update after long hiatus (Ejection Fraction dropping low)

[dughr]

Hi everyone.

It has been a long time since posting here for me. I had my myectomy now 15 months ago. Time sure does fly!! Some may remember that I was the first myectomy done at NEMC which kicked off the clinic there being able to be a one stop shopping center for anyone with HCM. I have always had very good treatment there and am grateful they are there...especially since I live right here in Boston and can stop in on my way home from work.

Well, after all this time I went through the usual recovery that most all of you know all too well. I presented a few additional problems however. The pathology of the heart tissue uncovered a new and also rare disease called Sarcoidosis. They found these things called granulomas in the heart. Dr. Maron and Dr. Udelson decided that I would need to be followed very carefully as this disease can cause some of the symptoms that are very like HCM with obstruction--breathing dificulties etc.

Well I had a rough summer with deaths in the family and general bad cards beaing dealt so it was easy for me to just cancel all my appointments and forget I had these problems. Now this fall I got a call from Doc Maron and he was concerned he had not heard from me so I comitted to go in and get a follow up.

One year later the sarcoidosis is in check (no prednisone thank god), but, and now I finally get to the jist of my post, when they checked me over at the HCM clinic I show zero obstruction but a significantly reduced ejection fraction. They tried not to get me too worried and said they thought it was the sarcoid causing it but I have a doctor who in the literature is one of 2 nation wide authorities on sarcoid and he said it is NOT the sarcoid. So now I am looking at???????? I understand that the EF is a good measure of the overall efficiency of the heart. I never really recovered from this and still have breathing problems a a general lack of energy. I turn 40 in November and thought by now I would be feeling better than I do.

Is this something any one else has experienced?

What drugs do they throw at a low ejection fraction?

Anyone have any ideas? I am waiting to hear back from the doctors as to what they conclude but in the mean time this is a good place to find some advice or learn more about what this all means.

Thanks a bunch.

Doug

[Pam Alexson]

Re: update after long hiatus

Hi Doug,

Welcome back. You have been missed and though of very often.

I will always remember how kind you are and how special it was to me that you came to visit me after my myectomy at NEMC( # 3, first girl).

I am sorry to hear about the tragedies you have had to go through and especially about you not doing well since your surgery. I am glad that you are following up with them at NEMC.

As far as the low ejection is there also thinning of the walls or ventricle chamber ,leading to a dilated type cardiomyopathy ?
This info needs consideration in making choices. Sometimes the ace inhibitors are started , sometimes pacer therapy ( you still do not have a pacer/ defib ?), and depending , I have read about left ventricular assist devises. Of course unfortunately the other biggest is heart transplant.

Nothing else comes to mind, I hope these are of some help.

There appears to be a lot of possibilities up in the air and I can imagine the wait is not comforting to say the least.

Hang in there, we are all here for you. If I can be of any help , please let me know . I will send you my phone number if you want to talk or , I am listed if you want to look me up either way don't hesitate.

My thoughts and prayers to you .
Pam

[mtlieb]

Re: update after long hiatus

Hi Doug,

I'm glad to see you post and update us on how you are doing.

As far as the ejection fraction issue, i don't know what your specific numbers are, but i've been under the impression that with HCM, lower is better than higher? Mine dropped a bit after the myectomy (low 50's now) and i was told that this is not only perfectly normal... but actually a good sign that my heart is not working as hard as it was before the surgery. Again you may be facing a completely different situation entirely so forgive me. Perhaps others will come along and shed some light on this as well.

Take care, and nice to hear from you!

Jim

[dughr]

Re: update after long hiatus

Thanks Pam,
And thank you too Jim.

I enjoyed meeting you as well.

I do not yet know (to answer your question) if there is thinning of the walls. That is why I posted...this way I have a better idea what to ask the doctors. Currently, I have no devices implanted and am only on 120 mg. Verapamil for high blood pressure. Average for me is 150/105. The verapamil isn't controlling the BP at all. Not sure why I have the high BP...I eat VERY healthy and lost 35 pounds after the surgery.

Anyway, thank you for the input and kind words.

Doug

[Pam Alexson]

Re: update after long hiatus

Just to add comment about the ejection fraction .

50 and above is considered normal, ( we with HCM tend to run higher then normal , often above 75 due to the hyperdynamic contractibility of the muscle , ie, force of compression), however 40 is heading into the lower range. This deals particularly with the compression /strength of the contracting muscle. Answers to the % of blood ejected from the ventricle with each beat/ contraction. If the ejection fraction is decreasing over time then this is usually an indicator of failure; loss of ability to squeeze so to speak. Often referred to the "burn out phase" .

There are many variables to this and not all hearts fall into the same reference range. It is very important to get all the facts from ones doctors and to talk candidly about these references and how they are specific to that individuals heart condition .

In summation, the more questions you can ask the more clarification .

Pam

[dughr]

Re: update after long hiatus (Ejection Fraction dropping low

Hi again,

OK. I talked with Dr. Maron tonight and have some more info. Turns out that is my recent echo the EF was measured at 38-42. I also had this new Cardiac MRI with contrast and it concured completely. They are still being careful as to where we go with it but at 38-40-42 it is lower than last year when it was at 55%. I also do have some thinning of the walls. Just wanted to update the findings.

Thanks again. Doug

[Pam Alexson]

Re: update after long hiatus (Ejection Fraction dropping low

Doug,
Thanks for the update. We are all here for you . Remember, if you need to talk , or for me to just listen ,I am available to you.

Thoughts and Big Prayers.

Pam

[Lisa Salberg]

Re: update after long hiatus (Ejection Fraction dropping low

Doug,

While much of your case is treated like HCM - it is not going to be the same due to the 'Sarcoidosis'. An EF of 40 is not great - but it is also not that bad. For a lower EF they use Coreg and other ACE drugs - basicly all of the drugs you avoid in HCM are now appriopriate for you to use as the EF falls.

You have a great team at NEMC who will help you find your way through the process of changing your treatment.

Take care,
Lisa