Medication for severe scarring

[Bob in Texas]

Hello
I recently returned from Tuft's and was diagnosed with HCM non obstructive with severe diastolic dysfunction and scarring. I have been prescribed Nadolol, Verapmalil and I am in a research study with Spironolactone/ placebo. My symptoms have not gotten any better after a month already. They also placed an ICD/Pacer in me. Dr. Maron has been outstanding and continues to followup with me since my return to Texas.

Is there anyone out there that has been diagnosed with non obstruction with severe fibrosis? If so what type of symptoms have you been getting? Has the medicine helped? Just looking for a little support from someone who might be on the same boat as me. Any responses are appreciated.

Bob

[macbeth]

My son and daughter (ages 23 and 21) have non-obstructive HSM with severe scarring and LV dysfunction. Both have ICD/Pacemakers. Both have been taking Spironolactione. One takes Nadolol and Verapamil, the other is on Coreg and a beta-blocker.

They've been on the Spironolactone for about two years. They're seen at the Clevend Clinic, and the doctors there said that the purpose of the spironolactine is to reduce and/or delay the progression of scarring. Based on MRIs and echocardiograms, the Spironolactone has not (for them) had any effect in reducing the scarring or preventing its progression. They are not part of any formal study, they are simply taking the drug.

My understanding is that the effectiveness of the Spironolactonre may depend on the specific gene that causes the HCM in the patient. There is no reason to extrapoltae their results to your case.

One month is much too soon to look for any effect from the Spironolactone. We were told that the benefit of the drug (if any) occurs over a long period of time (six months to a year at least).

If your concern is about the verapamil and Nadolol reducing your symptoms, hang in there, it takes a period of time to be effective. My daughter was very symptomatic when she was first placed on a combination of Nadolol and verapamil. It took the doctors at Cleveland about 6 months to fine-tune the amount of the drugs she was taking. When they were done, she was nearly symptom-free. Her quality of life increased significantly. Nadolol is a beta-bloacker, and verapamil is a calcium channel blocker. It is much more difficult for the doctors to prescribe the two drugs in parallel because of the ways that they interact in your heart. Once the doctors determine the proper doses, they can be very effective.

Also, the drugs build up in your body over a period of several weeks. The effects of changes in doses sometimes take a while to become apparent.

The effects of the spironolactone are essentially hidden. It's unlikely you'll feel any difference from it except over a long period of time.

[Bob in Texas]

Thanks for the response. I quess more people have symptoms from an obstruction. This part puzzles how does scarring just cause symptoms?


Bob

[Pam Alexson]

Bob... you can think of it as ischemia like when a person has coronary vascular disease and suffers an ischemic cardiac attack or coronary or whatever term you use or have heard. The ischemia or scarring is because there was a blockage that caused the tissue to die. That tissue will never come back in its effectiveness, only tissue surrounding the scarring; the non- ischemic tissue will be alive .. virtually that area is very impaired until some re-vascularization occurs... a little restructuring will occur over time. You often here of individuals having repeat heart attacks or dying or needing a transplant... that is because the impaired area functions very less then optimum and often too much damage/ scarring has taken place. AND.. often there are complications as a result w/ the electrical stability of the heart. Much of this starts with coronary arteries and disease --regarding the vessels that lie on the heart.

With HCM... sometimes the same can happen but, inside the heart-- remember the HCM heart has multiple dysfunctions because it is HCM .. damage takes place from wear and tear .... obstructions... heart failure...elevated pressures.. overworking or stressing the heart too much..etc. Over time the heart tries to fix itself often making things worse. In time cell death occurs ( apoptosis). Those dying cells lead to scarring taking place... the muscle becomes fibrous like a tough old piece of meat and it is called replacement fibrosis or scarring.

Now the course of the 2 heart diseases take a slightly different approach but , in the end the same happens, same symptoms, same malfunctions and inefficiency is inefficiency or dysfunction is dysfunction .. however you want to name it. There will be symptoms from the ineffectiveness of a scarred heart no matter what the etiology( origin).

