Broken Hearts Club

Written by Craig Outhier Category: Health & Fitness Issue: April 2013
Group Free

A PHOENIX magazine editor scours the Valley for answers to his heart disease, consulting cardiovascular comrades like Suns player Channing Frye and local doctors.

Well, this is it,” the surgeon says, with an encouraging smile. “As you can see, it’s pretty compact. Nothing so terrible.”

Admittedly, the smooth, teardrop-shaped device in his hand – slightly larger than a Zippo lighter, and the same silvery color – is hardly terrifying. And it is relatively small. By smartphone standards, for example, it would be record-breakingly compact.

But since the man holding it wants to cut open my chest and implant the device under my collarbone, along with three barbed electrical wires that will slither into my heart and nest there like prop pythons from an Indiana Jones flick, the sight of this gizmo chills me to the bone. It might as well be a suitcase bomb.
“Will people be able to see it?” I ask, vainly considering the cosmetic consequences.
By way of response, the physician takes my hand and guides it to the area just above his left shirt pocket. The large bump underneath feels solid and unyielding, as if someone taped a pager to his chest.

Ah, so, I think. The heart-club president is also a client.

“When you have your shirt on, no one will know it’s there,” he says. When you have your shirt on. But when you don’t, it’ll look like a gerbil crawled under your skin and died on your chest.

At 41 years old, I’m not too old to care how an Implanted Cardioverter Defibrillator (ICD) will look when I casually take my shirt off at the beach. Still, as a proud recipient of hereditary heart disease, I’m precisely the kind of person who needs one – specifically, the kind of person who might kick over and die at any second.

For me and people like me – a not-so-hearty group that includes professional athletes, amateur athletes, students, moms, dads and folks from every walk of life, including, as I will later learn, at least one standup comedian – the ICD is an effective stop-gap measure against cardiac disaster. It’s also crudely invasive and fraught with risk. And it looks bad. So, despite this doctor’s rather kind display of commiseration, I leave his office with little or no intention of opting for ICD surgery. Besides, there are whispers of a new, less invasive ICD in the Valley, and before giving up one’s active, non-chest-gerbil-impeded lifestyle, isn’t it prudent to check out every option?

Illustration Courtesy of Boston Scientific  Shocker: Artist's illustration of the new, less invasive S-ICD, which delivers a life-saving shock through the breastplate.My condition is called hypertrophic cardiomyopathy (HCM), informally known as “enlarged heart,” and even more informally known as “that thing that causes sudden death in young athletes,” most famously Loyola Marymount basketball star Hank Gathers and NBA All-Star Reggie Lewis. Dozens of young Arizonans have died from the disease, including former Mountain Pointe High football player Anthony Bates, whose mother, Sharon, subsequently launched a foundation in his name dedicated to raising awareness of HCM.
For many HCM sufferers – which is to say, the unluckiest HCM sufferers – the first and only symptom is cardiac arrest and death, often in the midst of strenuous activity. More fortunate patients experience non-lethal symptoms like chest pain, light-headedness and heart murmurs. That’s what I have – heart murmurs. Almost non-stop for 15 years.
I remember clearly the moment of my first murmur – or, at least, the first one I felt. It was on a drive to Sedona. The desert landscape was just pleasantly rolling by when my heart stopped – if only for a beat. Two quick beats later the organ was back in rhythm, a brief disruption to be sure, but long enough that it inspired a rising lump of alarm in my throat. Later, I would tell friends that it was like being tapped on the shoulder by mortality itself. Nothing too dramatic or painful. Just a tap. A reminder.

Hey, bro. Remember me?

Around the same time, I also had my first abnormal electrocardiogram (EKG) – the standard frontline test for abnormal heart rhythms. Referred to a cardiologist, I took comfort in the initial diagnosis: athlete’s heart, a condition marked by slight cardiac over-muscling, due to vigorous exercise. “You have nothing to worry about,” the cardiologist told me, with a recommendation to take magnesium supplements for the heart murmurs.
I was in my late 20s at this point, and none too eager to entertain the thought of a serious heart ailment. So I let the diagnosis ride, even as the tappings became more intense and frequent, and even as members of my family started getting diagnosed with HCM. Finally, years later, a second visit to the cardiologist revealed a dramatic thickening of the heart’s central structure, the septum, and its main chamber, the left ventricle, both definitive pathologies of HCM. The thickening is caused by an overabundance of muscle cells, laid on top of each other willy-nilly in a way that disrupts the heart’s natural electrical flow, a phenomenon known as myocardial disarray.

There are varying degrees of severity in the estimated 600,000 Americans afflicted with HCM; mine was deemed on the severe side of moderate. By no means a death sentence, but critical enough that my risk-averse cardiologist recommended the ICD.

