FDCM in my family history. I went in for my second echo in 4 years. My LV size is in the normal range, but my EF has reduced from 60% to 47%. My question is: in what cause and effect sequence do symptoms of cardiomyopathy take place? Is there heart damage that causes a reduced EF and then as a result the heart enlarges in order to compensate? I was just wondering if that's how it works. I'm still awaiting MUGA test results (and holter monitor results)...if anyone can give me suggestions as to what to look out for, and/or if you can think of any specific questions I should have prepared for my cadiologist when it is time to discuss my results please let me know. Thank you.

Hi jeri L.

There are etiologic differences, but the end result almost always is an enlarged heart that is and has compensated. With SOME compensation the enlarged heart provides more blood/oxygen into circulation with each heart beat. But the heart cannot continue to expand and there is over compensation. The heart loses its contractility and that leads to heart failure.

Failed contractility results from heart muscle disease, heart wall damage (MI), wall thickness (loses flexibility), valve regurgitation and/or stenosis (less blood output with each heartbeat), hypertension (heart has to work harder), arrhythmias (interferes with adequate filling and pumping, burdens the heart), etc. These conditions can/will increase the heart rate (compensation) and enlarge the heart to compensate. The conditions should be treated before there is overcompensation and heart failure.

KenKeith, yes I understand what you're saying about an enlarged heart being the end result and thus eventually leading to heart failure. Now I am wondering if a diagnosis of "early stage" cardiomyopathy is possible based on a low EF alone eventhough the heart ventricals are within the normal size range. A geneticist recently left me with the impression that an EF of 45% or under is generally considered an indication of cardiomyopathy. My most recent echo revealed that my EF is between 45% and 50% but no signs of enlarged ventrical were observed on the echoes. It is the fact that my previous echo said my EF was 60% and my most recent ones showed a reduced EF (on two consecutive echoes within the same week). This is what led my cardiologist to send me for a MUGA scan (for an accurate EF) as well as a 24 hr holter monitor before jumping to conclusions as far as a diagnosis goes. Now, from what I gather it sounds like you're saying if I have any conduction problems and/or valve problems they could have caused damage and possibly led to inefficient pumping ability, and consequently a reduced EF which could cause the heart to overcompensate and put me at risk for heart failure...is that correct? So, are you saying it is possible for a diagnosis of cardiomyopathy based on a low EF alone and no obvious signs of enlargement? Do you believe it could be reversed if caught at such an early stage? I think that's about where I'm at with this thing unless I get lucky and my MUGA results come back to prove my EF is actually higher than the echoes read (which would be nice). Thanks again.

jeri L.

"Now, from what I gather it sounds like you're saying if I have any conduction problems and/or valve problems they could have caused damage and possibly led to inefficient pumping ability, and consequently a reduced EF which could cause the heart to overcompensate and put me at risk for heart failure...is that correct? So, are you saying it is possible for a diagnosis of cardiomyopathy based on a low EF alone and no obvious signs of enlargement?"

Your logic is well expressed but there is no technical dilemma. You're not a lawyer are you?:D

My response was a top-down explanation going from an enlarged heart to possible causation. An enlarged heart will compromise cardiac output due to loss of contractility (we all know that)!

Going the other way (bottom up) will the signs ans symptoms discussed alway cause a dilated LV? That poses the question can heart failure happen without and enlarged heart? Yes, heart contractions can be impaired without LV enlargement. Some examples that come to mind are myocarditis, structual abnormality, pericarditis, etc.

Measurements of the chambers are subjective as the borders to be outlined by a transducer are fuzzy. So it is an estimate with + - 10%. In addition chamber sizes changes regularily to adjust for pressures and equalize blood flow between chambers.

Two years and 6 months ago my LV was dilated and my EF was below 29%. It was dilated due to afterload (high bp) and damaged muscle to a prior MI. Recent report from an echo shows EF 59% and no appearance of any muscle damage or impairment. The condition was reversed with medication, exercise, diet, etc.

