Hypertrophic Cardiomyopathy

I had a echocardiogram last week. the diagnosis is HCM my internist informed me of the disease, and instructed me to be extremely careful with exerting myself.  this came out of the clear blue to me. I am trying to learn as much as I can, I have an appt with a Dr Naidu at Winthrop University Hospital, Mineola NY on Tues June 15.  Please help me compile a list of questions I should ask.  I have 2 other sisters and my children and theirs are very concerned since it seems to be a genetic predisposition.  I will have a 2nd opinion, no matter what this cardiologist says, because I believe knowledge is the most important thing. I am so thrown off guard, I am almost frozen with what to do next.  Should I make an appt at Cleveland Clinic and with who or is that going overboard..The only thing my internist did was put me on Toprol XL until I see the Cardiologist on Tues.  The only info I can understand in the written report of the Echo is that my heart is 49 at rest and 116 when working.  What does this all mean.  Time is of the essence.  I would appreciate any input so that I ask the right questions and do the right thing for myself and family members. Thank you

[heart_of_mine]

Hi, I have Long QT Syndrome which is different than HCM but it can have the same results and needs to be treated by specialists familiar with the condition. Treatments are very specific to this condition and include anti-arrythmic medications and sometimes ICDs. Your whole family needs to be assessed as well. Contact SADS www.sads.org or CARE www.longqt.org for doctors in your area that specialize in HCM if you feel the need for a second opinion. If it makes you feel any better, I did a quick google search on Dr. Naidu and it seems he is quite qualified to diagnose and treat HCM.
I know exactly where you are at right now and how you must be feeling. I went through all that when our lives were totally disrupted with our Long QT diagnosis. We are all now adjusting and life is going on as usual. As you research your condition you will quickly realize how lucky you are to have been diagnosed as most families with our 2 conditions find out only after a loved one has suddenly died, but with treatment we can and do live fairly normal lives with these conditions.
Questions you should ask are what your limitations should be. What treatments you will have. Who and when should your other family members be tested. And what your genetic testing options are to help diagnose asymptomatic people in your family. After a defect is found in you all other members can be tested against your results.
I wish you the best at your appt next week and please post how things went.

[kenkeith]

Do you have a copy of your echo report?  An echo ESTIMATES the thickness of heart walls, and if you haven't had a prior estimate of heart walls' dimension to compare there may not be a medical problem nor any progression.  
For some insight to help you understand and ask the right questions...Hypertrophic Cardiomyopathy (HCM), the muscle thickening occurs without an obvious cause.   While HCM has typically been recognized by its structure ie., hypertrophy, the electrical function of the heart are also adversely affected (have you had an EKG?)  Do you have any heart rhythm problems?  The 49 heart rate at rest is somewhat low, but I doubt that is associated with DCM. The 115 with exertion is not unusual nor unexpected.

The main feature of hypertrophic cardiomyopathy is an excessive thickening of the heart muscle (hypertrophy literally means to thicken). Thickening is seen in the ventricular septal measurement ( wall that separates right and left chambers...normal range .08-1.2cm). In HCM, septal measurements may be in the range of 1.3cm to 6.0+cm (does you echo estimate show dimension outside the normal range). Other considerations heart muscle may also thicken in normal individuals as a result of high blood pressure and apparently,you have high blood pressure and treated with toprol.  

If the high pb gets under control, the HCM may be helped IF YOU HAVE HCM or just a marginally increase in wall dimension that may or may not be significant.

[23laurie]

Thanks for all info.  I was put on Toprol only because of the echo's result. I have had a good bp even now.  I do have dimensions, but the wording is not exactly one I  can understand.  I will give you the following measurements and maybe you can help me translate. AORTIC ROOT DIASTOLIC 2.6; SEPTUM DASTOLIC THICKNESS 1CM; LV DIASTOLIC DIMENSION 3.7CM; LVPW DIASTOLIC THICKNESS 1CM; LV SYSTOLIC DIMENSION 1.5; LV OUTFLOW TRACT 2.1; LEFT ATRIUM SYSTOLIC DIAM. 3.1;  Does any of this make sense to you? Also, I did have an echo about 12 years ago, not because of a heart murmur, but because of a job physical.  All was normal then.  I appreciate your interest.

[kenkeith]

Aortic root diastolic 2.6  (normal 2.0 - 3.7 cm) This dimension is the size of the opening for blood passage into the system from the heart.  

Septum diastolic thickness 1 cm (normal 0.6 - 1.1 cm).  That is the wall that separates the right and left chambers.  

Left Ventricle diastolic dimension 3.7 (normal 3.5 - 5.7 cm) This is the size or capacity of heart chamber at end of the filling phase.  If you have HCM, usually capacity/size is reduced as a thickened wall is not very flexible and the increased size crowds out filling capacity.  There doesn't appear to be a problem!

LVPW (left ventriclde posterior wall) 1.0 cm  ( normal 0.6 - 1.1 cm).  That would/should be higher for HCM.  A thickened wall would not contract effectively and also reduce size of chamber to fill adequately.  No problem!

LV systolic dimension 1.5  (normal is 3.0 - 4.0 cm).  This is the measurement at peak time of pumping phase. IS THIS AN ERROR!

LVOT 2.1 (normal is 2.0 - 2.2)

LA systolic diam 3.1 (normal 1.9 - 4.0) This is the size of the upper left chamber during  pumping stage.

Does the conclusions of the report state something similar to: Left ventricular chamber size was normal with normal wall thickness. Normal left ventricular systolic function with hypokinetic (wall movement impaired) function.  Left ventricular ejection fraction is 55 % or higher, or does it state your EF (ejection fraction, normal is about 55-75%...the amount of blood pumped into circulation with each heart beat is below 50%.  If you have HCM, your EF would be compromised.