So with medication, device management and different theraputic approaches wrongs are attempted to be made right. Remember the heart with the proper cueing from experts and interventions like medication could just make a remodeling job lalapaloosa and things could settle down.

Hope this helps to explain it.

[Bob in Texas]

Pam,

Thanks, alot going on... you know.... the wheels are always turning. You are a wealth of information.


Bob

[macbeth]

Dittos to everything in Pam's note, just thought I'd add some info based on the varient of HCM that my kids have.

Some forms of HCM are caused by a genetic mutationthat impairs the ability muscle cells in the heart to make structural proteins used in the construction of the heart itself. Our understanding from the cardiologists is that, over time, the damaged proteins build up in the heart and form a fibrous material.

The fibers stiffen the heart walls and make it more difficult for the heart muscle to contract. In turn, this leads to the ischemic damage in the heart mentioned in Pam's note.

In my daughters case, she has so much fiber in her heart that it is difficult for the heart muscle to contract. This leads to her symptoms - shortness of breath, angina, etc..

In her case, the verapamil slows the rate that her heart muscle contracts, and this improves her heart function. The beta-blocker (Nadolol) suppresses some of the arrhythmias that result from electrical pathways in her heart that are caused by the fibers and the ischemic damage.

Our understanding is that the spironolactone was intended to decrease the amount of fibrous material in her heart. It also has an effect on scar tissue resulting from ischemic damage. Apparently some cardiologists believe that if a drug can be found that will reduce the fibrous material in the heart, then the proggression of HCM might be delayed (by delaying ischemic damage and remodelling of the heart).

[Pam Alexson]

I am very hopeful that this process will one day be fine tuned and used for HCM hearts...Pam

Reported July 22, 2009
“Beating Patch” Delivers Healthy Cells to Diseased Hearts



(Ivanhoe Newswire) –What looks like a tiny beating heart is actually a piece of synthetic, gauze-like mesh, barely the size of a fingernail, floating in a Petri dish.

Researchers at The University of Arizona's Sarver Heart Center and the Southern Arizona Veterans Administration Health Care System (SAVAHCS) have come a step closer to repairing hearts damaged by a heart attack or weakened by chronic heart failure.

"We have developed a delivery system that allows us to introduce living, healthy heart muscle cells into damaged areas of the heart in a way that is much more efficient than the conventionally practiced method of injecting cells into heart tissue," study leader Steven Goldman, MD, is quoted as saying.

Dr. Goldman and his team discovered that when they "seed" a vicryl mesh patch with a sufficient number of heart muscle cells (2.5 million or more), the cells start behaving just like their counterparts in the real organ. They contract synchronously at about 70 beats per minute even without any outside stimulation. "Our work shows that we can put living cells onto a biodegradable, 3-dimensional scaffold in a way that not only allows them to survive, but to spontaneously beat in a coordinated fashion," Jordan Lancaster, BS, a pre-doctoral fellow in Dr. Goldman's lab who will present the research at the meeting on July 21, 2009, is quoted as saying.

The group's latest achievements have attracted the attention of the American Heart Association, who picked the research as one of the most noteworthy achievements of this year's Cardiovascular Sciences Annual Conference in Las Vegas, Nevada. "Ultimately, we hope to use our system in patients with chronic heart failure and, possibly, to prevent heart failure in patients who had a heart attack," said Lancaster.

Dr. Goldman believes the construct developed in his lab provides a better vehicle to introduce cells into damaged heart muscle than conventional cell transplantation techniques, in which cells are injected directly into the heart. "I think the main reason for the disappointing results people have seen with those clinical trials is that the cells end up in an environment that is not optimal for them to thrive in. Scar tissue offers poor blood supply and weak structural support for new cells to attach, survive and grow. Our patch offers just what cardiac muscle cells need: structural support, increased blood supply and chemicals secreted by the supporting cells on the patch that help the heart muscle cells grow and function."

SOURCE: Presented at the Cardiovascular Sciences Annual Conference in Las Vegas, Nevada, July 21, 2009

Wan