Tuesday, July 23, 2013 - 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. HCM specialists Harry Lever, MD, Milind Desai, MD and genetic counselor, Christina Rigelsky MS, CGC from Cardiovascular Genetics, answer your HCM questions including diagnosis, genetic factors and treatment options.
General Hypertrophic Cardiomyopathy Questions
saba: I have tested negative for any genetic marker for HCM and have a history of hypertension-could my asymmetric HCM (which is latent/Provocable and obstructive) have been worsened by long term hypertension? Also could any other etiologies for the hypertrophied heart wall exist?
I have found it very difficult to obtain term life insurance after declaring my HCM. However, I have been told that most people whose heart wall(s) hypertrophy in mid life have a normal life expectancy...is there anyway to obtain term life insurance with HCM ?
I was forced to retire from a career in law enforcement due to a Provocable gradient/HOCM. Is it possible to stay in law enforcement/military service with a diagnosis of HOCM?
Harry_Lever,_MD: You can not get life insurance with HCM. It depends on your job description and your level of disability if you are in law enforcement.
KJM: Can HCM be caused by something other then genetics? I have no genetic markers and no family history.
Milind_Desai,_MD: HCM like appearance (such as obstruction to flow of blood) can be caused by long standing HTN and is frequently observed in older females. Having said that , we do not understand the full spectrum of genetic transmission of this disease and you can still have HCM and we have not yet recognized this disease. In about 5% of this population there could be a masquerader such as Fabrys disease.
Norajab: My son who is 15 now was diagnosed with HCM at 7 years old after he fainted . He had Icd . 2 months ago his doctor diagnosed him with restrictive HCM and she wants to put him on the list for heart transplant although he still in early stages of the disease. Can he be managed by medicine for restrictive HCM? He is on verapamil and metropolol since he was 7.
Harry_Lever,_MD: Yes. You can begin with medical management.
Milind_Desai,_MD: We always have to rule out masqueraders however the testing is still in evolution, hence we will have many false negatives.
fran49: My question really was do I first need a HCM cardiologist to assess my condition and recommend treatment for me rather than assume my CHF cardiologist is on board as much as your department is. I don't want some one to say a year or two from now if things should get worse that I should have been seen by your department. I guess what I'm saying is that I'm scared.
Harry_Lever,_MD: Then come and be seen - we are happy to evaluate you here.
saba: Can hypertension make HCM worse?
Symptoms of Hypertrophic Cardiomyopathy (HCM)
collers8: Can non-obstructive HCM progress to obstructive HCM? If so, would gradual exercise intolerance, and increased chest pain with exertion be symptoms of this remodeling?
Milind_Desai,_MD: Could be. You would need to be re-imaged with dedicated echo to provoke obstruction.
collers8: I am a 53 year old woman with non-obstructed HCM, with septal ventricle wall thickening of 25 mm, moderate mitral regurgitation and an enlarged left atrium, >42 ml/m2. Had an ICD, defibrillator and pacemaker, set at 60 BPM, implanted three years ago. Chest pain is often a daily occurrence. One can't be running to the ER all the time, but I am concerned that I may be causing more damage to my heart by not going. Also, I am counting on my defibrillator to protect me if things really get ugly. My question is, at which point is it advisable to seek medical assistance for this pain?.
Harry_Lever,_MD: We need to know what medications you are taking and some generic medications do not work the same as brand type medications. There have been times when we change the manufacturer, the patient feels better. We would need for you to let us know the exact medications and brand.
Milind_Desai,_MD: IF you are on the right brand, we may need to titrate your medications as long as your blood pressure can tolerate it. in terms of our understanding about further damage to your heart, we do not have enough information. We would be happy to see you.
46970560: I have dizziness often in the evening when I first stand up. I have to stand still and it goes away in 30 seconds or so. Is that the dizziness that concerns you?
Harry_Lever,_MD: The kind that worries me the most is the kind that occurs when you exercise. Both should be evaluated.
skindr: History of obstructive Hocm . One chief complaint is the inability to stoop down or squat for longer than about 20 secs. What do you suspect is the the etiology. Any solution ?
