommend that I would do a comprehensive evaluation at an experienced center. In terms of sporting activities you would be prohibited from doing these, assuming you have advanced HCM with symptoms. After myectomy, majority of the patients feel much improved and can go back to doing a lot of sporting activities. However, I caution you that they must be individually tailored after recovery - if you have aspirations of taking over LeBron James' lucrative career, I am afraid we may disappoint you.

Tahoe: Is there a database or resource that lists the number of procedures a hospital does per year?

Milind Desai, MD: Ideally should be on hospital website. Cleveland Clinic was the pioneer in reporting the number of the procedures we do as well as outcomes in a very transparent manner. A lot of institutions are following our lead.

Gretchen7: What are your thoughts on robotic-assisted myectomy surgery? Any post-surgical studies available? Also - what is your opinion on apical myectomy surgery? I am apically "aneurysmal", but I haven't remodeled into an official apical aneurysm yet. Also - I'm watching my LA diameter grow with each yearly echo. At what point do you get concerned?

Milind Desai, MD: We do not do robotic myectomy as we believe in doing a complete job in doing a complete job in outflow tract obstruction relief. Robotic inherently makes this not impossible but definitely more challenging. Apical myectomy surgery is an option in selected symptomatic patients with promising data. LA diameter increase reflects worsening diastolic dysfunction or stiffening of the heart which is part of natural progression of HCM. I am not sure if there is a cut off as to when I would get concerned. The concern arises with progression of symptoms and decrease in functional capacity.

Beachguy: With respect to the myectomy, which I think I am facing someday (I am 70 now), how is it possible to stitch up a heart muscle and have the stitch hold once the heart is restarted? Seems almost impossible??

Milind Desai, MD: We do more than 4000 heart surgeries per year at Cleveland Clinic and have somehow managed to figure out your exact concerns with very good results. You will have to give us the benefit of the doubt on that one-----

Beachguy: Thank you.

Milind Desai, MD: You are welcome!

Alcohol Ablation for HCM

Rrieger1: I had an ASA procedure Dec. 7 of 2016, while Doctor who performed the procedure insists it was a success, I have felt more fatigued since having the procedure...what might have happened, or not?

Milind Desai, MD: Unfortunately this is not unusual after a septal ablation by alcohol. In fact I have a couple of patients this week that have the same situation who are going to heart surgery in the very near future. I would get yourself evaluated at an experienced center of excellence such as Cleveland Clinic to make sure you do not have residual obstruction.

Rrieger1: Are there any other procedures that might help me feel less fatigued (post ASA)?

Milind Desai, MD: First we need to make sure you do not have residual obstruction - then we can decide further. If you do not have residual obstruction, aggressive cardiopulmonary rehabilitation may need to be considered.

BryanfromVT: I read recently that alcohol ablation is a good option for young people with HCM. What are your thoughts? I am 26 with HCM. Do not really want to go through surgery if I don't have to - your thoughts?

Milind Desai, MD: If you carefully read the guidelines related to HCM, it is recommended that younger folks like you should not have alcohol ablation over myectomy. There are plenty of reasons for that. One of which is myectomy provides a long lasting relief from symptoms and does a more complete obstruction relief, in fact I had two patients I saw earlier in the week (younger than 40) both with failed ablations, needing surgery in the very near future. If a patient with a failed alcohol ablation, needing a myectomy, it is very likely that he/she will be pacemaker dependent.

davidsmu1: I had an ASA (successful) and an ICD nine years ago. My blood pressure is random. It can be 108/60 or 140/86 on any given day. I do take 5 mg bystolic.

Milind Desai, MD: I think this sounds ok. Even my blood pressure fluctuates depending on my wife's mood.

Left Ventricular Hypertrophy

sinaihospital: I have Left Ventricular Hypertrophy since 2004. I see a heart doctor for this. What tests and treatments are for this problem doctors? Thank you.

Milind Desai, MD: There are many causes for LVH - most common is long standing high blood pressure. HCM could potentially be a cause. I would discuss with your local cardiologist about your specific situation and if you feel compelled, ask him whether he has ruled out HCM.

Reviewed: 07/17

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