"Development of atrial fib and loss of atrial systole may further reduce forward output because of impaired filling of the hypertrophied left ventrice." Comment from an article on Mayo website.

From what I have read an enlarged heart can disrupt and disorganize electrical signals causing arrhythmias. And arrhythmias can enlarge the LV. Apparently, one or the other can precede the other. Depending on the circumstance and degree of arrhythmia there may not be any further complications nor a need to compensate.

The trend of your EF rather than the numbers itself should be followed up. That is the best procedure given the circumstances.

Thanks for clearing that up. Good to hear medication can turn things around. Yes I'm following up on my EF trend (basically that's it). And no I'm not a lawyer:rolleyes: just trying not to get too confused seeing how most of these medical terms and heart physiology is still a bit foreign to me. Thanks for putting up with my so-called logic. :wave:

Hi~
This is an interesting post, and what I was kind of trying to figure out also. I had a recent echo, with an EF of 52%. The letter said my heart was working well as a pump with an EF of 52%, and my valves and ventricles were normal and functioning properly. I found out from looking it up on here, though, that 52% is rather low, and so I am concerned about the same thing, that I could have early stage cardiomyopathy, even though everything else with my heart seems ok. I can't really figure out if 52% is that bad or what. I have checked on some sites that say anything above 50% is ok, but given the test has a +/- of about 8% from what I read, that could put me in the danger zone. My Dad died of congestive heart failure at 69, so this is a concern for me. I am 46 right now, but also out of condition and overweight. I was also wondering if a low EF is expected if you are overweight, and out of shape. I've been walking each night for an hour, and feel pretty good otherwise. Thanks for any help! Val

hypertension (heart has to work harder)

Now I know why my blood pressure is so good 120/80 even though I had mild enlarge LV, mild posterior and distal anterior wall hypokinesis. It is a lazy heart. It doesn't want to work harder, it just "Pause" there and wait for the pacemaker to beat! Some how it pauses at 3.2 sec but didn't see the pacemaker kick in at 1 sec. :confused: Pacemaker gets lazy as well? Don't forget to post your holter result here. I prepare to share mine one with you all. My cardio said my holter report "It didn't make sense!"

Pika

Thanks for clearing that up. Good to hear medication can turn things around. Yes I'm following up on my EF trend (basically that's it). And no I'm not a lawyer:rolleyes: just trying not to get too confused seeing how most of these medical terms and heart physiology is still a bit foreign to me. Thanks for putting up with my so-called logic. :wave:

Not meant as a criticism...impressed.:)

A dilated LV (remodeled) can certainly be reversed when the underlying cause is controlled. There may be only be a window of opportunity to reverse though. A grossly enlarged heart may in itself cause irreversible impairment due to damage to the cellular structure of the muscle when overly extended.

Hi~~
Again, thanks for all the very helpful information. Each person on here has thier own set of worries for whatever reason, and it really helps to get on here and get the information that sometimes even the doctors don't take the time to tell us.
In my case, my Dad died at 69 from some type of cardiomyopathy, and so that makes me obviously nervous about it, especially when I do have some heart issues. I recently got back my echo results, and my EF was 52%, and I figured that was kind of low, so I wanted to make another appointment to go in and discuss this with the doctor, and the nurse called and said that I could do that if I wanted, but that is a very normal number, and that it dosen't show any sign of cardiomyopathy. I was worried because of all the PVC's I get, but she was very reassuring that everything was fine. From everything I read on here, 52 seems rather low, but she seemed to think it was fine. I just want to catch any decline early. I had my records sent over from 3 1/2 years ago, but I don't know if that will help or not. She sort of left it up to me whether or not to go into the doctors, but she was very reassuring that it was an ok number. I was thinking maybe I should just wait a bit, keep exercising, and stay on track. Do you think that would be ok, or is 52 getting dangerous? Also, I still am very confused from reading different things on the internet, some say that once cardiomyopathy is present, you can't improve your EF number, or strengthen your heart muscle, and other places say there is alot you can do. I am 46 right now, and my Dad started running around my age, and we sometimes think that is why he stayed healthy for so long, that he got himself in great condition. So, I want to make sure I make the right choices also. Anyway, thanks again for the information, and the help. Also, she said that my LF was normal size and function, does that mean heart size? I asked her if my heart was enlarged at all, and she said, no, that my LF showed normal size, but I didn't know that is what LF meant. Don't they actually take a measusrement of your heart muscle itself during an echo? Thanks again!! Val

