Genetic (lamin A/C) Dilated Cardiomyopathy

[Giorg]

I am 30 years old, I was diagnosed of DCM about 4 years ago with an EF of 17% and, after a few months, with a VO2max of 19.
My mother and my uncle and were transplanted more then 10 years ago. They are doing fine.

With betablockers, ACE, diuretics and aldactone in one year and a half my EF went up to 45% and my VO2max to 37: I felt good.  The size of my heart decreased from 6,5 cm (diameter) to 5,5 cm.
Lately my EF is down again to 30% and I am waiting for a stress test next week. My heart size is aroung 6 cm now. I do not feel as good as I used too: I have many bad days, at least one per week and I have to take diuretics (25 or 50 mg per day) that I had stop when my EF got to 45%. It is very tough to work for me in some days, even if I work in a office.

Three months ago doctors (in Pavia, Italy) found a defect in my family's DNA in the lamin A/C gene: they told me that defect usually brings a poor prognosis and many arrhythmias. Luckily three years ago I had an ICD implanted... so I am quite safe on the arrhytmias aspect. I do not tolarate Amiodarone, so as arrhytmias were increasing I had to switch from Coreg to Sotalol
My therapy is 200 mg of Sotalol, 10 mg of Triatec (an ACE), 25/50 mg of Lasix, 25 mg of Aldactone, 2 mg of L-Carnitine, 1 g of fish oil.

My questions:
1) Is there any hope that my EF will go up again?
2) Do you know something abot lamin A/C caused DCM? Is there any specific therapy?
3) Do you use Sotalol instead of Carvedilol in DCM with many arrhytmias?
4) Why is it that even with diuretics now I tend to retain fluid? Ef of 30% is so low

[CCF-M.D.-MJM]

Hi Giorg,

It is tough sometimes to predict how cardiomyopathies will respond to medications, but I will try to answer your questions.

1) Is there any hope that my EF will go up again?

Very tough to say.  There is some variability in interpretations of echocardiographic ejection fractions (if this was by echo and not nuclear).  There is always a chance they over estimated a few years ago and it hasn't gone done as much (numerically).  The important part is how you feel and it sounds like you are not doing as well.  Certainly I would continue on the medications -- it sounds like a good regimen.  Only time will tell about your response to medications.  Any thought about a biventricular pacemaker??  I know you didn't discuss the extent of your symptoms and your ekg, but it may help.  ask your doctor.

2) Do you know something abot lamin A/C caused DCM? Is there any specific therapy?

As of yet, there is not specific therapy for any genetic cardiomyopathy.  The standard care is essentially the same for all cardiomyopathies and you are already on the right medications.

3) Do you use Sotalol instead of Carvedilol in DCM with many arrhytmias?

If you have significant ventricular arrhythmias and do not tolerate amiodarone, sotalol is a good choice.  I would prefer ot use carvedilol if I could, but ventricular arrhythmias sometimes limit this.

4) Why is it that even with diuretics now I tend to retain fluid? Ef of 30% is so low?

Are you restricting salt?  You may need some adjustments to your diuretics, a biventricular pacemaker/icd may help, but it is also possible that your cardiomyopathy is progressing and causing the edema.  It may take some time to adjust your diuretics.  I would consider talking to a transplant specialist or a transplant center.  If two family members  required transplant, you may eventually need one as well.  They may require you to stop smoking, drinking, check family support structure....it is always good to have these things in line in case you need a transplant.

I hope this helps and good luck!

[DottyCece]

I sympathize with you.  I had an EF of 30% and felt awful.  Retaining fluid is a constant battle for me.  I take demadex which is like lasix and vary the dosage based upon my weight...so I have been better lately.  Hope you do better and you are able to get on the right track.  From what I know, taking the coreg and ace inhibitor  will improve the prognosis.