From what you have submitted there doesn't appear to be any heart problem, but there should be an explanation to LV systolic 1.5 cm.  If your EF is above 50% there is no left function impairment, and you don't have a serious problem if any.

[23laurie]

I am reading my report along with your info.  I am curious as to how you are so knowledgable.  anyway my EF is 65-70% left ventricular chamber normal septal hypertrophy observed,  left ventr. hyperdynamic.  LV systolic is 1.5,Lv outflow gradient is 49 at rest to 116mmhg,
Grade 1 diastolic dysfunction consistent with impaired LV relaxation Systoloic anterior motion of MV, mild Mitral regurgitation.  Is it possible i have been diagnosed wrong? Please let me know as I have been led to believe I have a life threatening disease that need urgent attention by a specialist in HCM and even more distressing is that my children an siblings could be carriers of this abnormality in our genes and should be tested.  Looking forward to learning more Thanks

[heart_of_mine]

I would be cautious of taking someones diagnostic medical advice over the internet. You should go to your appointment and see what the doctor has to say. It could be possible that you had a wrong diagnosis, but from what I have read about the doctor that you will be seeing, he will be able to give you a definite diagnosis.

[23laurie]

Thanks, I am thinking exactly what you said.  I am definitely keeping the appt. Unfortunately, it is a human response to false hope, when we want to beleive their could be a wrong diagnosis.  But I am now back to reality and realize that I absolutely need to see doctors to get the CORRECT diagnosis.  Thanks for your concern and support, I will let you know how things turn out. Laurie

[kenkeith]

I have provided parameters and description for the numerical values that were posted, and provided no advice but based my answers on info as it was provided.  I agree don't take any medical advice from the internet, but this forum can provide information and an understanding, insight and perspective of the medical condition discussed, and this will enable the interested party a plateform to discuss their medical condition with their doctor and grounds for a better understanding.

Not important but my experience is going on 5 years of reading, researching, and answering inquiries on several ************.  I'm not being defensive, nor trying to impress (not important to me to impress anyone and that seems to be implied!) but the question was asked.  I have had some educational courses that helps understand the biology, terminology  and other scientific aspects, inaddition to my own medical condition experience.  There are several members on this forum that also fit that description and in my opinion better.

The latest post regarding the medical problem of interest that stated  "Grade 1 diastolic dysfunction consistent with impaired LV relaxation Systoloic anterior motion of MV, mild Mitral regurgitation" is not inconsistent with having a preserved EF. The doctor states there a relaxation impairment which means the filling phase is impeded.  If serious and not treated it can eventually lead to a systoic dysfunction, and then heart failure.  

The doctor or nurse should not have led you to believe your condition is life threatening or at least have pointed out your conditrion is not that serious at the present time. Take care, and I will now return to my week-end activities.:)

[23laurie]

I am going to cardiologist and will let you know his opinion

[heart_of_mine]

I didn't mean to say that you were giving medical advice, I just wanted to make sure that 23laurie didn't think that she should not continue with her appointments with the false hope of a wrong diagnosis. HCM like LQTS is a tricky diagnosis at the best of times but is always worth the extra steps involved in finding it because of the possible deadly arrythmias both conditions can cause without much warning. I do appreciate your knowledge and you always respond appropriately to people looking for answers.

[maggiemag]

HCM causes an elevation in EF, not a decrease like DCM (DILATED) would.  
So if the OP's EF would be over 70%, they could indeed have HCM, although the other numbers sure look pretty normal to me.  I cannot interpret the gradient though.  Grade 1 DD needs to be watched but certainly is not life threatening.  A severe HCM can cause fatal arrhythmias, so you need to make certain of the diagnosis.  FYI also, as a diast. dysfunction progresses, it leads to DIASTOLIC heart failure with a preserved EF.  They are doing more and more research on DD nowadays and I think it comprises  almost half of the patients with heart failure today.

[BionicBabe]

Hi, I have a couple of friends with HCM (serious enough to necessitate ICDs). They are both doing very well and live very active lives with positive outlooks. I have a focal wall cardiomyopathy, which I don't totally understand, but anyway, in my case it causes VTach, which is a serious arrhythmia and necessitates an ICD. It sounds like you are being very proactive about getting the diagnosis right. I hope that further assessment indicates that either you do not have HCM or that it is a very mild case. Regardless of the results, there are many great treatments! Beta Blockers are commonly prescribed nowadays to reduce the workload on the heart, regardless of whether you have a high BP. Everyone I know who has cardiomyopathy is on some kind of beta blocker. The good news is that they really help the heart muscle! The bad news is it can take a while to overcome the side effects. I've been on Coreg (beta blocker) for 4 months and am just now starting to feel normal again. You asked for possible questions for your doctor...I'm not technical enough to know what to ask, but I would recommend you bring someone with you to your appointment, because it might be overwhelming depending on what the doctor says and having someone else there to take it all in might prove helpful.  Good luck!

[23laurie]

Thanks for your words of encouragement.  I have seen the cardiologist and all I know right now is that I definitely have HCM. Further testing will determine severity and treatment mode.  I am already on a beta blocker and it hit me like a ton of bricks.  I am sleepy always, spaced out sometimes.  I am going to try and focus on the journey my family is on.  Good news both of my sisters who did come with me to my Dr. appt, do not show any signs via echocardiograms. My son is being tested this week and I pray more than anything for his well being.  Thank you all so far for all the info and support.  Laurie