Treatment options for HCM

[chellonl]

I'm 41 years old, male, diagnosed with heriditary HCM, with an obstruction causing a resting gradient of about 60 (without any medication). Currently i'm on betablockers and will try Norpace. I'd classify my symptoms as medium (class 1 sometimes 2 hardly 3) with occasional AF usually responding well to extra BB's. Though formally diagnosed in 2004, i'm probably affected much longer by (mild) symptoms.

My (Dutch) cardiologist suggests i should get an alcohol ablation. He thinks a myectomy is a not the best treatment option because it's such a heavy operation (note that i'm in good general health, non-smoker etc.). I'm heasitant to go with AA because it appears to have more side effects and less history than M, although i also recognize that M is quite intensive and carries its own risks. Also AA is less recommended at my age.

Now, any advise on how to proceed? I think i have several options, depending on medium term and long term expectancy compared to the risks:

1 Norpace (offsetting the benefits against its sideeffects)
2 M or AA (and which should i prefer, also based on my assumption that in Europe the trend is towards AA and in the USA seems to be (still) in favor of M. AA seems to have improved in recent years, and if the risks are coming down and keep coming down i think it's an attractive option...

How long can i wait before considering further treatment? Does waiting (to long) negatively influence long term expectations? Note that my mother died at 65 from it with burnt-out / endstage symptoms (i'd like to avoid that).

Appreciating input! Ad

[CCF-M.D.-MJM]

Hi Chellonl,

Very interesting question.  You have perceptively picked up on local and national trends to use different treatments (AA vs. M).

There is no head to head comparison of the two treatment modalities.  I can give you some unpublished Cleveland Clinic information but warn that it doesn't apply to most centers.  We do a myectomy operation here different from most institutions.  DRs. Smedira and Lytle are very experienced in myectomies (including doing my grandmothers myectomy just last year -- she was 76).  The classic operation involves taking out a 1 x 1 x 1-2 cm piece of myocardium.  Our approach is much more aggressive and uses interoperative transesophogeal echo to determine in the OR how much is safe to remove.  We have not had any operative mortalities (deaths) related to this procedure.  I favor this approach because it tends to give less or no resting gradient following the procedure.  There are no control trials to show this is better, at this point we just think it is the right thing to do.  We believe AA is an acceptable alternative if myectomy is not available by EXPERIENCED surgeons.

I do not favor medical therapy.  If you are having atrial fibrillation it is because your left ventricular pressures are increased and translating to increased atrial pressures.  The longer you have increased atrial pressure, the more likely you are to stay in atrial fibrillation.  I bet you don't like the way you feel in atrial fibrillation -- now with HCM does.  You need the atrial kick to help fill your ventricle.  Medications may help keep you out of AF for now, but the future may be more difficult to treat.

I am sure you  have discussed a defibrillator with your doctor (if you don't already have one).  If not, make sure it comes up.    There is not enough information above to definitively answer this questions, but it is worth looking into.

If you are ever in the United States (near Cleveland), Dr. Lever and Dr. Thamalarison are our two specialist in HCM.  They can help you with your decisions.  I know Dr. Lever feels very strongly about myectomy over ablation.

I hope this helps answer your questions.  Good luck!

[EchoTech]

The thing with the myectomy is that sometimes the muscle grows back and you are where you started at.  Sometimes it grows back within two years or sooner.  sometimes it doesn't grow back.  Unfortunately, they can 't distinguish between the people who will grow it back and those who will not.  At the center I work at, we use alcohol ablation.  We first see what area is going to die by using an echo contrast. It makes the part of the heart where the contrast is at show up like a bright white on the ultrasound machine while the rest of the heart is it's normal color.  This shows what area would be affected by alcohol.  Sometimes it is not where the coronaries usually go and they have to either move up or down a vessel off of the left main coronary artery.  There have also been cases where an injection would have killed off too large of an area, so a myectomy was performed.
  A defibrillator is a mighty fine idea and should definitely purseued.  Hope this helps a little bit.

[chellonl]

Well i wasn't aware yet that myectomy can be undone in time. Is this the same with ablation or is there a lesser change of the obstruction returning?

[gtojohnny]

I too have HCM and I am 36 yrs old. I have a dual ICD(defibrillator)as a precaution. I have had Alchohol Ablation at UCSF. Soon after the surgery, my gradient was still at 60 at rest. I then had a second episode of A-fib in which I was converted quite easily with Norpase. I was advised of the risk of stroke but, resisted blood thinners because of the constant need for monitoring and adjusting the dose. If I have another episode of A-fib, the doc will insist. I was originally taking Toprol XL and Verapamil. My doc decided to keep me on the Toprol and insert Norpase instead of the Verapamil. The Norpase was to prevent the A-fib, but is also used to treat HCM by some docs. At first, the side effects of the Norpase were unacceptable. We then lowered the dose and I can now tolerate them. I recently went back for annual tests after taking the Norpase for about 6 months. My gradient was down to 17 at rest. My doctor thinks it was a combination of the Norpase and a delayed result of the Alchohol Ablation. I think it was directly related to the Norpase. I have not had any more episodes of a-fib either. My test results are better, and I feel better as long as I am eating right. I have found my symptoms to be closely related to the way I eat. If I eat healthy, wholesome and fresh foods, I feel great. If I eat fatty, salty, and sweet foods, I feel like ****. By the way, I chose the AA instead of the myectomy for several reasons. I didn't want my chest cut open and I didn't want to be laid up for 6-8 weeks. I was back at work in less than a week with AA. As a side note, no mater what your doc says, you will feel better if you keep your weight down and eat right. I cannot stress this enough and the doctor's should stress it more.

[gtojohnny]

As far as the obstruction returning, it can return with ablation as well. Also, they may not kill enough the first time with ablation and have to do it again.