Best-case scenario: You go through life never knowing you have the disease, and die old and happy. Worst-case scenario: The hardened, over-muscled ventricle can’t eject blood properly and one day slips into a spastic electrical state called a ventricular fibrillation. Like a car engine sucking in air, it seizes, and you go into cardiac arrest. If that happens, you better hope there’s somebody nearby with paramedic-style shock paddles, or that you’ve been equipped with one of those Zippo-sized ICDs, which perform essentially the same function.
Your chances of surviving cardiac arrest without immediate electrical defibrillation? About the same as hitting 00 in roulette. Which is to say: not terrific.

PHM0413 HRT2a

After receiving my HCM diagnosis, I did the only natural thing: I went on the Internet and found out which celebrities had it, too. Unfortunately, most of them are not with us anymore. Names like Gathers and Lewis and strongman Jesse Marunde. Ex post facto, it was revealed that late actor Corey Haim had HCM; though heart failure and pneumonia figured prominently in the official autopsy, years of vigorous post-stardom substance abuse also played a role in his demise, one would imagine.

Last summer, when Phoenix Suns power forward Channing Frye announced he was taking a sabbatical due to an enlarged heart, I felt a pang of empathy, and interest. Like many Phoenix-area basketball fans, I admire the way Frye handles himself, from his deadeye perimeter shooting – so rare in a player of his 6-foot-11 stature – to his upbeat court presence and smart locker room sound bites. I imagined us bro-ing out over HCM war stories.
As it turns out, Frye wasn’t diagnosed with hypertrophic cardiomyopathy, but its Bizarro World sibling: dilated cardiomyopathy (DCM). DCM affects the same part of the heart as HCM but in the completely opposite way – instead of becoming overmuscled and cramped, the stricken ventricle expands and loses tensile strength like a piece of worn elastic. Ultimately, the chamber simply becomes too vast and capacious for the compromised muscle to handle.
Though they take opposite routes, DCM and HCM arrive at the same ultimate destination: ventricular fibrillation and cardiac arrest, in the worst cases.

Curious about Frye’s prognosis – and interested in his course of treatment – I meet him on the Suns practice court one weekday morning after team shootaround. The first thing I learn is that DCM, unlike HCM, is not necessarily hereditary or genetic. In clinical terms, it’s most often idiopathic – meaning physicians don’t know what causes it.

“It’s definitely not genetic with me because I didn’t have it last year or any year,” Frye says reasonably. “They think I probably got sick and a combination of being sick and traveling and overexertion of my body caused [the heart ailment].”

Frye suspected that something was wrong with his health shortly after the conclusion of the strike-shortened 2011-2012 NBA season. Despite twice-daily workouts over the summer, he was getting winded on stairways and was constantly tired. He tried chalking up the fatigue to the eight weeks of down-time that followed his shoulder surgery in April, but a routine pre-season physical in September settled the matter: He had cardiomyopathy, possibly caused by the combination of a draining strike season in which NBA teams played an impacted schedule; stress caused by his infant daughter’s double cataract surgery; and a theoretical virus that may have taken advantage of Frye’s compromised immune system.

Fewer athletes die from dilated cardiomyopathy than HCM, but that’s not because the disease is less pernicious, or prevalent – it’s because DCM announces itself better. Like Frye, most DCM sufferers experience fatigue and seek out a physician before the condition achieves lethality. There are exceptions, obviously – including the wrenchingly sad story of Wes Leonard, the Michigan high school basketball star who collapsed and died just seconds after hitting the game-winning shot in a March 2011 game on his home court.
Doctors told Frye he’d have to sit the season out and reassess his heart after six months. Their prescription: rest, rest and more rest. Absolutely no strenuous exercise. “That’s been the hardest part,” says the player, who limits his fitness regimen to yoga and golf. “Our natural instinct is to power our way through things. But some things you can’t power your way through. And this is one of them. I don’t want to half-ass it, but half-assing it is exactly what I’m supposed to do.”

In about a third of DCM cases, the heart will relax back into a more normal shape, Frye says. In another third of patients, the condition will worsen, and in the final third, it will stay the same.

Frye is hopeful. His “ejection fraction” readings – which measure how efficiently the left ventricle pushes blood back into the body – have improved since last September. “My heart has definitely shrunk,” he says, with about a month to go before his six-month checkup. “And my ejection fraction has gotten better. Not awesome. But closer to the low end of normal.”

