Makaila Ouellette, 18, of West Hurley, is a busy, happy senior at Onteora High School. She’s also a profoundly grateful one, never forgetting that, in her junior year, she nearly died. Ironically, though, “I don’t remember what happened,” she says of her brush with death in October 2013. All she knows is what others have told her—that while playing in a varsity volleyball game at Wallkill Senior High School, she keeled over. “People say I was about to serve, but then said ‘I don’t feel so good,’ and walked off the court. My coach ran over, and I collapsed.”
Ouellette’s heart had abruptly stopped beating, a deadly condition called sudden cardiac arrest. But she was lucky; her coach began CPR, assisted by a spectator who happened to be a sheriff. Meanwhile, someone located the school’s automatic external defibrillator (AED), a device that delivers a chest shock to someone whose heart has halted. It was used on Ouellette, jolting the 16-year-old back from the brink of death. Her good fortune continued: After being rushed to an area hospital, she was transferred to Maria Fareri Children’s Hospital at Westchester Medical Center in Valhalla. There, Ouellette had the benefit of both the region’s only pediatric intensive care unit and the region’s most comprehensive pediatric cardiac services.
A Rare, but Deadly, Risk
You probably don’t associate cardiac arrest with children or teens. But as scary as Ouellette’s story is, it happens to as many as 25 other seemingly healthy American kids every day—perhaps as many as 9,500 per year, according to the American Heart Association. This number, though, is disputed; some experts feel it’s lower, and no one’s sure whether cases are on the rise. “I don’t believe the number of cases is increasing,” says Christina Miliaresis, MD, a pediatric cardiologist at Maria Fareri Children’s Hospital. “The incidence may seem to be increasing, though, because cases are reported more frequently than they were in previous decades.” What’s undisputed is that not enough kids have CPR-trained adults and a defibrillator close at hand, as Ouellette did, when cardiac catastrophe strikes. As a result, only a small percentage of children who experience a sudden cardiac arrest survive.
Those who do pull through present a challenge for physicians. “When an adult has cardiac arrest, it’s because they frequently have coronary artery disease—in children, though, that’s not the case”, explains Irfan Warsy, MD, Director of Pediatric Cardiac Electrophysiology at Maria Fareri Children’s Hospital. “We must initiate an entire workup to investigate.”
Even after a thorough search, however, doctors often can’t figure out what’s caused a child’s heart to stop, unless there was an obvious injury. (A blow to the chest, for example, can disrupt heart rhythm.) Among known cardiac causes, the list is long and varied. Topping it is a genetic syndrome called hypertrophic cardiomyopathy—it can thicken heart muscle, restricting or obstructing blood flow, or cause devastating arrhythmias. Abnormalities in anatomic structure and origin of the coronary arteries are the second most frequent culprit in children’s cardiac arrests. Next up are channelopathies, genetic abnormalities that prevent the heart from adequately using sodium, potassium, and/or calcium to regulate its rhythm—the most infamous of these is a condition known as long QT syndrome. Another frequent culprit is the common cold—a respiratory viral infection can rarely cause the heart muscle to inflame, a problem known as myocarditis. Lyme disease, a relatively common phenomenon in the New England area, may also result in cardiac arrest due to a block in electrical conduction within the heart, although cardiac collapse is a very rare complication of Lyme in childhood.
No matter what underlying medical issue a child may have, these conditions generally share one thing in common: They often give few or no warnings before causing a young heart to seize up, most frequently during intense exertion. That’s why, across the country, some are increasingly pushing for heart-health screenings for teen athletes. The American Heart Association recommends all competitive high school and college athletes undergo a 14-step screening process by a trained screener before taking the field—it includes a physical examination for things like a heart murmur or abnormal blood pressure, as well as a detailed personal and family history.
Should the screenings go even further? Some advocates are pushing for them to include an electrocardiogram (ECG), a test in which a machine is used to record and measure the heart’s electrical activity. In some countries, such as Italy, they’re already part of the protocol. “There’s quite a bit of controversy here about it,” acknowledges Michael Gewitz, MD, Physician-in-Chief and Chief of Pediatric Cardiology at Maria Fareri Children’s Hospital and member of the board of directors of the Founders’ Affiliate of the American Heart Association (AHA). But, he says, “There’s as yet no real scientific evidence that ECG screenings prevent teen deaths.”