Harry_Lever,_MD: Obstruction - we need to evaluate you to see what is going on.
hrchief: My cardiologist is scheduling a cardiac MRI for me. I have many allergies, particularly to ragwood. I have seen many reports regarding reactions to the gadolinium and there is literature showing that premedication for this may not be effective. Is there any way for an allergist to pretest for reactions to gadolinium? Thank you.
Milind_Desai,_MD: In general, gadolinium is very safe and we do not have many cases. I would proceed with this test if your kidney function is good.
skindr: Is there any way to improve ejection fraction numbers. Pt with HOCM, had myomectomy previously . Number recently declined from 40 to 35.
Milind_Desai,_MD: If the echo was used for measurements, it is within error of measurement. If there is continued clinical concern, then a more accurate modality to measure EF should be considered such as cardiac MRI.
REW: Aside from echo cardiogram, what if any other imaging should be considered (a teen-age patient, genotype positive / phenotype negative but cardiac anomalies) to assess risk and minimize restrictions on physical activities.
collers8: I have two adult children, 21 and 25. There were tested, via echo, for HCM three years ago and were fine. My HCM was diagnosed a age 50, therefore adult on-set. How often should my children have echos done?
Milind_Desai,_MD: Every 3 - 5 years is the recommendation at an experienced center.
cmack216: What do think about echo AOR diam 2d 4.1 left atrium 4.6 left ventricle IVSD 1.81. LVIDB 2D 5.14 LVID 2D 3.38 LVPWD 2D 1.18 SHOULD I have more test done my cardio said it is a mild condition.
Harry_Lever,_MD: We need more information - would be happy to see you here.
Duncan1: Is it common for ejection fractions to drop to the less than 50 percent range after myectomy surgery? Thank you Drs. for hosting this discussion.
Milind_Desai,_MD: If you have concomitant mitral valve regurgitation it could drop a little bit before it gets better.
Genetic Counseling for HCM
skindr: Hocm suddenly presenting in a 65y/o male following Aortic aneurysm repair along with replacement of Aortic valve . Would this late presentation make genetic etiology less likely?
Harry_Lever,_MD: Theoretically yes. Most likely your heart has remodeled resulting in HCM like physiology along with HTN that you most likely have. Having said that one cannot rule out genetic mutation.
Christine Rigelski - genetic counselor: There are rare cases of a single genetic cause causing both aortic disease and HCM.
Exdsm: My daughter was diagnosed at age 9 with RCM/HCM and was found to have one of the rare HCM mutations. My son was checked for the same mutation and was found not to have it. He was told he was fine and need not be monitored. Is that correct?
Christine Rigelski - genetic counselor: I would make sure the result was reviewed by a genetics professional to make sure it was a disease causing mutation. If it is truly a disease causing mutation, then your son who does not have the mutation is not at risk.
Stafer: If genetic testing shows negative, Dr Desai says that imaging is still necessary, then, why do the genetic testing? Just do the imaging.
Harry_Lever,_MD: You don't know before you test, but if the index test is positive, then anyone who is NEGATIVE, do not have to worry about getting HCM.
fran49: I was diagnosed with HCM in late 2006 during a heart cath. and biopsy looking for heart reasons for my SOB(shortness of breath). They found HCM which was unexpected and other heart issues. Within a month I was lucky to survive SCA and am now on my 2nd ICD (CRT-D). I now only see a CHF and EP Cardiologists at the CC.I have heard HCM can be genetic so I am also concerned for my family also. My question is would it benefit me to see a HCM specialist since no one really mentions that I have it or what they may be doing for me other than a yearly echo? Thank You.
Harry_Lever,_MD: Family screening is important and perhaps consider genetic testing.
Christine Rigelski - genetic counselor: We can talk to your cardiologist about genetic counseling.
saba: If a mother and son both have HCM does it have to be the same genetic version.
