WebMD Medical News
Brenda Goodman, MA
Laura J. Martin, MD
Nov. 8, 2011 -- When an athlete dies suddenly on the court or playing field, often an undiagnosed heart condition called hypertrophic cardiomyopathy is the cause.
Now new guidelines aim to shine a light on the diagnosis and treatment of hypertrophic cardiomyopathy (HCM), a disorder that affects an estimated 600,000 Americans.
"It is an important cause of sudden cardiac death during competitive sport, and from that standpoint perhaps is brought to the headlines more frequently,"says Bernard J. Gersh, MD, PhD, who co-chaired the committee that wrote the new guidelines.
"It’s one of the commonest inherited cardiac diseases, and it probably hasn’t had the attention it’s deserved. I think if you talk to people out there there’s a great deal of confusion about how to treat this condition," says Gersh, who is a professor of medicine at the Mayo Clinic in Rochester, Minn.
In HCM, the muscular walls of the heart thicken abnormally, making it harder for the lower chambers to fill and pump blood.
Misaligned muscle fibers can also cause electrical abnormalities that can cause heart rhythm problems.
"Those electrical abnormalities can cause someone to drop dead," says Tara Narula, MD, a cardiologist at Lenox Hill Hospital in New York City.
Symptoms of the condition may include chest pain, shortness of breath, fainting and dizziness during exercise, heart palpitations, high blood pressure, and rarely, heart failure.
In many cases, though, there may be no warning signs, and the condition is discovered during an autopsy.
Doctors want people to know that having HCM isn’t a death sentence. In fact, most people with the condition will have a normal life span.
"This disease, once, not that long ago, regarded as mysterious and almost universally associated with poor outcomes is now a highly treatable genetic heart disease,” says Barry Maron, MD, director of the Hypertrophic Cardiomyopathy Center at the Minneapolis Heart Institute Foundation. Maron co-chaired the committee that developed the recommendations.
The guidelines are jointly published by the American Heart Association and the American College of Cardiology Foundation. They are published in the journal Circulation.
Among the key recommendations:
Cardiologists welcomed the new guidelines, saying they would improve the care of people with HCM.
"This can only be helpful. The fact that this is laying out very clearly not only what drugs are good but how to screen for it, that patients should be genetically counseled -- all of the different aspects -- they really are pinning down" what should be targeted by a cardiologist, Narula says.
SOURCES:Gersh, B. Circulation, Dec. 13, 2011.Bernard J. Gersh, MD, PhD, professor of medicine, Mayo Clinic, Rochester, Minn.Tara Narula, MD, cardiologist, Lenox Hill Hospital, New York City.Barry Maron, MD, director, Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minn.
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