Kyle McCabe was a multi-sport athlete before a screening revealed, at age 13, a rare disorder that can lead to sudden cardiac arrest. (Family Photo)

Every year, seemingly healthy young people drop dead from sudden cardiac arrest. Kyle McCabe is not among them. His life was saved, his mom says, by a screening that might never have happened.

Kyle had played in a youth football league in East Norriton, Pa., for years before a friend suggested to his mom, Josie, that they take their boys to a free heart screening. The 13-year-olds, including Kyle’s fraternal twin, Josh, moved through the stations: family history, blood pressure, an electrocardiogram.

When the other boys were done and ready to go home, Josie McCabe told her friend there was a problem with Kyle. “I can’t go,” she said, crying. “They found something.”

She was advised to make an appointment for Kyle at the nearby Children’s Hospital of Philadelphia. No more football, she was told, “until he’s checked out.”

Two evaluations, one at CHOP and a second at Nemours Alfred I. duPont Hospital for Children in Wilmington, Del., showed that Kyle had a congenital form of long QT syndrome, a rare disorder that can disrupt the rhythm or rate of the heartbeat and cause sudden cardiac arrest. For people with the syndrome, many sporting activities are limited as a precaution.

For Kyle, a multi-sport athlete, the news was “the end of the world as he knew it,” McCabe said.

Sudden cardiac arrest kills more than 3,000 children every year, according to one estimate, perhaps as many as 150 of them athletes who collapse during competition or training. The deaths raise questions about how to prevent losses so devastating to families and communities and to identify those at risk.

It’s a complex and emotional issue, which the American Heart Association and American College of Cardiology acknowledged in a scientific statement two years ago: Should an EKG be required for each of the 60 million Americans between ages 12 and 25? Would mass screenings be feasible, cost-effective or even reliable methods of reducing sudden cardiac death?

The report notes that more data is necessary but hard to come by. Its primary recommendation is that health professionals follow the heart association’s 14-point screening process. Many parents of adolescents are familiar with the Preparticipation Physical Evaluation, or PPE, which many schools require for sports. Both the PPE and the screening guidelines ask questions related to heart health and family history. If the screening raises questions, an EKG might be in order. But the report says there is no evidence that administering universal EKGs before the 14-point screening would save lives.

“Those who do not sign up for sports are just as likely to have the genetic heart diseases that raise the risk for sudden death,” Barry J. Maron, a cardiologist who helped write the report stated when it was released. “Since there are by far more non-athletes — only about 1 percent of college students and 30 percent of high school students participate in competitive sports — there are more deaths in non-athletes participating in recreational sports and normal daily activities.”

But the report did not end the debate over whether mass screenings should be mandated and what they should include. And much of the conversation still revolves around athletes, because their deaths are so widely publicized and there is more data available about them.

Theodore Abraham, an associate chief of cardiology at Johns Hopkins Hospital in Baltimore, developed a screening process for student athletes that he calls the Heart Hype protocol, which involves an EKG as well as an echocardiogram, or echo.

He wanted to help identify hypertrophic cardiomyopathy, or HCM, a thickening of the heart’s walls. It’s the most common cause of sudden death in young athletes and is underdiagnosed in African Americans, who may be at higher risk than whites.

For several years, Abraham recruited volunteers and used donated equipment to provide free screenings at Maryland high school track and field competitions. With the idea that half the people with HCM do not have a known family history of it — in part because it can be confused with a heart attack — Abraham added the EKG and echo to look closely at the heart.

To Abraham, relying on family history and a physical, or adding an EKG but not also the echo, does not do enough to identify risk. “Why would you push a policy that doesn’t make sense?”

An expensive approach

But the Hopkins screening is expensive — about $1,200 when billed to insurance — and sudden cardiac death in young people is relatively rare.

The discussion surrounding screening strategies is confusing, Abraham said. It’s at “the intersection of family, society and medicine and is a controversial question.”

Even if he had the funds to continue, Abraham said, he would be conflicted about where they would do the most good.

“I am a reductionist. It’s about [saving] one person at some level.”

Screening for cardiovascular disease in children begins early — before birth, in fact, according to Marie Gleason, director of cardiac outpatient operations at CHOP.

After birth, a newborn’s pulse and oxygen level are measured using a bedside test called pulse oximetry, helping to identify congenital heart defects that require immediate surgery. Pediatricians monitor for signs of distress and later check for high blood pressure, a possible sign of problems with the aorta.

As for underlying risk factors for sudden cardiac death, “if you have a 14-year-old with no heart murmurs and is otherwise healthy, there is no reason to believe they might have something like that,” Gleason said. “Bottom line is, the due diligence has to come on both the part of the family and the pediatricians — the ones who have to sign off on the forms to make sure they know the family history and know the cardiac risk implications,” she said.

The American Heart Association has provided consensus recommendations since 1996 on clearing youngsters for participation in sports. And several medical societies, including the American Academy of Family Physicians and the American Academy of Pediatrics, publish a book that includes guidelines and forms for the PPE. But there is no single standard or form mandated nationwide.

Making adjustments

Last fall, New Jersey became the first state to require physicians signing off on PPEs to watch a video on cardiac assessment. Families must also certify that they have read a pamphlet on sudden cardiac death in young athletes.

The clinic that flagged Kyle McCabe’s heart problem was run by Simon’s Fund, a nonprofit created by a Philadelphia-area couple, Darren and Phyllis Sudman. After the death of their 12-week-old son, Simon, the couple got unique advice from their pediatrician and coroner: Get your hearts checked, because babies just don’t die. Tests showed that Phyllis, like Kyle McCabe, had long QT syndrome, a hereditary condition that, because it is electrical in nature, would not have shown up in her son’s autopsy.

The Sudmans wanted to spare other families from discovering too late that their children were at risk of sudden death. In 10 years, they have screened more than 10,000 students and found heart conditions in 100. They’ve also built a digital platform called Heartbytes to make their data available to researchers and created a website, Screens Across America, to help parents find screening organizations.

Kyle McCabe is now a senior in high school and has adjusted to a lifestyle of modifications: no caffeine, no chocolate and no theme-park thrill rides. He cannot swim alone or run long distances. He takes a beta-blocker drug and sees a cardiologist whose other patients, his mother joked, are mostly 80-year-old men.

His mother remains grateful for the happenstance that led to the Simon’s Fund screening.

“What do you say,” she said, “to the man who saved my child’s life?”