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Following the Trail of Broken Hearts

Dec. 10, 2007
Dec. 10, 2007

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Dec. 10, 2007

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Following the Trail of Broken Hearts

A congenital cardiovascular abnormality has become a leading killer of young athletes in the U.S. So why isn't more being done to save those who have it?

THE KID'S playingwith me, William Batts figured. One second 17-year-old DeCarlo Polk had gone upconfidently for a jumper, his chiseled 5'9", 237-pound frame rising justbeyond the free throw line in their game of one-on-one. The next, he hadtumbled to the Nashville blacktop like a piece of laundry at the end of adryer's cycle. Now, on the afternoon of June 27, DeCarlo lay flat on his backin the simmering heat. Real funny, the 37-year-old Batts thought. But then hetook a step closer and saw that DeCarlo's pupils had rolled back into hisskull, and he knew it was no joke.

This is an article from the Dec. 10, 2007 issue

Somewheremid-jump the electrical signals that cued DeCarlo's heart to pump had misfiredterribly. Rather than flexing and contracting rhythmically, his heart trembledlike jelly in a shaken jar. His left ventricle, the chamber that takesoxygenated blood from the lungs and sends it through the body, stopped working,causing a circulatory traffic jam. As blood piled up in the lungs'capillaries—vessels so small that red blood cells have to pass through themsingle file—water in the bloodstream breached the capillary walls and settledinto the air sacs of his lungs, spaces normally reserved for oxygen. DeCarlohad stopped breathing and started drowning.

Batts called hisboss—DeCarlo's uncle, Jimmy Boulton—at his paint shop three doors down, andBoulton came running with Rick Jones, a coworker. Jones became DeCarlo's lungs,blowing oxygen-rich air into his mouth. Then he became his heart, pushing downon his chest to force the oxygenated blood through the lungs and into the restof his body. But CPR could only buy time.

Don't let thishappen, Boulton prayed, not now. On June 8,DeCarlo had graduated from HillsideHigh in Durham, N.C., where he had been an honor student, homecoming king andstar defensive lineman. Football scholarship to Division II St. Augustine'sCollege in nearby Raleigh in hand, he was baptized two days later, then jumpedin Boulton's truck for his annual summer visit to Nashville, his hair stillpearled from the baptismal dip. Within a week of his arrival DeCarlo became theproud cousin of Jimmy's third child, James Kerrigan Boulton Jr.

It took at least10 minutes for the rescue workers to arrive; already DeCarlo's oxygen-deprivedbrain cells were dying in droves. The paramedics applied defibrillators toDeCarlo's chest, trying to fix the deadly rhythm that had begun in his leftventricle. Throughout his heart electrical signals were ricocheting likepinballs, spurring spastic muscle twitches. With each jolt from the paddlesDeCarlo's powerful body would lift and then flop limply to the ground. His17-year-old heart was broken.

DECARLO WAS oneof an estimated 600,000 Americans with hypertrophic cardiomyopathy (HCM), agenetic disease that causes the walls of the left ventricle to enlarge, usuallyduring adolescence; the thickened ventricle does not relax fully, inhibitingthe flow of blood into the heart. While many will never exhibit a symptom, some6,000 will die from HCM each year, more than from asthma and Hodgkin's diseasecombined. In those HCM victims, the left ventricle's muscle cells, rather thanstacking up flush like bricks in a chimney, are arranged at odd angles, allaskew, as if the bricks had been tossed into a Dumpster. With the cells in suchdisarray, an electrical signal crossing them is liable to travel in an erraticpath and fatally misfire.

"Hypertrophiccardiomyopathy is the most common cause of sudden death in young athletes,"says Dr. Barry Maron,director of the Hypertrophic Cardiomyopathy Center at theMinneapolis Heart Institute Foundation and one of the top experts on HCM."And it's the most common cause of [natural] sudden death in young peoplein the general public. But it's still a little-known disease."