I ask Frye if he’s considered the possibly of getting an ICD – a procedure that would curtail the risk of death by cardiac arrest but almost certainly end his basketball career. “No, nothing like that,” he says, shrugging off the mere suggestion of it. For now, he’s entertaining only best-case scenarios, focused on repairing his health while raising awareness of heart disease testing through his “One Heart” T-shirt program, the proceeds of which benefit the nonprofit Frye Family Foundation.
“I look at guys like Ronnie Turiaf, Chris Wilcox and Jeff Green,” he says, checking off names of NBA players who successfully treated heart defects and returned to the court. “You look at their stories, and you know it can be done.”

The unspoken caveat: Each of those players had ailments that were correctable by surgery. There’s no record of an NBA player returning from dilated cardiomyopathy. Frye is cutting his way through unknown territory. And all he can do is take it easy.

To state the obvious: I’m not a professional athlete. Unlike a pro baller, I don’t have to worry about an ICD implant curtailing my earning power. And I’m probably past the point in my life where the cosmetic drawbacks of such an implant are that important.
“It’ll make me look old,” I complain to my wife.

You are old,” she shoots back. Touché.
Though the deadly consequences of HCM in young people are well documented – according to a 2003 report published in the New England Journal of Medicine, it’s the leading cause of sudden death in athletes, accounting for a quarter of all cases – the mere fact that I’m no longer in the 18-to-35 age group considered “critical” by cardiologists doesn’t insulate me from the danger. A recent research study in France found the majority of cardiac arrests happen in middle-age men playing recreational sports.
But that’s not necessarily newsworthy, is it? We expect old people – for the sake of political correctness, let’s call them “post-youth” – to have heart problems. Only when 18-year-olds start collapsing on basketball courts and track ovals does the public snap to attention. And it’s why many influential people have started lobbying for mandatory cardiac testing for high school athletes.

Illustration by Michael GellatlyTop: Hypertrophic  Cardiomyopathy
Pathophysiology: Thickening of the septum (middle) and left ventricle (shaded) may cause fatal arrhythmia.
Prevalence: 0.05 to 0.2 percent of general population.

Middle: Normal Heart
Proper proportion of left ventricle (shaded) to companion heart structures.

Bottom: Dilated Cardiomyopathy Pathophysiology: Enlarged left ventricle (shaded) impairs the “systolic” pump function of the heart.
Prevalance: .92 percent of general population.

Perhaps the most active such organization is based here in the Valley. Founded by former Suns team physician Dr. Paul Steingard, TOPS (Teams of Physicians for Students) has provided cardiac testing equipment to school districts all over the country, curtailing the cost of an EKG or more sophisticated echocardiogram, which can run upwards of $1,000.

Many school officials resist implementing cardiac testing, according to Steingard – even today, only a handful of Valley districts have opted into the TOPS program. Part of the reason may be that cardiac arrest in young athletes, while tragic and newsworthy, is not particularly common; various studies put the incidence rates at anywhere from one in 40,000 to one in 200,000 each year. Barry Maron, a Minneapolis cardiologist and one of the world’s leading authorities on HCM, estimates there are between 200 and 300 sudden deaths in young athletes per year in the U.S., out of roughly 14 million. As such, even the American Heart Association refuses to endorse mandatory testing.

I suspect an additional reason: the nanny-state-backlash rationale. Yanking a child from competitive sports because he might have a risk for cardiac arrest seems antithetical to the whole idea of childhood wonderment. It is, after all, just one of many risks associated with being young. Do we lock our children in sound-proof bunkers to block out all of them?

I have the same problem with getting an ICD. Quality of life is important. Enjoying one’s body is important. Being active while you still have the physical wherewithal to do so is important. Won’t a lithium ion Iron Man heart battery change that?

On a tip from legendary Valley comedy promoter Dan Mer, who has contended with his own heart issues, I make a call to Ahwatukee-based standup comic George Cantor, who was fitted with an ICD seven years ago after suffering two heart attacks in quick succession. A devoted gym rat, Cantor always avoided rich foods and took reasonably good care of himself. Still, heart disease was a genetic fait accompli – his father died from it in his late 40s.
Months of rehab and a robust mosaic of heart medications have allowed Cantor to live a more or less normal life again, even with the ICD, which he characterizes more as an irritant than a handicap. “I have to hold back [at the gym] because when my chest gets pumped with blood, it pulls on the scar,” he says. “It’s always on my mind... it messes with my mojo, if that’s what you want to call it.”

Going through airport security checkpoints, which he does frequently, given the nature of his work, is also a hassle. The ICD is sensitive to X-rays. “I have to pull someone aside and say, ‘Excuse me, I need a male-assist,’” the 54-year-old says, using the phrase for an individual pat-down. “About one out of every 10 security people say ‘Wow, you’re pretty young to have one of these.’”

