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The Not-So-Tell-Tale Heart 2007-06-03
By Gretchen Reynolds

The Not-So-Tell-Tale Heart

Sue Cox says that her 17-year-old son, Tim, “started playing sports practically in the womb.” A standout on junior-league basketball and Pop Warner football teams in Hudson, N.H., he was, by high school, among the top basketball players in the state as well as a starting safety on the football team. “I lived for sports, you could say,” Tim says.

 
 

But could he also have died from them? Last July, at his parents’ insistence, Cox had an electrocardiogram, which measures electrical activity in the heart. It’s the same test his father had 15 years ago, after experiencing some shortness of breath. That EKG and follow-up tests showed hypertrophic cardiomyopathy (HCM), a thickening of the heart muscle. Cox’s grandfather had been diagnosed with the same condition and died at age 63. But Cox, with the insouciance of youth, was sure that his own heart was fine. He was therefore stunned when the test came back positive. He would never be allowed to play competitive sports again, his cardiologist told him flatly. “It was a terrible time,” says Cox’s father, also named Tim. “We wanted to keep our son alive, but to do that, we were taking away everything that he loved most in life.”

Athletes, particularly young athletes, don’t worry too much about dropping dead from heart failure. But it happens. The 1990 death of the Loyola Marymount University basketball star Hank Gathers, who had been diagnosed with cardiac problems after a fainting episode but was allowed to continue to play, made headlines around the country. So did the deaths of 27-year-old Reggie Lewis of the Boston Celtics in 1993, and the 28-year-old ice-skating Olympic gold medalist Sergei Grinkov in 1995. In 2005, Atlanta Hawks center Jason Collier died at 28 of an undiagnosed heart abnormality; that same year, three N.C.A.A. basketball players also died. Just three months ago, in February, Damien Nash of the Denver Broncos died not long after playing in a charity basketball game. By sad coincidence, the game was to raise money for heart-transplant research — Nash’s older brother had had a transplant, after passing out during a basketball game.

Though exercise-induced sudden death most commonly affects recreational athletes over 35, cardiologists are realizing that more young people die of heart failure than was previously thought. “We used to see figures saying there were 20 cases or so a year,” says Barry Maron, the director of the Hypertrophic Cardiomyopathy Center at the Minneapolis Heart Institute Foundation. But a national database that Maron has helped compile suggests a much higher prevalence. “We’ve documented around 125 cases a year of sudden cardiac death among young competitive athletes,” he says. “That represents about one death every three days in the U.S. And that number is almost certainly an underestimate.”

The incidence is alarming enough that this season the N.B.A. began mandating echocardiograms (ultrasounds of the heart) for all of its players, along with electrocardiograms. The International Olympic Committee recently began recommending EKG’s for its athletes. And the European Society of Cardiology issued a report in 2005 calling for all European athletes, including teenagers, to pass an EKG before being cleared to play. (EKG’s are generally cheaper and easier than echocardiograms and so are the test of first resort. Since each test provides different information, some sports organizations think it’s safest to have both.)

In the United States, the push for mandatory cardiac testing of athletes has been slower and much more contentious. In March, the American Heart Association issued its first new guidelines in a decade for the cardiac screening of competitive athletes. The conclusion was that requiring EKG’s would be expensive — costing about $500 million nationwide, not all of which would necessarily be covered by insurance — and result in too many false readings.

Cardiac arrest in those under 35 typically results from an undiagnosed heart abnormality. The most common — accounting for about one-third of all sudden cardiac deaths in young competitive athletes in the United States — is HCM, in which the heart muscle thickens, leading to possible disruptions in the heart rhythm. There may be a known family history, as in the Cox family, or there may not be. It can be accompanied by symptoms, like chest pain or shortness of breath, but isn’t always. Even if it is, an athlete might not recognize the symptoms. As Tim Cox says: “I was running up and down the basketball court all the time. Of course I had shortness of breath. We all did.”

Other frequent causes of sudden death due to heart failure include Marfan syndrome, a disorder affecting the body’s connective tissue (Marfan is commonly found among the tall and lanky — some believe that Abraham Lincoln had it); and Long QT syndrome, in which heart rhythm can suddenly become erratic. Again, these conditions may or may not cause obvious cardiac symptoms.

Doctors are not absolutely sure why heavy exertion sets off arrhythmias in people with these conditions, but it does. “A child with HCM could, of course, die in the library,” says Paul D. Thompson, the director of cardiology at Hartford Hospital in Connecticut and one of the authors of the new American Heart Association guidelines, “but they tend to die out on the playing field.”

As the poets say, the heart is a thing of mystery. Some of these cardiac abnormalities, particularly HCM, can be almost indistinguishable from robust good heath on an EKG or echocardiogram. In HCM, the walls of the left ventricle grow thicker. The same thing can happen to an extremely fit athlete. This can be part of a benign condition known as “athlete’s heart,” which is often typified by an increase in the overall size of the heart’s chambers and a slowing of the heart rate, sometimes to 40 beats per minute. “It’s a normal physiological adaptation to sustained, heavy exercise,” says Adolph M. Hutter Jr., a professor of cardiology at Harvard Medical School and the team cardiologist for the Boston Bruins and the New England Patriots. Hutter points out that about half of the EKG’s of well-trained athletes look unusual when in fact there is nothing wrong.


 
 
 
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