For those whosuffer from some of thenation's most common health problems, such as diabetes,high cholesterol and coronary artery disease, exercise is a potent remedy. Butfor those with HCM it is the most activeindividuals—the young (usuallyteenaged) paragons of fitness—who are at higher risk of sudden death because oftheir athletic pursuits. At least every two weeks an athlete with HCM will dieduring or immediately after exertion, when his or her abnormally thick heart,triggered by strenuous exercise, will start beating in a lethal cadence.

Some of thevictims are famous, such as San Francisco 49ers offensive lineman ThomasHerrion, 23, who collapsed in the locker room minutes after a preseason game inAugust 2005, and Jason Collier, 28, the Atlanta Hawks center who died in themiddle of the night two months later. But the majority are less well-known,their lives just beginning, such as 16-year-old Adam Litteken, who wentlifelessly sliding across a hockey rink after he collapsed during an Octoberpractice in St. Peters, Mo., and DeCarlo Polk.

And KevinRichards. During my senior year at Evanston (Ill.) Township High, Kevin, aprecocious freshman, became my friend and training partner in track. After Iwent to Columbia in August 1998, Kevin helped Evanston win its first 4 √ó800-meter state title the following spring. By his junior year Kevin, the sonof Jamaican immigrants, was on his way to becoming the first member of hisfamily to attend college, most likely on a track scholarship. Indiana was highon his list.

On the afternoonof Feb. 12, 2000, Kevin was locked in a tight race in the indoor mile. The bellhollowly signaled the final lap as Kevin kicked to the shoulder of Dan Glaz, atop Illinois distance runner from Amos Alonzo Stagg High in Palos Hills. Duringthat lap Kevin's heart struggled to function. Still he kept coming, finishingsecond by a meter. After crossing the finish line Kevin walked three steps,dropped to his knees and flopped over on his back. It's entirely normal for abone-weary runner to sink to the ground after a hard race, but never Kevin. Yetthere he was, quaking and heaving as he died on the track.

Each of thesevictims had HCM etched into his genetic code. Despite their apparent goodhealth, they were destroyed by one mutation in the three billion base pairs—thechemical compounds that form the rungs of the twisting DNA ladder—that make upthe 25,000 or so genes crammed into the nucleus of a human cell. That's theequivalent of a single typo in 60 full sets of The Encyclopedia Britannica.

HOW CAN a diseaseabout 20 times more prevalent in the U.S. than Lou Gehrig's disease kill inrelative anonymity? Perhaps it is because HCM, unlike ALS, isn't always deadly,and when it is, it often strikes without notice. There is no progressivelyweakening immune system, no final battle embodied by a courageous ballplayer."Right now we really don't know most of the time who is at risk of suddendeath," says Dr. Paul D. Thompson, a cardiologist at Hartford Hospital. Thefirst diagnosis is often made by a medical examiner, if it is made even then.With few live patients to examine, clinicians have had trouble getting a fix onthe disease since British pathologist Donald Teare first compared HCM to atumor of the heart in 1958. The disease would acquire more than 80 differentnames, from apical hypertrophy to subvalvular aortic stenosis, until becomingwidely known as hypertrophic cardiomyopathy within the last decade.

While an enlargedheart might serve as a conspicuous sign of trouble, it is also characteristicof a highly conditioned athlete. It is no surprise to anyone who saw DeCarlorace sideline to sideline for a sack that he had, literally, a lot of heart. Anormal, fully mature human heart weighs about 300 grams, or two thirds of apound. DeCarlo's heart—perhaps enlarged from working out, and certainlyenlarged with HCM—weighed 600 grams. (Ryan Shay, 28, who died 5 1/2 miles intothe Olympic marathon trials in New York City last month, and Toronto Blue Jayspitcher Joe Kennedy, 28, who died at his in-laws' home three weeks later, alsoreportedly had enlarged hearts. Their autopsies are pending.)