Cantor seems to have emotionally tolerated the device extremely well – he’s even worked the ICD into his standup act, with a bit about what would happen if the device shocked him, and his partner, in the middle of sex. His philosophical attitude about the experience is encouraging to me. According to a study in the Journal of Cardiopulmonary Rehabilitation and Prevention, clinically significant anxiety (13 to 38 percent) and depression (24 to 41 percent) are common side-effects of the device.
Where the ICD is concerned, my biggest fear is not that I’ll slip into cardiac arrest and die. I’m not even that worried about the shock itself, which some patients have likened to being kicked in the chest by a donkey. My ultimate fear: that it will corrupt my outlook, make me a bummer to be around and sap my ambitions. In my ICD nightmare, I envision myself growing obsessed by the ominous, gerbil-like lump on my chest, fixated on its unseen electrical pacing and ever-ready voltage, slipping into paranoia – tick tock, tick tock – like the conscience-stricken narrator in Poe’s The Tell-Tale Heart. Maybe that’s melodratic. No – that’s definitely melodramtic. But it’s there.

Cantor understands. But he’s a big advocate of the device, sex-partner-shockings notwithstanding. “I just don’t see the downside,” he says, noting that he’ll probably get a newer, better ICD in a few years. “I’m looking forward to seeing what they come up with in the 2020 model. I bet it’ll be sweet.”

ICDs wear out, and that’s a problem. A big problem. Studies indicate that roughly 1 percent of patients die during extraction of the devices. Which is why scientists have developed a new ICD that doesn’t actually touch the heart. And Scottsdale is one of the few places you can get one.

“I think this device is ideal for someone like yourself,” Dr. Thomas Mattioni of Arizona Arrhythmia Consultants says. “It’s better for an active lifestyle and better for many younger patients.”

Perusing the Internet for alternative ICD options, I came upon an article describing a new “subcutaneous ICD” – or S-ICD – being developed by Cameron Health, Inc., a medical technology firm. Mattioni and his colleagues conducted local trials of the device in 2010 and now are the first and only electrocardiologists qualified to implant the S-ICD in Arizona.

The advantages of the S-ICD are manifest: No part of the device touches the heart. Gone is the three-headed electrical hydra that snakes into the left ventricle. Instead, the S-ICD uses a more sophisticated sensory operating system and delivers a bigger charge through the breastplate, like a traditional defibrillator.

It’s not just a comfort issue; according to Mattioni, the three leads in a traditional ICD are notoriously prone to failure. “It’s such a dynamic environment in the heart, so much blood pumping, so much muscle contracting, that they’ve never really found a reliable way to make those leads,” he says, noting that two extant ICD models are currently in recall. “They’ve gotten much better over the years, but they’re still unreliable.”

Dr. Thomas Mattioni of Arizona Arrhythmia Consultants, currently the only electrocardiology clinic in Arizona offering the S-ICD.

A few months before my meeting with Mattioni, I asked my cardiologist about the S-ICD. He flatly told me not to get one, citing its lack of a pace-making function, which can stop fibrillations before they really get going.

Mattioni shrugs when I tell him this. “Cardiologists are like anybody – they resist the unknown. But I can tell you that the S-ICD is entirely reliable. For someone like you, who doesn’t need regular pacing, the pluses may very well outweigh the minuses.”

Unlike the traditional ICD, which sits at an inopportune spot just below the left shoulder, the S-ICD rests just south of the pectoral muscle, toward the armpit. According to Mattioni, this offsets the tendency of the wires to fracture during strenuous activity, requiring surgery to remove them from the heart.
Mattioni concedes one drawback to the S-ICD, which he says has “discharged” in three of the 20 or so patients he has outfitted with the device since 2010, with no deaths. It’s a bit bulkier than the traditional ICD, owing to the larger battery and capacitor required to deliver a jolt through the breastplate. Even so, the S-ICD is every bit as effective in shocking its host back to life. “In terms of sensitivity and specificity, identifying fibrillations and discharging appropriately, it’s equal in performance,” Mattioni says.
He also expects the device to shrink in size commensurate with improved battery science.
“And if you need to remove the whole system, it’s a simple procedure for the S-ICD, as opposed to the very risky extraction procedure involving [the traditional ICD],” he says.

I take leave of Mattioni’s office, not exactly sold on the S-ICD, but certainly more jazzed on the idea of an implantable heart-shocker than I was upon arrival. It seems likely that one day, science will improve ICD technology to the point that a high school athlete – or, for that matter, a pro baller, or an early-middle-age treadmill enthusiast – will be able to get a defibrillator implant and not necessarily have to curtail his athletic activity. I look forward to that mountaintop.
In the meantime, I suppose I should see if my PPO covers $50,000 heart-battery implants. But that’s another story altogether.