Question: Difference between PPCM and other Cardiomyopathies

[SarahG]

Dr. Fett - Can you clarify something for me? What is the difference between PPCM and the cardiomyopathies you hear about athletes dropping dead from? I often hear of athletes in prime condition dropping dead and it turning out to be a cardiomyopathy... is there any known risk of someone with PPCM just dropping dead - especially if being treated and having no symptoms other than PVCs?
I'm feeling perfectly fine but my EF is hovering at 45-50 (depending on who reads the Echo - one reading was 50-55, another was 40-45). I was diagnosed last June at 45-50% and have been on Toprol XL and Enalapril since then. Since DX I have had NO symptoms other than PVCs, so no outward reason to believe I'm in any danger, but I get nervous when anyone mentions those athletes.

I think I'm starting to sound a bit paranoid, but it's so scary when there's a problem involving the heart. I have an appointment with a new Cardiologist (moved to a new county in CA) on April 4th, but I'd love to get your opinion beforehand.

Thank you again for all of your time and support.

Sarah

[JAMESFETT]

Thanks for posting. I'm glad for your questions, they are very important for most unrecovered PPCM'ers.

The unique thing about PPCM is its appearance during or after pregnancy. Otherwise, it seems very much like a dilated cardiomyopathy that began with a viral infection. Whenever there is an unexplained dilated cardiomyopathy, there are many different causes that need to be ruled out, including amniotic fluid embolism, severe anemia, beriberi heart disease or other nutritional deficiencies, familial dilated cardiomyopathy, acute fluid overload, hypertensive cardiovascular disease, hypertrophic cardiomyoathy, ischemic heart disease, sepsis, thyrotoxicosis or other endocrine disorders, tocolytic agents or other toxic exposures, tuberculosis or other lung diseases, severe toxemia of pregnancy, a variety of infections, and valvlar heart disease. I go through a check list like this with every new possible PPCM patient and try to make sure none of these is the cause for the heart failure.

I assume that you do not have any of those conditions, so that leaves you with the diagnosis of indeed having PPCM. Right now, the question is, "Why aren't you back to normal, or above 50 % LV EF (even though 40 to 45 represents a good improvement from originally)? Again, your situation is unique, but in similar situations I review everything to make sure there is not a missing link. In such situations the question always comes up about additional evaluations, which COULD include:
1)blood high sensitivity CRP test (about $30), to see if the level is above 10 mg/liter, indicating ongoing inflammatory process, maybe in the heart.
2)blood cardiac troponin (about $30) to see if the level is above normal, indicating some inflammatory process in the heart.
3)blood BNP (about $30), to see if the level is above the "cut-off," indicating continuing stress on the left ventricle. This also serves as a baseliine value for future reference when one may wish to repeat the test.
4)cardiac MRI with gadolinium contrast (about $1500) to see if there is evidence of scarring and/or inflammation in the heart, and to have a better idea if an endomyocardial biopsy could give some answers (such as enhancement in the septal area, where the endomyocardial biopsy is usually taken).
5)cardiac catheterization (about $3000) with endomyocardial biopsy, to include "quick-freeze" tissue for PCR testing for presence or absence of viral particles.

After those considerations, the question becomes "Is there room to change or increase medications in order to achieve better function?" I don't know the dosage level of your metoprolol long-acting or enalopril. Possibly you are not yet at maximum? It may also be worth changing your metoprolol to carvedilol, which possibly has some advantages, [in research some articles suggest extra benefit from carvedilol over other beta-blockers in healing effects mediated through the immune system to correct imbalance or dysfunction] particularly if dosage level can be gradually worked up to 25 mg twice daily. The enalopril max is considered 20 mg twice daily.

The greatest danger of sudden death (from ventricular tachyarrhythmia) is in early stages of PPCM, when the LV EF is down in the 25 percent or lower range. After that, with the treatment of ACE-I and B-B, that would be very UNLIKELY to happen, always making sure that electrolytes are in balance. In the very early stages, when LV EF is <25 %, consideration can be given to an ICD (implantable cardioverter/defibrillator) to deal with ventricular tachyarrhythmias. I emphasize that is NOT for you, because you are thankfully beyond that stage.

I hope that helps and wish you ongoing progress.

JD

[SarahG]

Dr. Fett - I don't know how to thank you. You have made me feel so much better... AGAIN... I will bring up what you said at my next appointment and in the meantime, I will try to relax and focus on how much better I am and not what could be or what could have been!

