As reported by the New Haven Register, May 15, 2005.

Rare Heart Condition Kills Young Athletes

By Abram Katz

If they weren’t high-profile sports stars, chances are no one would notice them dying.

But young athletes do die every year.

The number is low, yet the death of a young, promising, teenager or college student stuns the community and leaves friends and family grieving and wondering why.

And athletes from 12 to 32 will continue to drop without warning because there is no practical way to identify those at risk, doctors say.

Almost all succumb to congenital cardiac and coronary artery abnormalities.

"It’s well known that sudden death in athletes is almost always a heart problem," said Dr. Stuart Zarich, chief of cardiovascular medicine at Bridgeport Hospital.

The first symptom of a problem is often sudden death.

Stanley Myers, 19, a 2003 Register All-State basketball player at Wilbur Cross High, collapsed Tuesday while jogging in Baltimore.

Thirteen-year-old Eboni Johnson McCotter of New Haven died May 6 after finishing basketball practice with the Connecticut Starters, an AAU program.

And there are more, recently including Western Kentucky basketball player Danny Rumph, 21.

The most common cause of death is a problem called hyperthrophic cardiomyopathy, or HCM, cardiologists said.

Though the details of these deaths are generally private, and in some recent cases pending further study, young athletes most commonly die from HCM, said Dr. Peter Schulman, cardiologist and associate professor at the University of Connecticut Medical School.

Affected hearts are enlarged, with unusually thick walls, and the organs contain irregular muscle cells.

The condition affects less than 1 percent of the population and rarely shows symptoms.

This low prevalence means that even the most thorough screening would yield thousands of false positive results for every identified case, at a cost of billions of dollars.

"We cannot prevent these deaths," said Schulman.

"Socially this is a major issue. The victims are in shape, trim, firm and healthy. It’s a shock to people," he said.

"The only solace we can take is that there is a very low probability of sudden death" in young athletes, Schulman said.

Various estimates place the number of victims at one out of 100,000 to 300,000 people a year in the United States.

About 12 high school athletes die annually in the U.S., according to the National Center for Sports Safety.

HCM may reduce the pumping capacity of the heart. More importantly, the muscle is not quite normal, Schulman said.

When a sudden demand is put on the heart, electrical signals that regulate beating veer into chaos, causing irregular rhythms.

The rapidly beating heart descends into a lethal arrhythmia called ventricular fibrillation. Instead of contracting to pump blood the ventricles start to quiver, Schulman said.

Death follows in three to four minutes.

About 6 percent of people with HCM experience fainting, chest pains, shortness of breath or other symptoms.

To diagnose the remaining 94 percent doctors can take a family history, listen to the heart, and order an electrocardiogram and echocardiogram, Zarich said.

The "EKG" measures electrical signals generated by the heart, while an "echo" images the heart with ultrasound.

Schulman said many athletes, especially on the college level, develop physiologically enlarged left ventricles through intense training.

Sometimes HCM is mistaken for healthy enlargement, he said.

"Many of those who have died were screened," he said.

That leaves a careful family history as the best way to identify athletes with HCM.

Meanwhile, HCM is so rare that even a test with 99 percent accuracy would be of little help, Schulman said.

For example, if the 99 percent test is administered to 1 million people, the doctor could expect 10,000 false positive results. This would lead to undue worry and the expense of unnecessary further testing.

Studies suggest that screening the 4 million to 5 million young athletes in the U.S. for cardiac problems would cost $5 billion to $10 billion, he said.

"People think we can always find these patients. There is no practical way to find people with HCM," Schulman said.

Schulman, who helps screen University of Connecticut athletes, said that over the past decade hundreds of students have been screened, and not a single case of HCM has been found.

Zarich, of Bridgeport Hospital, said, "There’s no routine way to screen people. You’ll have a significant miss rate."

One way to address the problem is to position portable defibrillators at athletic events.

"If you can defibrillate within four minutes the person will probably recover. But you must have the equipment, you have to be on the ball, and you have to get the heart beating within four minutes. That’s a lot harder than it seems," Zarich said.

Dr. John H. Drury, associate chief of cardiology at the Hospital of Saint Raphael, said the difficulty of screening athletes for potentially lethal heart problems is a dilemma.

"Fortunately deaths don’t happen very often. It’s tragic when it happens," he said.

"As a group, the vast majority of people with HCM don’t have sudden death," he said.

Only about 6 percent of people with HCM display symptoms, making the condition extremely difficult to diagnose, Drury said.

Other causes of sudden death include congenital coronary artery anomalies; myocarditis (an inflammation of the heart); Wolff-Parkison-White Syndrome, which makes people susceptible to arrhythmia; and Marfan syndrome.

People with Marfan syndrome tend to be tall and have long arms, desirable traits in a basketball player. They also have a high rate of aortic aneurysms, or bulges in the main artery leading out of the heart.

Most athletes with Marfan syndrome are screened out before they can play, Drury said.

Drury said that though student-athletes are bigger and training harder than in the past, there is no evidence that sudden death is increasing.

There is probably a threshold for sudden death, meaning that beyond a certain level of exertion the risk does not rise, he said.

Ultimately, genetic screening may be useful in identifying people at risk for hypertrophic cardiomyopathy, doctors said.

HCM is apparently carried by dominant genes, although the number of children born with the condition varies.

Drury said more than 10 genes associated with HCM have been identified.

Some day identifying people with HCM may be simpler, but testing 5 million young people a year is likely to remain an impossibly expensive job, he said.

"We need to recognize that sudden death happens in young athletes, fortunately not very often," Drury said.

"When it does happen, 80 percent of the time we will find a heart abnormality," he said.

All of the doctors urged athletes to report family histories and symptoms to their physicians.

A moment’s lightheadedness could be the clue that saves a life.