Christine Rigelski - genetic counselor: In most cases, it is caused by the same genetic mutation. In rare instances a younger or more effected family member may have an additional genetic cause. It is important to test the youngest most affected member of the family.
cattie1: I took genetic testing which confirmed that I have HCM; my mother was later tested and results showed that she has the same genes as I do. Therefore it was concluded that my mom passed the gene on to me. Is it accurate to say that my mom has hypertrophic cardiomyopathy? she suffers from afib, and heart palpitation.
Christine Rigelski - genetic counselor: Does she have evidence for hypertrophy? She is at risk but with no evidence on imaging you do not have HCM.
Harry_Lever,_MD: Afib could be related to HCM - she needs a thorough evaluation.
sjjohnston: What role does genetics play in HCM? We were told it is a genetic disease when our son was diagnosed 9 years ago. We were all tested and searched the family's medical history with all coming back negative. We were then told it was probably a "spontaneous gene mutation". Is this something that happened in utero?
Christine Rigelski - genetic counselor: New mutations can occur at conception but due to variability in families with HCM, first degree family members need ongoing cardiovascular screening. Since it has been 9 years, additional genetic testing can be considered to determine if it is truly new to him.
Milind_Desai,_MD: It is however important to know that up to 40% of people can have a negative genetic test yet be at risk for HCM. So - if everybody is genetically negative, family members do need periodic imaging testing, including the son.
saba: The 40% what etiologies...all HCM or something else.
Stafer: Why do genetic testing? Why not have your family checked out for HCM is echo cardiogram if they have it?
Christine Rigelski - genetic counselor: It is a reasonable option to consider cardiology screening rather than genetic testing but if genetic testing is positive (a gene is identified) family members who test negative do not need further screening.
sjjohnston: Thank you Dr Desai. Another question, when my son was diagnosed 9 years ago, we were told genetic testing wasn't very good. Has it improved greatly? Does insurance cover testing?
Harry_Lever,_MD: Yes - it has improved greatly. A lot of insurance does cover it - you need to check with your insurance company and discuss with a genetic counselor.
Exercise and HCM
sjjohnston: My son was diagnosed with HCM when he was 16 by Dr Barry Maron. He is now 25 and has gained a great deal of weight since he stopped conditioning for football 9 years ago. He walks on a treadmill occasionally, but needs more direction on what sort of activities in which he is able to participate. I have researched a bit on line, but cannot find any clear cut information. Is this a discussion he needs to have with his physician or are there any general guidelines for HCM patients?
Milind_Desai,_MD: First of all - every patient is different and we would need to understand him more to give him recommendations. In general, light to moderate aerobic activity is ok. Avoid lifting beyond 20 - 30 lbs. Avoid intense isometric exertion such as sudden weight lifting. Avoid competitive sports.
MaryBK: My son is 16 and was just diagnosed with HCM. He is very fit, has played baseball since he was little and was trying out for high school varsity. Diagnosed during a physical and then echo. Right now they are saying he is not allowed to play. This has devastated him. Is there any chance that kids can play sports with this diagnosis? I do not want to lose him and do not want to put him at risk but understand how sad, angry and depressed he is. What should I do?
Milind_Desai,_MD: This is very difficult but current recommendations are no competitive sports with this diagnosis and we should respect that. We do not want a bad outcome as difficult as this is for him.
Atlas217: What is the current opinion of the HCM patient engaging in resistant weight training?
Harry_Lever,_MD: NO WEIGHT TRAINING.
cmack216: Hello staff. My name is cmack216. After a echo I was diagnosed with HCM nonobstructive what about diet and exercise, is walking and running good.
Milind_Desai,_MD: Sure - diet and exercise are always good - maintain an ideal weight is optimal. Under most circumstances there are no limitations to moderate aerobic activity. Avoid weight lifting, competitive sports and intense isometric exertion.
collers8: You say a restriction of 20-30 lbs for weights for HCM pts. Is that only for hand weights? For my legs I like to use universal machines and vary between 50lbs, for calf curls, to 170 lbs to leg press. Is this okay? Also, is it okay to use the bench press for free weights, use 12 lbs per arm? Exercise is my biggest challenge with HCM. Still try to jog, but starting to have chest pain during it.
