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Cardiac Concerns & Care

It is important to remember that your heart is a muscle too. And one should take great care to understand all of the information.

“By the age of 20, almost all patients with Duchenne’s or Becker’s muscular dystrophy have experienced dilated cardiomyopathy, a condition that contributes significantly to their morbidity and their mortality.” so states the article Dilated Cardiomyopathy in Muscular Dystrophy originally published June 2007 by J. Rhodes, R. Margossian, B.T. Darras, S.D.Colan, K.J. Jenkins, T. Geva, and A.J.Powell.

“We are asked frequently about cardiac care and have hosted a workshop on this very topic so that we can begin to understand the issues better.’ says Pat Furlong, PPMD President. “What we do know is that physicians may consider shortening fraction to be abnormal if less than 28% or ejection fraction to be abnormal if less then 50%. An initial approach for adolescents with Duchenne and borderline function, regardless of symptoms, is the ACE inhibitor (but often several are already on the ARB Losartan) followed by a repeat evaluation in 6 months. If still abnormal, discuss adding a beta blocker which generally has a few more side effects but is mostly well tolerated. Beta Blockers should be started slowly and increase based on heart rate and blood pressure. The rationale being that when the heart muscle is weakened, the body invokes a neuro-hormonal cascade of events to support cardiac function leading to increased adrenaline (often evident as elevated heart rate). In the short term that is beneficial. Over the long term it is detrimental. Beta blockers have been shown to prolong life after heart attacks and in adult heart failure. Now, there is data that is Duchenne specific.”

Related links

Dilated Cardiomyopathy in Muscular Dystrophy (download)
Beneficial Effects of Beta-Blockers and ACE Inhibitors in Duchenne (download)
Safety and Efficacy of Carvedilol Therapy (download)
PPMD's Cardiac Initiative

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