If, during highschool, DeCarlo had been given an electrocardiogram, or ECG, a $50 test thattakes a few minutes and records the electrical signals in the heart, acardiologist might have noticed a suspicious pattern indicative of an enlargedventricle. The doctor might have followed up with an echocardiogram—or echo,for short—an ultrasound that gives a real-time picture of the heart and costs$1,000 to $2,000. To a cardiologist who had seen HCM before, an echo coulddetermine whether the heart was merely enlarged by exercise (in which case boththe muscle of the left ventricle and the chamber it surrounds would expand) orwhether it was afflicted by HCM (in which case the muscle walls would grow butthe chamber would not). The doctor might have recommended that DeCarlo tradehis cleats for golf clubs, tools of a less vigorous sport that he loved. Hecould have lived a long, normal life with a surgically implantablecardioverter-defibrillator (ICD) standing sentinel inside his chest. Thetitanium-encased, computerized device is about the size of a matchbox and isprogrammed to give the bearer's heart a 750-volt shock when it detects anabnormal rhythm.

DeCarlo wouldhave undergone at least an ECG had he grown up in Italy, where all competitiveathletes, from grade-schoolers to pros, must, by law, have their hearts undergogovernment-subsidized screening. When an American child's heart fails,crestfallen parents often, and understandably, speak out in favor of anationwide screening program that includes ECGs. If Italy can do it, why can'tthe U.S., the richest and most sporting of nations?

For one, the U.S.has 37% fewer doctors per capita than Italy, a nation that opened the doorswide to its medical schools after World War II. Some Italian doctors have spentalmost their entire careers screening athletes. With an American citizenry thatis far more geographically and genetically diverse, and that has more thantwice as many high school athletes (7.3 million) as Italy has high-school-agepeople, marshaling enough experts to do quality, uniform screening isimpractical, with the costs prohibitive. "The U.S. health care system doesnot have the mechanisms to pay for an ECG for every athlete," says LisaSalberg, who has HCM and founded the Hypertrophic Cardiomyopathy Association."Nor do we have enough trained professionals to evaluate the results. Therewould be a lot of athletes placed on alert for no reason, and a lot of misseddiagnoses."

And yet,immediate steps can be taken given the hereditary nature of HCM. "Askingwhether there is anyone in the family who has died before the age of 50 ofcardiovascular causes is incredibly valuable," says Dr. David Glover, aWarrensburg, Mo., physician and expert in presports participation screening,"and that doesn't cost a nickel."

Although Gloverhas seen improvement in preparticipation screening questions, he has alsodocumented a dangerous trend. In 1997 there were 11 states that allowedchiropractors or other nonphysicians to perform the exams that deem a highschool athlete fit to play. By 2005 that total increased to 18 states thatsanctioned, Glover says, "practitioners with little or no cardiovasculartraining" to conduct athletic screening. "You need somebody thatlistens to hearts every day," Glover says. "A trained examiner knowsthat if a heart murmur is very loud, or if it occurs during the relaxationphase of the heart, or if it gets louder when you stand up, it raises suspicionthat a person could have HCM."

THERE IS a sleek,steel-and-glass building about a mile away from Boston's Fenway Park where themen's and women's rest rooms are labeled with diagrams of chromosomes. On itssecond floor is Harvard's Seidman Lab, run by the husband-and-wife team ofJonathan and Christine Seidman. "Looking at a patient's genes can tell usthat somebody has HCM or is going to develop HCM as they get older," saysChristine. The lab is putting mice genetically bred to have HCM through thefirst HCM drug trial, with the hope that, Christine says, "in a decade ortwo, the [manifestation of the] disease may be preventable."

Perhaps thosefuture patients will have had HCM diagnosed with absolute certainty throughtheir genes at the nearby Harvard-Partners Center for Genetics and Genomics,where each week researchers identify a new HCM mutation, of which more than 800are currently known. For $3,000 the center can pop the blood of a living person(or a deceased one, if a medical examiner is astute enough to freeze a sample)in what looks like an oversized gray microwave and find out if that individualgot HCM from any of the currently known mutations.