Milind_Desai,_MD: 20 - 30 pounds - legs, arms, whatever. If you are having chest pain, stop during chest pain. Looks like you need to be evaluated further.
mbertels94: What level of exercise would be recommended for a young HCM patient (19) who has had a recent myectomy with fantastic results? I would like to be able to run again and do everything a normal 19 year old would be able to do without over doing myself. My local doctors and the doctors at Cleveland Clinic recommended I do not participate in a cardiac rehab program because I am doing so well it would not benefit me. I have been cleared to do "everything short of an iron man." Where is a safe place to start?
Milind_Desai,_MD: One advantage of rehab as it does give you an understanding of what you can do. We would recommend rehab or at a visit with our exercise physiologists to provide you with guidelines.
Treatment of Hypertrophic Cardiomyopathy (HCM) - General
chef ed: I was diagnosed with HCM. My questions are:
- What are the available Non-invasive or lesser invasive procedures available to correct my condition? I was informed by my cardiologist to see a cardiac surgeon for surgery, but I understand there are other procedures available that does not have to "open" my chest. Truly, I am scared. My cardiologist says surgery is the best choice compared to alcohol ablation, pacemaker, etc. because ultimately I would still have to undergo surgery in the future.
- How soon should I have the "procedure" to correct my condition?
- Are there any medications that I can take to treat my condition? (Am taking only metoprolol 50mg BID).
- I am confused & was told to have the surgery within a month because my condition might get worse.I would have chest pain on & off especially when I try to walk fast or lift heavy objects, but with rest in-between, I can walk and move normally with no or minimal pain in the chest.
- Are there any precautions also I have to undertake to prevent any untoward incidents (God forbid!) on my daily routine? Diet? etc.I am alone in NY working as psych nurse (manage care) and with no immediate family members which makes it more scary.
Please help. Your forum really came at the right time for me and I also believed it was meant to be! Thank you so much. Respectfully, Chef Ed
Milind_Desai,_MD: The first step is to make sure you are maximized on appropriate medications such as beta blockers (atenolol or metoprolol) or calcium channel blockers (verapamil or diltiazem) . If you are still symptomatic on these maximized drugs, we would need to consider the gold standard therapies which is surgery and looking at your imaging data you have provided, I would recommend surgery as the next step if meds do not work.
Regarding timing, if you are symptomatic now despite meds, then you should theoretically have it done in the next few months.
Precautions: avoid dehydration and avoid excessive alcohol as it can dehydrate. You can do light aerobic activity but avoid severe isometric exercise such as weight lifting beyond 20 pounds and avoid competitive sports.
pinkcure: My mom found out this year she has hcm 62 years old and the doctor put her on diltiazem and now she sleeps a lot is that aside effect from meds or the hcm?
Harry_Lever,_MD: Could be both. We would need to evaluate.
Duncan1: I am several years post-myectomy and am on multiple medications for hypertension. The problem I have is when my blood pressure gets below 120/70, I pretty much am non-functioning mentally. My energy level is horrible with such numbers. Is there any relationship to an HCM heart and having BP get too low?
Harry_Lever,_MD: Medications need to be adjusted.
Milind_Desai,_MD: Since you are post myectomy, the number of meds we can use to control your BP is increased so you can take some which may have a better side effect profile. Talk to your doctor.
pinkcure: What is the best medication to treat a non-obstruction HCM?
Milind_Desai,_MD: I would start with low dose beta blocker. Make sure you are getting the right brand as some companies have had problems with their genetic beta blockers.