val,

Please don't confuse secondary DCM with primary DCM. Secondary heart enlargement can be due to the heart overworking from high blood pressure (indicating high resistance), fast heart rate, poor contraction due to MI (sometimes cells only appear and function as necrotic, referred medically as hybernating, and sometimes begin functioning again when oxygen is again supplied to the deficit area), valve malfunction (regurgitation or stenosis), etc. Correct those problems can reverse remodeling.


Some information on PRIMARY DCM: "Viral infection and autoimmune disorder can cause DCM. However, a familial trait is present up to 50% of cases, indicating a major role of genetic factors. The analysis of the phenotype (visible traits that characterize memebers), the pattern of genetic transmission, and molecular genetic findings have allowed the characterization of different forms of familial DCM, suggesting genetic heterogeneity. Furthermore, the risk of disease has been estimated as high as 20% in relatives of familial DCM patients, which is significantly higher than the normal population. Taking into account that DCM can be clinically not evident due to its low penetrance (% of organisms having a given genetic constitution showing corresponding hereditary character...in particular in the young population), a reproducible and reliable method for the diagnosis of familial forms is critical in the management of the disease. To address this issue, consensus guidelines for the diagnosis and screening of familial DCM have been developed. The screening method for familial DCM is based on physical exam, electrocardiogram, and echocardiogram of first-degree relatives of affected subjects. The family screening should be followed-up every 2 to 3 years, in particular in unaffected relatives (in the absence of a molecular diagnosis), to exclude a late onset of the disease."

If your father died of DCM, it may or may not be a familial trait passed on to you. Your EF is normal, and possibly raised (if need be or desired) by aerobic exercise. There is medication to increase contractility but you are far from a need for that.

Hi, and thanks so much for the very informative answer! As I have said before, definitely more information than I even get at my doctor's office!
I guess I mentioned that I spoke with the nurse today, who I do like, and have been in contact with for years about my PVC's. She really seemed to think that 52% was ok, because she said it's very subjective, in that if you lined up ten cardiologists, they would all have a very different EF in someone's case. I guess that could also put me below 52, though, but I guess I have to think positively. She said that it's not like a math quiz, in which a higher number is better, she was like if your in the "normal" range, then that is good. I do get the swollen ankle thing, though, but I have been getting that for years. I get slight swollen ankles when I sit too long, such as on an airplane. It's not really bad, but noticeable to me, and I can kind of feel it.
My Dad's case was a very strange thing. He was in very good health, a runner, and just someone who took great care of himself. He was on a long drive, and suddenly started getting the symptoms of CHF, swollen extremities, shortness of breath, and by the time he got home, he went to the doctors. They could never figure out what was wrong with him, because really he was in really good health other than these symptoms. They were throwing around the CHF word, and also Restrictive Cardiomyopathy, which I've read is the worst. I think they said he had some "stiffening" at the bottom of his heart. All his tests kept coming out ok, though, and so he was a few days away from having a heart biopsy at Stanford Med Center, and he passed away suddenly on the couch with my Mom. They were going to check for some kind of infiltrative heart disease, such as Amyloidosis, I don't know if you have ever heard of that. It's an extremely rare disease, hardly ever seen in the U.S. So, he died before we knew what he really had. It sure would be nice, for closure and other reasons, to know if he had Amyloids or not. They can be familial, but the expert from Stanford got on the phone with me, and said his type was probably not, I guess because of his age at onset, which was 69, and maybe other factors. There was one doctor in Reno, where he lived, that said that he "overworked" his heart maybe, but again, that was another doctor trying to figure it out. We were worried for a while, and actually still am, that he died because of some pill interaction that he was on. They gave him some kind of diuretic, and they gave it to him with a potassium supplement (I think it was).....anyway, my brother looked it up after his death, and supposedely you are never supposed to mix the two that he took. I called the doctor on this, and he said, no, we were trying everything just to see if it would work, I think he kind of acted like they thought there wasn't much hope really. I think when someone is having all the symptoms of CHF, there isn't much the doctors can do. Also, if it was restrictive cardiomyopathy, and/or amyloidosis, that has a really bad prognosis. The hard thing now is to live with not knowing if we dropped the ball on not really knowing what he was taking, or if he had something that was really bad. I'm actually kind of glad that he never knew what he had, because I was really worried about him getting that kind of news. He would have been a wreck about it. He just went so fast.
Anyway, I just wanted to explain my Dad's situation, and thanks again for the very valuable information! I'm sitting here tonight thinking my ankles look swollen, so off I go for a walk. I think I might be obsessing, who knows. Anyway, thanks again!! Val