And once amutation is identified in one member of a family, other members can be testedfor the same mutation—at $250 apiece. That's what Jimmy Kogut's three youngersiblings are doing. A 21-year-old junior at Pittsburgh, Jimmy spent his leisuretime playing basketball and lifting weights until last December, when testsrevealed that he had HCM; eight months later genetic screening found that hehas one of the most common HCM mutations, on a gene that helps to regulateheart contraction. Jimmy's mother, Eileen, had long known that somethingdangerous ran in her family. Her brother Joe, then 15, died at the dinner tablein 1978 while horsing around with Mark, his older brother. Mark died 20 yearslater, at age 37, while running on a treadmill.

Because of herfamily history, Eileen had Jimmy's heart checked regularly from the time he wasthree, and everything seemed fine. At seven Jimmy complained of shortness ofbreath and was told that he had asthma. Fatigue and shortness of breath, evenfainting, are common symptoms of HCM, and a misdiagnosis of asthma is alsocommon. This can be especially hazardous because asthma inhalers can causeirregular heartbeats in people with HCM.

Jimmy now knowsthat he never had asthma. He has HCM, and he got his gene mutation from hismother, along with his hazel eyes and freckles. An HCM mutation follows ahereditary pattern known as autosomal dominant: In other words, it has a 50-50chance of passing from parent to offspring. Those who inherit the gene willeach have a 50-50 chance of passing it on to their children, and so on down thefamily tree. Those who do have it may, like Jimmy, undergo surgery, have adefibrillator implanted and give up intense exercise for good. "I can stilldo some light weightlifting," he says, "but nothing over my head orthat stresses my left side too much so that it might damage the ICD." Asmore families submit to genetic testing and the list of mutations grows, theHarvard-Partners Center is keeping an eye toward categorizing which are deadly(and demand an ICD and cessation of vigorous sport) and which are relativelyharmless (and might permit life as usual).

BUT WILL peoplefeel safe to seek that knowledge? In September 2005, six months after ChicagoBulls center Eddy Curry felt his heart skipping beats, the team added a genetictesting clause to the one-year, $5 million contract offer it had on the table.If the tests showed that Curry had HCM, the team would not let him play, but itstill promised to pay him $400,000 a year for the next 50 years. Fearing thatthe public disclosure of the results might jeopardize his career, Curryrefused, and the Bulls traded him to the New York Knicks. "As far as DNAtesting, we're just at the beginning of that universe," Alan Milstein,Curry's attorney, told the Associated Press. "Pretty soon, though, we'llknow whether someone is predisposed to cancer, alcoholism, obesity, baldnessand who knows what else.... Hand that information to an employer and imaginethe implications."

The GeneticInformation Nondiscrimination Act (GINA) would prevent employers fromrequesting employees' genetic information, and employers and insurancecompanies from discriminating against a person based on the content of theirgenetic code. In effect, it would give people like Curry the freedom to submitto a potentially lifesaving test without fear of reprisal. Last April the Houseof Representatives voted 420--3 to pass the bill, and President George W. Bushhas already said that he will sign it should it clear the Senate. But SenatorTom Coburn (R., Okla.), a physician who voted for a similar version of the billthat passed the Senate 98--0 in 2005 (but never made it to a vote in theHouse), has placed a "hold" on the latest GINA, forestalling a vote.Coburn initially argued for specific language ensuring the rights of the fetus.The language of the bill was altered to that end, but his hold persists.

Coburn says thathe supports a genetic nondiscrimination law, but that the current version ofGINA does not provide enough protection for employers. "What if an employeefiles a form to take family leave from work and they write on the form thatthey have to take care of their mother who has breast cancer?" he says.Because breast cancer can be hereditary, "the employer may have gatheredgenetic information accidentally, and they are vulnerable in a lawsuit. We needprotection for [employers and insurance companies] who don't mean todiscriminate, but have accidentally collected information."