Irregular Heart Rhythm in HCM
skindr: My heart rhythm is usually bigeminy/trigeminy ,with recent rare runs of V.Tach usually 6 beats to as long as 20 beats. Usually self-correcting but one 20sec run was paced. Have in place a medtronic pacemaker/defib. checked regularly. Any thoughts or can I place my full faith in my defib. and its protection? I was diagnosed with HOCM in 2005, pacemaker placed in 2009. On Carvedilol and lisinopril . Thank you.
Harry_Lever,_MD: Usually we have to use a defibrillator plus a drug like sotolol or amiodarone to control the rhythm. we want to minimize the possibility of shocks by the defibrillator. It is not clear by me why you are on lisinopril. we usually do not use that drug unless heart muscle function is impaired because in patients who have tendency for outflow tract obstruction, lisinopril can make that worse.
Stafer: Had Septal Myectomy (for hocm) beginning of June - 7 weeks ago. Still in atrial fibrillation and have swelling of ankles.
- Questions: Does the atrial fib go away on its own? after 3 to 4 Months?
- Will a cardioversion help (what are the chances?) or should it be waited out 3 to 4 Months?
- Is swelling of the ankles a result of the heart surgery or the atrial fib?
Thank You for this discussion.
Harry_Lever,_MD: It is now 7 weeks - you can certainly try cardioversion. Sometimes the rhythm does spontaneously convert but if it has not at this time, it is less likely that it will without intervention.
Milind_Desai,_MD: The ankle swelling could be due to atrial fibrillation. If he is in atrial fib you should be on anticoagulation unless there are contraindications. You should speak to your doctor.
madhow: I am currently on a waiting list to receive the new Boston Scientific S-ICD when it be comes available. Could you please explain the advantages of this new type of ICD?
Harry_Lever,_MD: You need to talk to your electrophysiologist. There has not been experience with this device in HCM patients.
Beau: 61 yo male diagnosed & ICD placed in 06, quite symptomatic now with fatigue, dizziness, sometimes intolerance to walking inclines. Recent echo shows 38cm thick, SAM, 29/87 gradient. Metoprolol 25mg & aspirin 325 mg 1x. Cardiologists says myectomy seems to be next. Psoriasis that is worsening began about 2 years ago. Opinions? I have more data if needed.
Harry_Lever,_MD: You need to be seen.
Beau: In the 7 years since I was diagnosed & ICD placed, the progression of symptoms has been quite marked. As I come to terms with the near future possibility of a myectomy, am I correct thinking that not only for current symptom relief but also for halting the progression of symptoms a myectomy is indicated?
Milind_Desai,_MD: Yes - it sounds like it. You would need to be evaluated thoroughly to provide you with advice.
Stafer: Vein isolation via radio waves which was done during the septal myectomy. Does it take a while to heal and will the Atrial Fib last longer because of the radio wave treatment?
Milind_Desai,_MD: In fact, it should help or relieve the afib - if you still have afib, you may need to have other treatments for afib.
REW: Any typical HCM drugs (beta blockers/calcium channel blockers...) linked to or have a side effect of Rheumatoid arthritis? Or any higher incidence of HCM patients having Rheumatoid Arthritis?
Myectomy – Surgical Treatment
nellie36: Is a high gradient with mild symptoms reason enough to get a myectomy. Does living with a high gradient over many years have a negative impact on your heart, possibly shorten your life?
Harry_Lever,_MD: It depends on mild symptoms and what symptoms you are talking about. Dizziness is one that worries us more than the others. Dizziness may be a reason to do it. Further evaluation with stress echo is needed to look at blood pressure response. We don't believe that surgery is indicated unless you are having symptoms, a gradient alone is not an indication for surgery. But you need to be periodic evaluated to see how things are going.
Milind_Desai,_MD: Sometimes patients may underestimate their level of symptoms that is why I would restate what Dr. Lever stated. If you have mild symptoms, I would suggest a stress echo at an experienced center to elicit symptoms.
apw105: In 2010, at age 53, I had a myectomy to relieve HCM symptoms which were obstructing mitral valve function. Is recurrence of these issues common, given my age? I had every complication imaginable, including sternal dehiscence, sternectomy, and a right ventricular free wall rupture due to an adhesion which almost killed me. Would like to avoid re-visiting invasive heart surgery in the future.