Val, Kenkeith is right. Your dad's cardiomyopathy may or may not have been familial. If it wasn't familial- meaning caused by an inherited gene- then you shouldn't have to worry about you yourself having the gene. But because you're not exactly sure it's wise to take precautions. It also sounds like you're not positive about the type of cardiomyopathy your dad could have died from. Have you acquired your dad's medical records. You might consider doing so for your own file simply because records are only kept for a limited time. My dad died of dilated cardiomyopathy in 1987, so his records have since been destroyed. My brother died of dilated cardiomyopathy in 2003 and I was able to get a hold of his records which included references to my dad's diagnosis of the same desease (same cardiologist diagnosed and treated both of them) and confirmed that it was most likely familial. If DCM is in fact familial this means that any child of a parent with DCM has a 50% chance of inhereting the gene. I think that statistic mainly refers to the "autosomal dominant" gene. (it's complicated and can get confusing). My brother didn't get a genetic test because it wasn't available until two years ago. However, some testing is available now, but it's unreliable because it's so new. In other words, even if a first degree relative of FDCM does develope it there is only a 20% chance the genetic test will show it because the test itself is not perfected yet. That's why it is still recommended first degree relatives get echo screenings every 2 to 3 years. Since you're searching the internet for info have you checked out Johns Hopkins website on familial cardiomyopathy? Their clinic does a lot of screening of families with history of DCM as well as genetic counceling. You can also call them and they can help put things into perspective and give recommendations. Your EF is 52% which is the low end of the normal range, but you're not sure how accurate the echo observation was. My EF was 47% on my first echo, and between 45% and 50% on my second echo. My cardiologist sent me for a MUGA scan (a type of nuclear test) because he said it would provide an accurate EF number and take out any discrepencies. I'm still waiting for my MUGA results. When you say your "LF" is of normal size do you mean LV? LV is left ventrical. Did you find out what LF is? Thanks for keeping in touch.

Hi~~
wow, thank you for all that valuable info! I will make sure to try to get my Dad's records, that is a really good idea. Especially since there is a question as to how he died, that would especially be a good idea. I know that at the time of his death, however, there was still no diagnosis other than speculation. I think they said he had Restrictive Cardiomyopathy, though, and am not sure if that is the same as Dilated Cardiomyopathy. That's why he was going in for the heart biopsy, because there are only a few rare things that can cause the Restrictive Cardiomyopathy. One of them, and the one I think that the specialist in Stanford was leaning towards, was infiltrative amyloidosis. It's not a common disease.
Maybe I did mean LV, I'm not sure. She said it on the phone, and maybe I'm thinking LF because of EF. My letter said that my ventricles are normal in size and function, and my valves are opening and closing properly. The thing is, I was just concerned that it could be some early stage cardiomyopathy because of the 52% EF, and also I tend to get slight swollen ankles when I sit too long, but I am overweight and out of condition. I've been walking each day, so I'm making the effort! Thanks again for all of the help, that is such good advice to get my Dad's records, even if it is hard to kind of live through it again. It makes me feel so guilty to see stuff like that, thinking how I could have helped him instead of trusting the fact that he was getting the best care.
So, anyway, I guess I'm wondering if he did have restrictive cardiomyopathy, that is different from the dilated type, right? Also, I have two brothers, one older and one a little bit younger, and they are doing fine. My older brother is 48, younger one is almost 40. The specialist told me on the phone that it was 99.9% not familial if it was restrictive cardiomyopathy caused by amyloidosis.
Thanks again for all of the help! Val