Thank you again,
Sarah :O)

[mlfranklin]

Dr. Fett,

I was reading this post and sudden cardiac death is also the thing that I have heard about that scares me very much. I can't shake the feeling that since my EF has been diagnosed at 25-30% that I am at risk for this. This causes a lot of anxiety for me that mostly at nighttime I cannot shake the idea that it could happen. I know you say that EF BELOW 25% is the risk zone....however how much different is that from 25-30? I mean, when cardiologists read echo's I have been told it is up to conjecture and everyone would say it is different. How do I know if I am closer to 25 or 30? I know in the beginning of the diagnosis that my Dr. said I had "funky stuff" going on with my heart sounds....he later said that it was the 3rd heartbeat sound associated with PPCM and that later he didn't hear it....I have also never gotten an irregular EKG...would it be safe to assume that I am in a safe zone at the moment? If I am...how fast can that turn around? i am still on low dosages of my meds so I am hoping we can start increasing those at a faster rate....

[JAMESFETT]

You do have cause to be concerned. Otherwise, I would not mention it to you. But the problem is that we have lost new PPCM mothers because of the rhythm problems. I would always take the precaution of using a Life Vest initially, until it is certain that the LVEF has gone above 25 %. If the low levels continue after 4 months post-diagnosis, then consideration should be given to an implantable cardioverter defibrillator (ICD).Are you close to Chicago? Dr. Joan Briller will be glad to see you soon, and then she can help you to deal with this issue, as well as to provide the best guidance to increase the BB as needed and tolerated. The Beta-Blocker helps because it raises the threshold for ventricular tachyarrhythmias, which means to say that the BB helps to prevent those rhythms. I worry about you until I am sure you are in a "safe" zone. If you are currently on carvedilol 3.25 mg twice daily, that is good, but I wish you could tolerate higher. How can I be of help?

[mlfranklin]

Dr. Fett.

Currently I am on the 6.25 mg of Carvedilol, I am about 3 hrs away from Chicago, so its for sure somewhere I could go once but not somewhere I could visit on a regular basis. I live mideast Indiana. I am concerned that this is something that my cardiologist hasn't mentioned to me. I would feel better having something around me to use in case of an emergency. I am praying so hard that my next echo will show improvement. i am tolerating the current levels of carvedilol and diovan very well. Of course the Diovan is only 80 (the cardiologist originally wanted me on 160) I still have the 160 perscription and pills. SHould I call me Dr. to see if he wants me to up those back to the original prescription?

Thank you for being honest. You are the first person to tell me to be concerned on this. I am tired of hearing "I know what I am doing, you just sit back and relax." I will probably call my cardiologist tomorrow.

Thank you, if there is any way I can send results or speak to someone over the phone instead of driving to CHicago on a regular basis this is something doable for me....

Melissa

[JAMESFETT]

Perhaps you could e-mail Dr. Briller? You would not need to go multiple times, she can help outline some recommendations that your cardiologist could collaborate with. Are you closer to Detroit or Cleveland? I am glad your dosage of carvedilol is now higher, is that 6.25 mg twice daily?. I think you may need higher dosage of Diovan, but one at a time, it is equally or more important to increase the BB. Of course need to work it out with cardio. Here are contact numbers/info for Dr. Biller:

University of Illinois, DOM Thurmond Faith M Coordinator faithmnd@uic.edu 312-996-2531Office 312-413-2948 FAX Dr. Briller Joan briller@uic.edu

[mlfranklin]

yes I am at 6.25 twice a day :) I spoke with my cardiologist's nurse to find out why a life vest hasn't been mentioned yet to me, she said that Dr. Nassar did not want to overwhelm me with too much at one time, and that I hadn't been on the meds long enough to get that through the system or insurance. SHe did say that if at my March 10th appt that I did not improve that would be something he would talk to me about. she also said that they like to increase the dosages a month at a time. She was pleased that I have felt so good and encouraged me to talk to whomever I needed when I was feeling anxious. She also said that until I improve or get an IUD ? that I could ease my anxiety by getting one of those "panic button" necklaces....because I do spend a lot of time by myself with the 2 babies. I will contact Dr. Briller, I like the idea of having a 2nd opinion and teammate on my side! Thank you so much!

Oh, and I will have to check how far away I am from the other 2 cities...I think Chicago is closest....

[JAMESFETT]

Most importantly, one must be protected against the undesirable side effects, and SCD (sudden cardiac death) is one of those, especially in the early phases, first several weeks, and especially when the LVEF 25 % or less. If your LVEF goes above 30 %, that becomes much less risk, and the beta-blocker is important to protect against that. Best wishes,