(Journal of Cardiology, 2008) Beneficial effects of beta-blockers and angiotensin-converting enzyme inhibitors in Duchenne muscular dystrophy

Hitoko Ogata (MD, FJCC), Yuka Ishikawa (MD), Yukitoshi Ishikawa (MD), Ryoji Minami (MD) - Japan

Background: Patients with Duchenne muscular dystrophy (DMD) often have severe heart failure with a high mortality rate. Most DMD patients with cardiomyopathy became symptomatic in their early to middle teens and usually die of congestive heart failure within 2—3 years from the onset of symptoms. It has been reported that the combination of an angiotensin-converting enzyme (ACE) inhibitor and a betablocker has additive benefits in patients with heart failure. The aim of this study was to assess whether the combination of an ACE inhibitor and a beta-blocker is associated with long-term survival of DMD patients with left ventricular (LV) dysfunction. Methods: We retrospectively analyzed the outcomes of 52 DMD patients who had begun treatment for heart failure with an ACE inhibitor and a beta-blocker at National Yakumo Hospital during the period from 1992 to 2005. All patients used wheelchairs in their daily lives. Patients were classified as symptomatic or asymptomatic at the initiation of treatment with these two drugs. Twelve patients who had already had apparent symptoms due to heart failure were enrolled in a treatment group. Forty patients who had no symptoms with reduced LV ejection fraction (≤45% in echocardiography) were enrolled in a prevention group. Results: Five-year and 7-year survival rates of all patients were 93 and 84%, respectively. In the treatment group, 5-year and 7-year survival rate were 81 and 71%, respectively. Survival rate became zero at 10.9 years. In the prevention group, 5-year and 7-year survival rates were 97 and 84%, respectively, and 10-year survival rate was 72%. Nine patients in the prevention group remained event-free over 10 years.

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what medicines come under beta blockers? Does this means ace-inhibitors help when you start early?
What are ACE inhibitors, and how do they work?

Angiotensin II is a very potent chemical that causes the muscles surrounding blood vessels to contract, thereby narrowing the vessels. The narrowing of the vessels increases the pressure within the vessels causing high blood pressure (hypertension). Angiotensin II is formed from angiotensin I in the blood by the enzyme angiotensin converting enzyme (ACE). ACE inhibitors are medications that slow (inhibit) the activity of the enzyme ACE, which decreases the production of angiotensin II. As a result, the blood vessels enlarge or dilate, and blood pressure is reduced. This lower blood pressure makes it easier for the heart to pump blood and can improve the function of a failing heart. In addition, the progression of kidney disease due to high blood pressure or diabetes is slowed.

Types of ace inhibitors

Captopril, vasotec ( enalapril), lisinopril, perindopril, accupril

What are Beta- Blockers and how do they work?

Beta-Blockers reduce the workload on the heart and help it to beat more regularly. Beta- blockers control, but does not cure, high blood pressure ( hypertension). Beta- blockers can also relieve chest pain ( angina).

Types of beta- blockers

metroprolol ( lopressor, toprol XL)


Our son's cardiologist said he wouldn't prescribe any cardiac drug for our son until he was about 9 or 10 years old. Every year he has a echocardiogram and EKG done to look at the condition, function, and rhythm of his heart. His results so far have returned benign but I would imagine if there were any issues, he would be placed on a cardiac drug.
Thanks Tina.

I recently requested Dr.Wongs's team (Dr Cripe) re. starting Losartin. She also refused to prescribe and would only start ace inhibitors when the echo starts showing abnormalities. I can not help but wonder if starting early would help to keep heart normal for a long time...

-bain
Tina - are you going to push your cardiologist, given the results from this study?

Also - does anyone have their sons on beta blockers? What are the side effects from these drugs?

Lastly, any opinions on whether ARB inhibitors would work as well as ACE inhibitors?

THanks.
My son was on Enalapril. He started taking it when he was 12. Kory was on a low dose for 4 years before he passed away from respiratory failure. Kory's BP was extremely low while he took the medicine. They even had a hard time getting his BP. His pulse ran on the high side. This was a concern of mine but the cardiologist was not concerned when I brought it to her attention. She did up Kory's dose one time but Kory's BP completely bottomed out and he became dizzy. She immediately dropped him back down to the lower dose. His heart function showed gradual improvement throughout those 4 years. I suggested a beta blocker during this time because of his high pulse the cardiologist never wanted to try it for some reason.
Isn't Cozaar/Losartan in this catagory?
My son who will be 15 in Feb. went to the cardiologist today, last appt. a year ago. Since he has been followed everything has been ok, ejection fraction of 55. On Lisinopril 5mg daily prophylactically. Today we were quite surprised to see such changes, ejection fraction of 34 % and some cardiomyopathy. Cardiologist wants to see him again in 6 months but hesitant to put him on anything, also his BP is elevated. I myself am a nurse, but cardiology is not my specialty. Is anyone else on the site have some insite/experience?
My son (14 years old) recently started on a beta blocker. His heart rate was elevated so he wore a 24 hour monitor to get his average heart rate.The cardiologist said that he wanted him to start on a beta blocker if his average heart rate was over 100 beats per minute. We have not seen any side effects. He also takes an ace inhibitor.
Our son's cardiologist prescribed an ACE inhibitor for our son at his cardio appt. a few months ago ( Matt wasn't quite 6 yet), He said that they are now wanting to start the inhibitor before the boys exhibit heart problems. It's supposed to make the work load on the heart easier. He also said the down side was a lowered blood pressure that could make our little guy a little more tired (like he needs that, right?) We hadn't started the drug yet, as we were waiting until after he had his tonsils and adenoids removed. Now that that surgery is over, we were getting ready to start. Now I'm slightly hesitant knowing that Dr. Wong wouldn't prescribe. Is there anyone else using an ACE inhibitor at 6? Since all the patients in this study used wheelchairs in their daily lives, it would seem that they may be a few years older than my guy. But, I guess the point is that you want to start before the onset of cardiomyapathy, and you can't really predict when that will be for each child. Now I'm not sure what to do.
Danelle,
My son was 5 when he was started on an ACE Inhibitor (Enalapril) for the same reasoning as your cardiologist gave. He did not have any heart problems then and does not still. He will be 8 in Feb. I have seen no negative side effects. We go to Cinc. and was told it is their policy to not discontinue any heart meds that have been started somewhere else unless of course the parents want to. Given the cardiomiopathy that is almost certain to develope, it made sense to me to start them as early as possible and NOT after heart problems had already developed..
Karen
I just wanted to add that the cardiologist that started him on this drug told me that there was no harm in starting it - even at his young age. He was prescribed 25mg/day.
Thanks, Karen,
That does make me feel better about it.

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