That’s in part because children’s heart activity, especially during teen years, can be fairly variable from day to day, he explains, so the ECG can sometimes either raise false flags or fail to detect a serious problem. “There are also important differences in what is ‘normal’ between different races and ethnicities,” Dr. Gewitz explains. And while ECG’s aren’t terribly expensive—Dr. Miliaresis says the cost can be as little as $10—it would mean untold millions spent on tests across the entire US teen athlete population. Many would likely be inappropriately recommended for more costly, and sometimes invasive, tests.
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Genetic tests for hints of heart problems are another type of screening that’s come under debate. Again, Dr. Miliaresis says they’re largely unnecessary. “There are only about 11 to 15 genes you can test for that may suggest hypertrophic cardiomyopathy,” she explains. “You can’t possibly test for every single genetic mutation.” And these screenings are expensive as well—about $3,000 to $5,000 for the first family member and $300 or so for each additional relative, which may or may not be covered by insurance. Bottom line: “Most doctors won’t push a family to take these tests unless there is a strong history of hypertrophic cardiomyopathy,” says Dr. Miliaresis.
So, what does work? The AHA has recommended a workable screening process, confirms Dr. Miliaresis, and parental vigilance is important, too. “Know your family medical history,” she recommends. “And be aware of any symptoms of heart trouble your children may show. Do they ever get very short of breath or dizzy when they play intensely? Have they ever fainted or felt chest pain while exerting themselves? Tell your children they should always inform you if these things happen.”
Dr. Gewitz also encourages parents—and everyone, really—to learn cardiopulmonary resuscitation (CPR), “and also how to activate and use a defibrillator if one is available.” Be familiar, too, with the AHA’s Pediatric Chain of Survival, the series of actions that should be taken to save children from cardiac arrest. They include injury prevention and safety, early CPR with sufficient chest compression, prompt emergency room care, pediatric advanced life support, and comprehensive post-arrest care. Westchester Medical Center is the Hudson Valley “Chain of Survival” sponsor, which works to promote education of these actions in the community.
The Best Medicine
Top-notch care is exactly what Makaila Ouellette received at Maria Fareri Children’s Hospital after her volleyball-game collapse. Dr. Warsy promised the teen and her mother, Cheryl Keator, who’d rushed to her bedside, that he’d do his best to come up with an effective course of treatment. “He never spoke down to us, and he always took the time to explain things,” Keator recalls. He explained that clues as to what might have caused Makaila’s cardiac arrest came from some recordings the defibrillator at the gym had made, showing she’d had a ventricular arrhythmia—that is, her heart’s pumping chambers had developed an irregular beating that led to its failure to work.
Ouellette would remain at the hospital for 12 days as the pediatric cardiology team investigated her heart’s sudden halt. As is often the case with sudden teen cardiac arrests, no avenue yielded insights: She had no infections that might have inflamed the muscle, nor did exams show a congenital blockage of her heart’s arteries, or the arrhythmias that Lyme disease can cause. Doctors threaded four catheter electrodes, just a pencil lead’s width, through Ouellette’s leg veins and into her heart, testing its potential to short circuit; it exhibited none. Tests for chemical imbalances or other abnormalities came up negative, too.
Still, there was a silver lining in Ouellette’s results. They did not detect any serious inherited problems. Ouellette felt fine, and though she’d had significant amnesia and confusion when she’d arrived at the hospital—a result of oxygen deprivation to her brain while she’d been in cardiac arrest—her memory was returning. And Dr. Warsy devised a plan of treatment. He arranged for Ouellette to be fitted with a defibrillating device, an implanted instrument that can correct sudden arrhythmias with a shock, and can also act as a pacemaker if necessary. “I wasn’t sure how it was going feel, but Dr. Warsy calmed me down,” Ouellette remembers. In the unlikely event of a future arrhythmia, there’s a 90 percent chance the device can correct it with stimulation so subtle, Ouellette won’t notice. If that doesn’t work, it can also deliver a larger, lifesaving shock.
So far, happily, Ouellette’s felt nothing—except thankfulness for surviving her scary brush with death, and for the physicians of Maria Fareri Children’s Hospital at Westchester Medical Center who helped her through. She’s gone back to doing virtually everything she did before, except rejoin her volleyball team; doctors generally recommend against varsity-level sports after an episode like hers, though less intensive physical activity is fine. While Ouellette’s excited for her future (college applications are in the mail), she looks back on the life-changing day with awe, and upon the present with gratitude. “I’ll probably never totally remember how I got there,” she says of her hospital stay. “But I’m glad to be here today.”
For more information about the Pediatric Cardiology program at Maria Fareri Children’s Hospital, please visit