Harry_Lever,_MD: It is not very likely to have recurrence. At your age for the heart to rethicken usually does not happen.
Milind_Desai,_MD: In general, we would recognize recurrence early on - in a recent paper we published in circulation, the rate of recurrence after surgery at Cleveland Clinic was only 3% in the very long term. It was mostly due to under recognized mitral valve problems.
Ttsula: As I am a possible candidate for surgery with obstructive HCM, I am concerned about the amount of relief it may provide. It seems to me that since the genetic mutation affects the heart at the cellular level, the basic problem will remain after the surgery. Just as this cellular issue existed for the 60 or so years before I developed symptoms. I understand that surgery is no "cure" but why wouldn't the cellular issue prompt the problems to simply reoccur? Thank you very much.
Milind_Desai,_MD: While it makes intuitive sense, it is not commonly observed. It is not common for HCM to re-occur after myectomy. In a recent study we have shown that long term freedom from reoperation is approx. 97% and none were due to recurrence of hypertrophy.
RoyCa25: I have had one surgeon tell me I need a myectomy and another tell me I need valve surgery and myectomy. How do they make this decision. I had a second opinion because people said I should - now I am more confused. Do I need a third?
Milind_Desai,_MD: We would be glad to see you at Cleveland Clinic.
zimmlm: What might be possible causes for a mitral valve to begin leaking months after a myectomy that resolved mitral valve obstruction?
Milind_Desai,_MD: Under recognized mitral valve problem at the time of surgery is the most likely problem. Another issue is if you have gone into afib and your left atrium has remodeled it can also result in mitral valve leak. It needs to be evaluated by an experienced center.
CeeBee: Generally, according to most studies I've read, a myectomy reduces the risk of SCA -- obviously it doesn't eliminate all risk. But do you endorse a thorough re-evaluation of the need for an ICD when the generator reaches ERI for a person who has had a myectomy?
Milind_Desai,_MD: in general, myectomy does reduce risk of SCD - it does not eliminate it. If you have certain risk factors for SCD, you need to continue having a defibrillator.
Myocardial Bridge and HCM
BobbyB: My 13yr old granddaughter, Carly has gene for HCM. Has had Echocardiocardiograms and heart MRI. Have found no clear cut evidence of HCM at this time. Myocardial Bridge was also discovered during her diagnostic imaging. About 9 months ago, Carly started complaining of chest pains (angina?). She is scheduled for a cardiac cath at all Children's in St. Petersburg on Monday, July 29th. Do you have any previous experience with a person having both HCM and Myocardial Bridge and if it is causing Angina, what can be done. What are your thoughts on this and do you have any advice?
Harry_Lever,_MD: We think that bridges in most patients do not cause symptoms. I certainly would not recommend surgery on just seeing a myocardial bridge.
Milind_Desai,_MD: Most bridges are shallow. One way to definitively evaluate this would be to do a stress echo to see if it is significant. Understand most of them are not significant and shallow bridges. Please consult an experienced center before any invasive procedure is contemplated.
zimmlm: What are acceptable dental anesthesias for someone with HCM and a mitral valve leak? I need to have root canal work done and the endodontist will be checking in with my own cardiologist for specifics on me but for my knowledge I wanted to ask.
Harry_Lever,_MD: We would prefer they not use epinephrine in local anesthesia.
Milind_Desai,_MD: If they were to use major anesthesia , make sure they monitor your heart rate.
a_collers8: Thanks you all so much for the info, time and care you have given. Thank you too, Dr. Lever for the med info I will check into this. Cheers.
Milind_Desai,_MD: Best of luck.
fran49: I will be making an appointment and think I should have been referred to your office or genetic office years ago. Since I am a Cleveland Clinic heart patient all my records will be there. Thank you for all your concern and the giving of your time.
Harry_Lever,_MD: Thank you. And avoid major fluid shifts.