As far asChristine Seidman is concerned, the passage of GINA, which was first introducedin 2002, is overdue. "Americans have to support this," she says. "Ihave Alzheimer's and breast cancer in my family. You may have schizophrenia. Weall have something. I believe that families have a right to know everythingthey can."

But should allpatients know everything about their genome? Even experts aren't certain."I see some kids, and they don't have a family history of death and theydon't have symptoms or a very thick heart, and I don't think a lot of them areat great risk," says Thompson, a former marathoner who competed in the 1972Olympic trials. "I usually say to them, 'I don't think you're at greatrisk, but I have to sleep at night, and I can't take a chance with you, so I'mprohibiting you.' For some acne-stained 17-year-old who's accepted at that highschool because he's a good linebacker, to tell him that's gone is aload."

THE MOSTcontentious case of HCM in sports is the one that Nick Knapp is sure he neverhad. When Knapp rises from behind his desk in Peoria, Ill., where he works as afinancial consultant, it's not hard to imagine him having once been one of thetop high school basketball players in the state. Even now, eight years removedfrom his playing days, he is, at 6'5", an erector set of a man, his sharpjaw line leading to a tautly muscled neck that tapers to his bulgingshoulders.

In September1994, Knapp was on his way to breaking the state record for careerthree-pointers when his heart stopped during a pickup game at Woodruff High inPeoria. He was revived, had an ICD placed inside his chest and two months laterspurned more prestigious programs to accept a basketball scholarship toNorthwestern. When he arrived in Evanston, however, the school's doctors,concerned that he might have a fatal heart condition, declared him ineligible.Knapp's left ventricle was slightly enlarged, and cardiologists were divided onwhether he had the disease. Knapp himself doubted that he had HCM and sued theuniversity for the right to play. Keeping him off the court, Knapp argued inhis lawsuit, violated the 1973 Rehabilitation Act, which protects"otherwise qualified individuals" from discrimination based on adisability.

Knapp won infederal court, but Northwestern got the ruling overturned by the U.S. Court ofAppeals for the Seventh Circuit. The court acknowledged that Knapp could playcollege ball, but that Northwestern also had the right to heed its doctors andto prevent him from potentially risking his own death at its facilities. Thoughthe university honored his scholarship, Knapp, who sat out for two seasonsduring the litigation, transferred to Northeastern Illinois, then to DivisionII Ashland (Ohio) University. He still ponders what might have been. "I'veplayed against guys who went to the NBA, like Brian Cardinal and StephonMarbury," Knapp says, "and I was at that level."

Since Knapp'sordeal, the use of implanted defibrillators has become more common in thepopulation at large, and doctors have permitted some athletes with ICDs tocompete. In January 2006, Washington guard Kayla Burt, who has Long Q-Tsyndrome, a genetic heart disease that, like HCM, is treated with an ICD, wassitting on the bench in a game against UCLA when her device fired. "Shethought someone came up behind and punched her in the head," recalls teamtrainer Jenn Ratcliff. The shock "usually gets a 10 out of 10 on the painscale from patients," says Dr. Mark Estes, an electrophysiologist anddirector of the New England Cardiac Arrhythmia Center. Burt, who had nearlydied on New Year's Eve 2002, when she went into cardiac arrest, gave up playingfor good that night. "The device worked, which is good, but not exposingyourself to potentially lethal cardiac arrhythmias is a good choice," Estessays, adding that an ICD's wires are not made to withstand the strain ofsports. "The leads can be damaged," he says. "I had one 20-year-oldwoman who destroyed a lead just doing aerobics."

