echo and ejection fraction

[grace77]

I am a 40 yr old female recenly dx'ed with mitochondrial myoapthy. It has affected my respiratory muscles,arms,legs etc. I have just started using a bipap machine at night. This disease can affect the heart either by a conduction disorder or by a cardiomyopathy. 5 yrs ago, I had a nl echo. Because of my recent dx, I have seen a cardiologist for evaluation . My ekg was nl but the echo showed mild lt atrial enlargement, decreased lt systolic function with an ef of 45 % . Trace aortic and mitral valve regurgitation. 5 yrs ago, my ef was >55%. My question is: do you think this could represent a possible early or developing cardiomyopathy as a result of the mitochondrial myoapthy? My cardiologist recommends ekg's q3mos and echo's q6-12 mos. I know 45% is not a horrible ef but it still concerns me. Also, why would the lt atrium be enlarged and what does this mean and could this get worse? DO you think adding a betablocker at some point would be helpful or at what point with your pt's do you get concerned and add medication etc? I do have tachycardia with mild exertion and dyspnea and mild ankle and lower extremity edema that is more noticable after being on feet . Could this be related to above? Thanks for your help.

[Cleveland Clinic]

grace,

A lot of the inherited myopathic disease have a cardiac component to them.  It would be helpful for you to characterize your disease fully and I would recommend for you to be evaluated at a center that specializes in these types of disorders.

It will be important to follow your disease closely as it sounds like your physicians are doing.  In all comers with cardiomyopathy certain therapies such as ace inhibitors and beta blockers have been shown to be helpful.  Its hard to tell if a specific individual will respond, but generally if you have documented cardiac dysfunction I have a low threshold for instuting these types of medications.

It is also paramount to control your blood pressure, choelsterol and maintain your physical fitness level to ensure you have no other causes that can possibly negatively affect your cardiac function.

Your symptoms are somewhat nonspecific and could be attibuted to the disease itself in additon to abnormal cardiac fucntion.  This point does highlight the importance of contrlling the risk factors that I mentioned above.

good luck.

[Momto3]

I went through a similar situation a couple of years ago.  Decreasing EF, swelling in my ankles, SOB and one or two EKGs suggested an enlarging atrium.  In my case it turned out to be a result of very frequent episodes of tachycardia (NSVT).  At first, it was speculated that the changes could be a result of an increase in mitral regurgitation.  I do have MR, but it was ruled out as a cause.  I noticed that you mentioned tachycardia?  Any possibility that could be a factor?  Just wondering.  Oh, my EF was also normal prior to these changes.  After 2 ablations in 2003, I've experienced increasing EF, no swelling and major decrease in SOB.  I do take an ACE inhibitor for BP control.  It is not high, but it is best for me to keep it an an optimum level.  A beta blocker was considered, but b/c of the great results thus far, I have not had to resume taking beta blocker.  

Good luck and keep us posted.

Connie