UI team to the rescue of children with heart problems
By David Pedersen
For Amanda Kurovski, the “episodes” began in 2006, when she was 15.
“Some mornings, as I was waking up, I’d just stop breathing,” she says. “Afterwards, I couldn’t remember where I was. Sometimes I couldn’t even remember who I was.”
The Des Moines teenager saw medical specialists and underwent tests. Was she experiencing seizures, or possibly symptoms of sleep apnea? All Kurovski knew was the episodes occurred without warning.
“It was pretty scary, because no one knew what was happening,” she says.
After another frightening blackout at home one evening—her father called 911 after finding her unresponsive on the living room sofa—Kurovski was hospitalized at Mercy Medical Center in Des Moines.
She was diagnosed with long QT syndrome, a disorder of the heart’s electrical system that causes sudden arrhythmias, which can lead to fainting spells, seizures, and even sudden death. In Kurovski’s case, she inherited the disorder from her father.
A day after arriving at Mercy Des Moines, Kurovski was transferred to University of Iowa Children’s Hospital, where pediatric cardiologist and arrhythmia specialist Ian Law (’88 BSE, ’93 MD) placed an implantable cardioverter defibrillator (ICD) in her chest.
When a middle-aged or older adult receives an ICD—typically after surviving a heart attack or living for years with tachycardia—learning that a device can manage their irregular heart rhythm is like winning the lottery. For a child or teenager, however, an ICD can bring psychological and social challenges typically not seen in older adults—feelings of “Why me?” as well as body image, self-esteem, and privacy issues.
The ICD procedure traditionally leaves a noticeable scar on the patient’s chest—a visible reminder, along with a noticeable “bump” outlining the device itself, that the patient has a heart condition. Minimizing the physical appearance of defibrillator placement can go a long way in young patients “accepting” the device. For most of these cases, the UI team makes the incision along the anterior axillary line just in front of the armpit, and the ICD typically is placed under the pectoral muscle. The result is a scar that’s less noticeable, if at all.
Law and other members of the UI pediatric electrophysiology (EP) program, in collaboration with researchers at Denver Children’s Hospital and other institutions across the country, are nearing completion of a large prospective study on the psychosocial impact of scar location in young ICD patients using quality-of-life surveys. The results should provide clinicians and families with additional insight into young patients’ attitudes and adjustments to life with a heart device.
For Kurovski, a less-visible scar suited her fine.
“Given that I was still in high school—with homecoming and prom and all that still to come—I was glad it didn’t show,” she says.
As an active teenager, Kurovski was determined not to let an ICD prevent her from participating in track, volleyball, color guard, and cheerleading. After the family had a long discussion with Law about the risks of sports participation, Kurovski convinced her parents, and together they signed paperwork indicating they would accept any what-ifs, including the possibility of a transiently disabling ICD shock during competition or damage to the ICD system itself.
“My parents were more afraid than I was. I just didn’t want to give up sports,” Kurovski says. “Thankfully, I never did get a shock during a practice or a game.”
Having now lived with her ICD for eight years, Kurovski says she’s used to it. She feels the device under her skin when she turns in her sleep but considers this a normal occurrence. This past summer, she received a replacement ICD during an outpatient procedure in Iowa City.
Kurovski remains physically active, and she participates in the multi-center ICD Sports Registry study.
“She’s a poster girl for the study,” says Jean Gingerich, BSN, RN, a nurse clinical specialist in the pediatric EP program at UI Children’s Hospital, pointing to a 2007 Associated Press story that appeared in USA Today. The story quoted Kurovski and pictured her holding a teddy bear she received after getting her ICD.
And as it’s been for the past eight years, she receives a shock if her heart rate goes higher than 200 beats per minute. The device will pace her heart if her rate drops below 50. For Kurovski, such moments are part of life’s rhythm.
She’s a role model for other young heart rhythm patients—as a past presenter and regular attendee at the annual “Young Hearts With ICDs” conference. And when she sees her UI pediatric care team for a follow-up appointment, “I’ll talk with younger girls who are getting an ICD, or just got one,” Kurovski says. “I like being in ‘Peds,’ even though I’m 23.”
When Law joined the pediatrics department in 2000, he looked to grow the number of interventional procedures the pediatric EP specialists performed. In the two years before Law came to the UI, the group had done fewer than two dozen pacemaker or ICD placements or catheter ablation procedures to treat arrhythmias.
Today, the pediatric EP program at UI Children’s Hospital is considered one of nation’s leaders in terms of case volume—more than 300 procedures a year, which is comparable to children’s hospitals in Michigan, Atlanta, Boston, and Texas.