Val, yes it can be a bit depressing reviewing a deceased family member's medical records, so don't put yourself through any unnecessary pain. I think I would have endured reading my dad's records easier than my brother's. It was hard. However, after learning my dad's records had been destroyed I wanted to make sure my brother's daughter would have some medical history when she gets older, so I got his records. Anyway, it was requested by the geneticist I am working with. Otherwise, I wouldn't have wanted to put myself or anyone else through that. But it is a good idea to have the info just in case. I don't know much about restrictive cardiomyopathy. I'm still new at learning about the familial dilated form. Check out the Johns Hopkins website. I think you'll find it to be informative.

Hi~~
Thanks again for the info and that website, I'll be sure to check it out. It's hard to remember, but I think when I was researching stuff for my Dad, and trying to help figure out what he had, I think the Dilated Cardiomyopathy usually comes on at an earlier age than his. He started getting symptoms at 69, and was gone shortly after that. Actually, it will be four years this Sunday, and I can hardly believe he's been gone that long. He was such a force in this family, we all turned to him for advice and support, and he did everything he could to live a long and healthy life. He used to lecture us on getting more exercise and eating right. Nobody could believe it when he suddenly got sick. When I spoke to the specialist on the phone when my Dad was still alive, he said that he suspected that it was Amyloidosis that was causing my Dad's decline, and if it was that, then he gave him 6 months to a year. He said for some reason, they are starting to see more and more of the Amyloid cases, and he didn't know why. It's actually a blood disease, where proteins infiltrate the heart, and cause stiffness, which is the restrictive cardiomyopathy, which if I remember right, is the worst type of cardiomyopathy to have. Anyway, I will be sure to look at that site, and also get my Dad's records. I think my Mom destroyed alot of the records she had at home, and so it's a good idea to get what I can. It's funny how it's hard sometimes to get family history. I ask relatives about older people who have died, and it's hard to get a straight answer, because most people don't really know alot of medical things. I think my Dad's side of the family might have had some strange things, maybe related to amyloid, but I'm not sure. His Dad had some nerve problems, and there are heart issues with others in his family.
At this point, I guess all I can do is gather up the records, and do some investigating on my own. The doctor dosen't seem to think I'm having any major problem right now, so I guess I should just go back for an echo in a year or so. I was thinking of it as 52% being so low, but the nurse sort of said that if you are in the normal zone, they are not so concerned with it being low normal. It seemed to me that was too close, but I guess with your heart, as long as you are pumping out at least 50% of the volume taken in, that is supposed to be sufficient. It's hard not to analyze everything now, though. I am always looking at my ankles, and noticing any swelling, and I notice how I'm always feeling tired. Anyway, sorry to babble on and on here, thanks again! Hope you are all well~~Val

Hi i just decided to look through this board and i saw this thread...are you guys saying that there is like medication for restrictive cardiomyopathy? I was told by my cardiologist that there wasn't

Hi i just decided to look through this board and i saw this thread...are you guys saying that there is like medication for restrictive cardiomyopathy? I was told by my cardiologist that there wasn't

RESTRICTIVE cardiomyopathy is almost always due to thickening and stiffening of the heart and septal wall reducing capacity to fill during the diastole phase. During systole (pumping) the stiff walls reduce contractions (wall motion) and the cardiac output cannot provide enough blood/oxygen to meet the body's demand and the symptoms would be shortness of breath, chest pain, fast heart rate. MEDICATION can treat (sometimes not) the symptoms. The dimensions and functionality (with med help) remain status quo or progress. HOWEVER, if appropriate an operation can surgically remove some heart muscle tissue sometimes enabling the heart to function more efficiently.

