New Focus on 'Sudden Death' Heart Disorder
An abrupt, fatal heart attack in a young athlete on the playing field is a tragedy destined to repeat itself over and over until more is understood about hypertrophic cardiomyopathy (HCM), a genetic disorder that is the most common cause of sudden death in young people but which affects people of all ages
So says a task force of cardiologists and cardiac biologists, headed by Thomas L. Force, M.D., James C. Wilson Professor of Medicine at Thomas Jefferson University, in the Sept. 14 online edition of the journal Circulation.
Their special report is the culmination of a 1.5-year effort to sum up the relatively little that is known, and much that remains a mystery, about HCM, and to list what future research priorities should be -- all with a goal of developing novel treatments. All 21 researcher-physicians, from institutions around the country, participated in an HCM working group convened by the National Heart, Lung, and Blood Institute. HCM is believed to affect 1 in 500 people, yet without a detailed family and genetic history, many people may not know they are at risk for sudden death, says Dr. Force, who, as a nationally-known cardiology investigator, had led a number of symposiums and study groups to focus on causes and novel therapies for patients with sickened hearts.
"Unbelievably to me, this problem is still not understood or even known to exist by many people, and it remains a very challenging disease to treat," he says. "The medical management of HCM has changed very little over the past decades."
HCM, a thickening of the heart muscle which makes it more difficult for it to pump blood, often manifests itself when it's too late, he says. "The reason it can be deadly is because people with the disease are often unaware that they have it and physical exertion -- such as sports -- can bring on the sudden, fatal series of events that causes the heart to go into arrest," Dr. Force says.
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