Dialated HCM treatment options and Transplant

[THW]

Hi there,
I'm curious (not for my current condition), what are the treatment options for HCM patients who were once obstructed but are now dialated? And what are the general "requirements" for being put on a transplant list?
Thanks.
Theresa

[NoCrash]

Theresa,

I thought I'd wait and see what everyone (anyone?) else had to say in response to your post, but it appears that the subject is a tough one (or at least "touchy"). Anyway, I pulled together a couple of short extracts from articles on these subjects.

The first extract discusses "non-obstructive HCM" and systolic ventricular dysfunction, although I can speak from experience that end-stage/burnt-out phase HCM with diastolic dysfunction can develop from cases of obstructive HCM. The treatments for end-stage HCM are similar to those for dilated cardiomyopathy (beta-blocker, diuretics, ACE inhibitor, etc.)

The second extract deals with use of exercise testing as a measure of functional capacity as in indicator of the need for a heart transplant. Basically, when someone's capability to uptake and use oxygen falls somewhere below 50% of normal capacity then a transplant becomes a possibility. I posted some links to other information on transplant criteria in another message at the following link.

Message thread: http://forum.4hcm.org/viewtopic.php?t=4752



"American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy", A Report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines


A small but important subgroup of patients with nonobstructive HCM develops systolic ventricular dysfunction and severe heart failure, usually associated with LV remodeling demonstrable as wall thinning and chamber enlargement. This particular evolution of HCM occurs in only about 5% of patients and has been variously known as the “end-stage,” “burnt-out,” or “dilated” phase (7,37,160). Drug treatment strategies in such patients with systolic failure differ substantially from those approaches in HCM patients with typical LVH, nondilated chambers, and preserved systolic function (i.e., involving conversion to after load-reducing agents such as ACE inhibitors or angiotensin-II receptor blockers or diuretics, digitalis, beta-blockers or spironolactone) (Fig. 1). There is no evidence, however, that beta-blockers prevent or convey a benefit to congestive heart failure and ventricular systolic dysfunction of the “end-of-stage” (by contrast with the experience in dilated cardiomyopathy and CAD). Ultimately, patients with end-stage heart failure may become candidates for heart transplantation, and they represent the primary subgroup within the broad disease spectrum of HCM for when this treatment option is considered (207).


The second extract, from http://www.americanheart.org/presenter.jhtml?identifier=1298


"Selection and Treatment of Candidates for Heart Transplantation", 1995

Standardized methods of exercise testing, with and without analysis of respiratory gas exchange, are performed to derive objective measurements of functional capacity. [snip] Measurement of oxygen uptake and the anaerobic threshold during exercise has proved to be an objective, reproducible, safe, and noninvasive method for characterizing cardiac reserve. [snip] Peak exercise oxygen consumption measured during maximal exercise testing (VO2max) provides a measure of functional capacity and cardiovascular reserve in patients with heart failure once maximal therapy has been instituted and maintained. Several studies have suggested that in patients with heart failure, VO2max is a good short-term predictor of mortality and that its deterioration frequently precedes clinical decompensation. Studies in patients with CAD and low LVEF suggest that VO2max measurements also provide long-term prognostic information. ...


Regards,
Rob

[THW]

Rob,
Thanks so much for your response and the information and links you pasted in. I skimmed over some of the articles, but I guess I have alot of reading ahead of me.

But I'm confused, if ANYone wants to jump in..... Are "end stage," "burn-out," "dialated," and "remodeling," all somewhat interchangable, or at least referring to the same condition??? I did read about "End Stage / Burn-out" under the "Complications" heading on the main hcma site, but I still don't quite understand how the 4 terms differ, if they do. I realize I'm showing my ignorance, but I've grown comfortable with doing that here on the board :roll:
Thanks again!
Theresa

[NoCrash]

You're welcome. Some definitions:

End-stage and burnt-out are different names for the same thing and are used interchangeably to describe a type or phase of HCM. There may be individual preferences for using one name or the other but there doesn't seem to be any rule about it.

"Dilated cardiomyopathy" is a form of cardiomyopathy that is different than HCM--they are different diseases of the heart muscle. It is kind of the opposite of HCM; instead of thickened walls, a dilated heart has walls which are "thinned" and the heart's chambers are larger as a result.

"Remodeling" is a process where the heart changes its structure. An HCM heart "remodels" as it transitions into end-stage/burnt-out phase (but not all HCM hearts remodel!)

Regards,
Rob

[Midge Rollins]

Theresa, I am sorry I have not responded. I have had flu for 2 days. I had a myectomy in 1981. My transplant was in 2004. I had diastolic failure. By the last 2 years before my transplant I was diagnosed with Restrictive Cardiomyopathy. Which basically meant medicines will not help your only chance for a survival is a transplant. My heart was so stiff that is would not contract with any form of exertion. How did I get on the list? My oxygenated treadmill test was down to about 12 if I remember correctly. Normal is 22-28 ( I think). Which meant I was not getting enough oxygen to my heart and lungs. The blood test for heart failure kept climbing higher and higher. My physical symptoms of SOB/ tiredness became more intense. I am sure others out there, can add to this and what their experience has been. Remember your best source of reliable information would be your cardiologist. Take care.

[THW]

Thank-you both, Rob and Midge, for the info. I wasn't asking for MY current condition, but I do intend to ask Dr. Lever more about it when I put a call into him next week to ask him other questions about my upcoming myectomy.
Thanks again.
Theresa