For insight the Frank-Starling law in physics states the fundamental principle of cardiac behavior is that the force of contraction of the cardiac muscle is proportional to its initial length. The energy set free at each contraction is a simple function of cardiac filling. When the diastolic filling of the heart is increased or decreased with a given volume, the displacement of the heart increases or decreases with this volume.

This principle is very important as the heart rate and left ventricle flexibility enables each heart stroke to provide a balance of blood flow equality as well as pressure. When one side or the other heart side begins to fail there will be over compensation due to reduced cardiac output. If the underlying condition that caused the heart to over compensate is successfully treated, there will be reversed remodeling (remodeling med term for increased dimensions) returning the heart to normal dimensions. If the underlying condition is not treated successfully, the heart will over stretch damaging the cells and myocytes of heart tissue and the heart will fail.

Your confusion is understandable as the thread intermingles primary and secondary heart enlargement, and then jumps to restrictive cardiomyopathy.





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Well i was always confused about this when I got diagnosed... I have a stiffened septum so i guess that messes with the way my heart pumps....thats why i quit school sports. I always thought this was restrictive cardiomyopathy...maybe not. I've heard me as having cadiomyopathy and restrictive cardiomyopathy kinda confusing lol:dizzy:

Hi~~
I think it's all very confusing, and here were are four years after my Dad died (actually today on October 8th).....and we still can't make heads or tails out of it. I suppose this type of thing happens alot to people, though, when someone dies before the tests are done to tell what it was. An autopsy could have been done, but my Mom did not want that. The doctor offered to go in with just a needle, and do the heart biopsy that he would have had anyway, but she wasn't thinking very clearly, and said no. I wish in hindsight that we had at least done that. But, I do know that they said he had restrictive cardiomyopathy. Something to do with the bottom part of his heart stiffening. Also, they did say his heart was enlarged (I believe). So, anyway, that is why I was concerned when my EF came out at only 52%, but I'm trying not to obsess about the actual number, and just take care of myself. If I remember right, I think my Dad's EF might have been normal. He didn't have much that they could find wrong with him, except declining symptoms. He could have been a good patient on an episode of 'House', I always think of that. He was such an unusual and strange case. He was just so healthy before he got these weird symptoms. I remember even thinking it could have been something to do with alot of mosquito bites he had gotten when he was at our house cleaning out the yard. Anyway, thanks for all the info on here! thanks, Val

Well i was always confused about this when I got diagnosed... I have a stiffened septum so i guess that messes with the way my heart pumps....thats why i quit school sports. I always thought this was restrictive cardiomyopathy...maybe not. I've heard me as having cadiomyopathy and restrictive cardiomyopathy kinda confusing lol:dizzy:

Cardioyopathy is a noun literally meaning a heart muscle disease. Restrictive and dilated are adjectives. Restrictive literally means to hold within limits, so capacity is limited during the filling stage.

Dilated literally means to expand. Dilated or enlarged heart usually refers to the left ventricle. Dilated and restrictive heart conditions aren't mutually exclusive. So one can have both.

If the septal wall has a defect, pumping blood into circulation could be compromised. The low output will cause the heart to expand in an effort to increase blood flow, and there will be an increase in fluids causing the heart to work even harder. Treatment by reducing the heart's workload will sometimes (depends on underlying problem) reverse remodeling, but if there is no treatment or treatment fails it will reach a limit in size and lose/diminish its contraction ability resulting in heart failure.

In a situation you suggest, a septal wall impairment could cause the LV to increase in size and work harder.