[lak]

I also have a terrible time with fluid retention.  I am on hyzzar 50 12.5 mlg once a day.  From what I understand the diuretic is built into my blood pressure medicine(hyzaar).  I have alot of arthymias, bigemy, trigemy, some runs of sinus tachy and alot of PVC's. I feel so much better if I take two of the hyzaar one in the morning and one at night.  I don't do it very often because I don't know if it is safe.  I am 46 and weigh 170.  I wonder what mlg other people take.  If taking two of the hyzaar would deem safe?  Or what could the harm be?  Can anyone tell me how many miligrams they take of blood pressure meds??

[tickertock]

Hyzaar is basically Cozaar and Esidrex combined.The drug in cozaar is losartan(a ARB) and the drug in esidrex is hydrochlorothiazide(a diuretic or so called water pill). The maximum dose for cozaar is usually 100mg daily and the standard dose of esidrex is usually 25mg daily, so that would equal 1 hyzaar 2 times daily of the dose you are prescribed.. You might need the higher dose to work for you. Talk with your doctor, always check with your doctor before lowering, stopping or upping the dose of your medicine.

[tickertock]

just want to add you are only on hyzaar once daily, that is equivalent to losartan 50mg and hydrochlorothiazide 12.5mg. daily, just wanted make that very clear.Don't do anthing with your medicine without checking with your doctor first.

[mmfd]

Hi.  I just wanted to say that, for me, I still really have to watch the sodium intake and keep my fluid intake under 64 oz. a day.  Otherwise I retain fluid right away.  And my EF is now 45%, up from 15%.  I was told to keep the sodium under 2000 mg./day, although some CHF'ers swear you need to stay under 1500/day.  The best way that I found to do it is to get in the habit of reading all labels of any food I eat that isn't fresh.  It really isn't that hard to do....you do get used to it.  Have you asked your doctor about your Lasix dose??  Maybe he would up the dose some.  Also, do you weigh every morning, and report a weight gain of 2-3 pounds overnight??  This is what I was told to do to catch fluid gain early.

[Giorg]

how much lasix do you take? I am supposed to take 25 mg per day, but I see it is not enough and so almost always I take 50 mg (two pills in the morning after breakfast).
I already watch sodium intake and drink less then 64 oz. Included soup, and pasta and fruits usually I drink about 48 oz per day. During weekend nights I go out and drink a little more, I eat slightly more salty and I can see weight going up and I feel more tired... Can you work?

[mmfd]

No, I still can't work, although I would like to find a little part time job.  I'm on SSD.  I take 80 mg. of Lasix.  Here in the states it doesn't come in 25 or 50 mg. tablets. *Watching the sodium* may not be enough for you, especially if you eat soup that isn't homemade, as it is LOADED with salt!  You might need to be a lot more strict with it.  Also, alcohol in any quantity, I've been told, is not recommended for anyone with cardiomyopathy or on drugs for it, as it can destroy heart muscle.  I will say that my sister who has mild DCM found a doctor who lets her drink a glass of wine or two!

[Giorg]

I have just fresh food, and I cook it by myself without adding any salt.I eat at restaurant at lunch almost everyday but I have just grilled meat with no salt.I do not drink alcholics, except for a beer every now and then.More then one cardilogist told me that less then a beer a day can not hurt me.I do not think there is evidence that a very small quantity of alchol can hurt us.Maybe two glasses of wine per day are too much...but I think a little glass of beer is like nothing.I see my friends drinking in pubs,I surely do not follow them: I just have a lemon soda or a coke.
Except for the months just after the diagnosis I have always been working but as my EF decreases....it is getting tougher. I guess that as long as one is diuretic dependent working is tough.I would like a part time job, but I do not think I would get enough money: better to have a full time job and take some days off once in a while (days off because of sickness are full paid in Italy). In Italy we have a SSD only for class IV patient, and it is just a little money. But we do not pay medications, that is great. I do not know, my job is a great concern... I am seeing how my disease evolves...then I will decide about it.

[sigprn]

new medications are not necessarily better...Vasotec,lasix,inderal are effective in treating dialated cardiomyopathy.Also they come in generics.