I've had several echos--they found out I was in heart failure (cardiomyopathy) before they discovered I have CREST. At this point, they are still unsure if the two are linked; there are 3 different possible causes for my heart dilation.
I'm not sure what grade 2 signifies. It sounds like they did NOT say you are in heart failure, though, right? That's a good thing!

There are also 4 grades of heart failure, which I think correspond to this. In Fact in their article about "Diastolic Dysfunction" Wikipedia refers to it as diastolic heart failure, and says this about it:

There are four basic Echocardiographic patterns of diastolic heart failure, graded I to IV. The mildest form is called an abnormal relaxation pattern or grade I diastolic dysfunction. On the mitral inflow Doppler echocardiogram, there is reversal of the normal E/A ratio. This pattern may develop normally with age in some patients and many grade I patients will not have any clinical signs or symptoms of heart failure. Grade II diastolic dysfunction is called pseudonormal filling dynamics. This is considered moderate diastolic dysfunction and is associated with elevated left atrial filling pressures. These patients more commonly have symptoms of heart failure and many have left atrial enlargement due to the elevated pressures in the left heart. Grade III and IV diastolic dysfunction are called restrictive filling dynamics. These are both severe forms of diastolic dysfunction and patients tend to have advanced heart failure symptoms. Class III diastolic dysfunction patients will demonstrate reversal of their diastolic abnormalities on echocardiogram when they perform the Valsalva maneuver and are called reversible restrictive diastolic dysfunction. Class IV diastolic dysfunction patients will not demonstrate reversibility of their echocardiogram abnormalities and are therefore called fixed restrictive diastolic dysfunction. The presence of either class III and IV diastolic dysfunction is associated with a significantly worse prognosis. These patients will have left atrial enlargement and many will have a reduced left ventricular ejection fraction indicating a combination of systolic and diastolic dysfunction."

My echogram report says I have the pseudonormalization pattern, so according to Wikipedia that again places me as having a grad 2 heart failure.

In everything I have read, grade 1 of both heart failure and diastolic dysfunction are symptomless. However, for a long time, I've been having mild swelling of the feet and lower legs, fatigue, and huffing and puffing with rather mild exertion. Again, that would seem to put me in grade 2 for both.

I am not a happy camper, especially because the day before I found this out, I had ordered an expensive treadmill for hubby and me for Christmas (mainly for me), and now I don't know whether using it will be good, or hasten my progression! Everything I've read says exercise exacerbates the heart action in this dysfunction, but I can't tell whether that's very temporary, and might have long range benefits. And I've asked my scleroderma doctor 3 times about it, and can't get an answer from him!

I know that exercise is important for heart patients, though the doctor needs to clear you for it. When I was in "stage 3" back in March, I was on orders not to exert myself, but since I've been encouraged to start exercising.

Well, that's GOOD to hear!

Gardener21, do keep us updated on how your feeling my friend.You are in my prayers.Have a blessed day and may the new year bring us all better news and information....

I had echocardiogram twice now. Big difference from a year ago. Waiting for a hearth catherization tests. Had a ct scan also but haven't heard results. Very short of breat now. Was told I have PAH and ILD from the slerederma. No reversing damage done in my lungs.. I too had little skin problems. hugs Marilyn

Hi, JenkinsBride, CJ60, and Maztaz,

Well I've been to a cardiologist and he did a metabolic stress test, and according to that, I've lost about 36% of my heart function. We talked about what sort of things can cause this type of restrictive cardiomyopathy:

Scleroderma
Amyloidosis
Fabry's
Hemochromatosis

We quickly ruled out hemochromatosis with simple blood tests. That leaves the other 3. He has referred me for genetic testing to rule out or confirm Fabry's, and the familial transthyretin version of amyloidosis, because there does seem to be a complex of progressive hearing loss, heart attacks/strokes, and neuropathy that seems to run on my father's side of the family.

But then I also have monoclonal gammopathy which can cause amyloidosis by the primary route, and there seems to be ankylosing spondylitis running in my family which can cause amyloidosis by a secondary route. On rare occasion even scleroderma can cause a secondary amyloidosis. And of course scleroderma can do it directly by causing heart muscle fibrosis. So it's a huge muddle, and the therapies for all these things are different. So I'll probably end up having to have a heart biopsy before it's all over to finish sorting it all out. I am NOT looking forward to THAT! Scary! Visions of dying if they take out one chunk too many and poke a hole in the bottom of my heart! Creates cardiac tamponade and death, and it HAS been known to happen.

And NONE of the therapy options for these things are good. I don't like this at all! I need someone to pat me on the back, and say There, there! :(

Ther there now gardener. LOL. Don't worry too much and sometimes the more we read the more we get ourselves scared. Have you had a heart catherization? I think they will do that before a biopsy. Do you have any breathing problems? My heart problems and yes is enlarged both sides is that I have scarring on my lungs and heart working to hard in lf ventricle. It is 55 while rt is 35 or vice versa can't remember. I am worrying about heart cat even though people say its no big deal. Hang in there. hugs marilyn

Hi Marilyn,

Thanks for the "There, there." I don't feel like I have much trouble breathing, but after the metabolic stress test, which measures respiration, the cardiologist diagnosed me with dyspnea, which means difficulty breathing. So I guess I do have it. Plus I've noticed that I sometimes cough a little, and huff and puff with mild exertion.

The doc said he would do the heart biopsy along with the catheterization. That means they add a little clip on the end of the catheter, and after using to catheter to measure the arterial pressure in the artery leading into the right heart, they then thread the catheter into the heart, through the right auricle, and down into the right ventricle where they use the clip to take a chunk of heart tissue which they then retrieve when they pull the catheter out.

I am really wondering if such a biopsy from the right heart will show anything because my heart's dysfunction is the the left ventricle.

Yeah Gardener they told me mine was in left ventricle too but are doing both. Who knows maybe they will be taking biopsy on mine too. yikes. lol. Yeah my breathing got worse very rapidly. Like three months. Sometimes feel like I should be on oxygen. scry. What it and let doc know if it gets worse at all. Hugs Marilyn

they did my echo when I was first dx and thankfully I have no heart involvment yet, they also did a barium swallow to see if there was any hardening of my esophogas again thankfully no. But lucky me it is only in my lungs. It doesn't seem to be progressing as of yet but they are concerned because there has been no improvement either. They expected some small improvement with the prednisone at least.

Hi gardner
Have they done an echo to measure your heart? Do you have cardiomyopathy?
The heart stuff can be pretty scary--especially with what is out there on the web to read. I thought for sure I'd be dead in 5 years according to the sites I saw after I was diagnosed with CHF. But my heart function has improved quite a bit since I started meds, and I have talked to tons of folks who've been living a long life with this issue. Don't get overly stressed--stress hurts your heart.

Yes, the echocardiogram was the first thing that was done. It showed left ventricular dyastolic dysfunction, grade 2, which is classified as a restrictive cardiomyopathy. Then they did the metabolic stress test which showed my heart lung function reduced by about 36%.

I'm really glad to hear that the medication has improved your heart function. We all just need to hang in there, I guess. My Dad had his first major MI at age 58, but he lived until age 82.

what was your exact result on the anticentromere, mine was 4+, and I've seen where some have 8+, did you have a high ANA too?

My ANA has been as high as 2560, with varying staining patterns: homogeneous, speckled, antinucleolar, anticentromere. My first anticentromere B pattern was high, but not off the chart. My second anticentromere B was ">8.0" where the normal range was 0.0 to 0.9. So high it's no longer measurable by LabCorp.

I feel like it, too!