How wellimplanted defibrillators hold up during sports is one of the many questionssurrounding HCM. There are still mutations to find. There is still much that isnot known about the progression of the disease—when and how the cellulardisarray develops, for example—largely because a live heart can't be put undera microscope. There is still no explanation for why Eileen Kogut's two brothersdied but she, with the same HCM mutation, leads a normal life. Says Salberg,"Somewhere between screening every kid in America for a couple of billiondollars and not doing anything lies the right answer."

DECARLO POLK'Smom, Tommie (Lady) Polk, knows a lot about HCM now, but she does not have anymore children to lose. DeCarlo was her baby, and she clings to his memory as iftrying to keep a balloon from drifting into the sky. There are mementos aroundevery corner in her yellow clapboard house in Durham: in the family roomDeCarlo's first football shoes, burgundy-and-yellow Redskins sneakers that fitin the palm of Lady's hand; in his bedroom the folded New Orleans Saintsreplica jersey with Reggie Bush's number 25 stitched on the back that he nevergot to wear. She keeps the bedroom door locked and the fan continually running."I can smell him when I'm in here," she says.

If only she hadknown about HCM, Lady Polk says. If only the woman down the street had spokenup when her son had died from the disease, Lady would have gladly paid for thetests to make sure her baby was safe. As for his football scholarship? Shenever counted on that anyway. "DeCarlo could have carried water for theteam," she says. "He wasn't born to play sports."

DeCarlo was morethan Lady's only son; he was her guide in a noisy world. In the last few yearsLady's hearing has deteriorated; nearly deaf, she had to leave her teachingjob. "I couldn't keep up with kids how I knew I should," she says. NickKnapp had to adjust to life without basketball, and Lady is just beginning lifewithout something far more precious. Most of the time she is alone, in hersilence.

AT LEAST EVERY TWO WEEKS IN THE U.S., A HIGH SCHOOL,COLLEGE OR PRO ATHLETE WITH HCM WILL DIE.
SHOULD ALL PATIENTS KNOW EVERYTHING ABOUT THEIRGENOME? EXPERTS AREN'T SURE.
IF ONLY SHE'D KNOWN, LADY POLK WOULD HAVE PAID FOR THETESTS TO MAKE SURE DECARLO WAS SAFE.

Anatomy of a Failing Heart

NORMAL HEART
Left Ventricle Wall
Right Ventricle
Septum
Left Ventricle Cavity

HCM HEART
Left Ventricle Wall
Right Ventricle
Septum
Left Ventricle Cavity

In the HCM heart the septum, the muscle wall thatseparates the ventricles, grows abnormally thick and invades the leftventricular cavity, where oxygen-filled blood collects before being pumpedthrough the body. Normally thinner than 1.2 centimeters, a septum thicker than1.5 cm is nearly always indicative of HCM. When the septum is between 1.3 and1.5 cm, however, doctors find it difficult to determine whether the heart hasbeen enlarged from exercise or from HCM.

EXTRA

A Tragic Turn
More from David Epstein on how HCM cut short one promising athlete'sdreams.

ONLY AT SI.COM

PHOTOPhotograph by Prestige PortraitsMIXED SIGNALS An enlarged heart often indicates a superbly conditioned athlete, but for 17-year-old DeCarlo it was a sign of his hypertrophic cardiomyopathy (HCM).TWO ILLUSTRATIONSPHOTOPAUL SAKUMA/AP (HERRION)A CRUEL TOLL Herrion (left) dropped dead after a preseason game, while Collier died at home in the middle of the night.PHOTOJENNI GIRTMAN/ATLANTA JOURNAL-CONSTITUTION/KRT/ABACA (COLLIER)[See caption above]PHOTODAVID BERGMANDON'T TEST, DON'T TELL Curry refused to undergo screening that might have revealed HCM, so the Bulls traded him to New York.PHOTOTOMMIE POLK AND FAMILYSTEPS TO TAKE DeCarlo wouldn't have played football with HCM, but with a defibrillator, he could have led a normal life.