The UI team’s success is due, in part, to a commitment to outreach across Iowa, in several states, and on medical relief mission trips to Merida, Mexico. Working since 2004 with a Des Moines pediatric cardiology group that has made the Mexico trips for more than 25 years, the UI team’s every-other-year effort serves several hundred underserved patients over the course of a week, with the UI specialists performing as many as 20 device placement or ablation procedures.
The expertise that comes from experience has helped move the Iowa team to the forefront of centers dedicated to new ways of fixing heart rhythm problems in young patients. Over the past decade, the UI group has been the first in Iowa—and among the first in the nation—to implement radiation-free cardiac ablation procedures for arrhythmias.
When an arrhythmia disrupts the heart’s electrical system, in some cases, it’s like a short circuit that allows the electrical system to loop back onto itself within the heart. A cardiac ablation is a catheter-based, non-surgical procedure that uses an energy source at the tip of a catheter to deaden small areas of tissue along electrical pathways of the heart—similar to a road block on a race track—in order to interrupt the heart’s abnormal electrical conduction routes that are causing the rhythm problems. Ablations initially were performed primarily using heat in the form of radiofrequency energy, but now many specialists use a cryothermal approach to “freeze” the abnormal electrical signals, especially in areas that are close to normal conducting pathways.
Traditionally, cardiac catheterization for ablation procedures used fluoroscopy—continuous X-ray images—which was not optimal for pediatric patients, says Nick Von Bergen (’02 MD), UI pediatric cardiologist and EP specialist.
“With fluoroscopy, you’re using X-rays to help you determine where you think the catheters are,” he says. “You can see them, but it can feel like you’re looking from space. Plus, we’re talking about the use of radiation, which is not ideal.”
By the late 1990s, new technologies such as 3-D mapping systems, used in tandem with fluoroscopy, provided greater accuracy in identifying catheter locations.
“By adding 3-D mapping, a patient’s exposure time to radiation was reduced, which was great,” Von Bergen says. “But after a while, we started asking ourselves, ‘Do we need to use radiation at all?’”
Beginning around 2006, the Iowa team—along with just a handful of other pediatric EP programs across the country—was among the first to incorporate a radiation-free approach for certain ablation procedures. Today, about three-fourths of their cases are done without the use of fluoroscopy. For the remaining procedures that still require X-rays, the length of radiation exposure is dramatically reduced, typically less than a minute.
“It’s a great example of embracing technology and innovation, but this isn’t simply ‘ooh-ahh’ stuff,” Law explains. “There’s a real benefit for patients, and not just pediatric patients. Our colleagues on the adult intervention side have reduced their patients’ radiation exposure times, as well, based on the results we’re seeing. And in fact, we’ve become a teaching center, where specialists from other institutions have turned to us to learn how to incorporate a radiation-free approach.”
Kate and Cory Simpson of Fort Dodge, Iowa, were expecting their first child together in spring 2012. At Kate’s 36-week checkup, her obstetrician noted that the baby’s heart rate had spiked briefly.
At Kate’s next appointment the following week, the baby’s heart rate raced to 180 beats per minute. The care team worked quickly to move Kate from the clinic to the facility’s birthing center. When the baby’s heart rate reached 220, the decision was made to deliver by C-section.
Chloe Simpson was born at 6:01 p.m. She looked fine—good color, no signs of distress. Best of all, her heart rhythm was normal.
But by 10 p.m., Chloe’s heart rate started climbing again. She was flown in the middle of the night to Blank Children’s Hospital in Des Moines. When Chloe arrived, her rate was approaching 280 beats per minute—well above the “normal” rate range of 100 to 200 beats per minute for a newborn like Chloe—and she was showing signs of distress. Her Des Moines medical team tried defibrillation six different times to reset the speeding heart rhythm.
“They vented her and tried everything until they finally stabilized her,” Kate says.
The Simpsons learned that Chloe had atrial ectopic tachycardia, a specific type of arrhythmia involving the atrium, or upper two chambers of the heart.
Normal electrical conduction in the heart begins with a “spark” of electricity in the sinus node, located in the upper right atrium. The signal moves from the sinus node through the atrium and is transmitted to the AV node to the ventricles. As the electricity moves through the ventricles, the heart muscle contracts. Chloe’s problem was an “extra” spark that beat too quickly, causing her erratic heart rhythm.
The Des Moines pediatric cardiologists, in consultation with the UI pediatric EP team, tried different medication combinations to keep Chloe stabilized and help her grow. The newborn looked healthy, but her heart output tests showed otherwise. After three weeks, when it was clear that medications would not correct the problem, Chloe was transported by ambulance to UI Children’s Hospital.
Law and Von Bergen’s plan was to first monitor Chloe while adjusting her medications, giving her more time to grow. Over time, however, her heart function began declining to a dangerous level.
At 5 ½ weeks old, Chloe became the youngest and smallest baby in the world to undergo a catheter ablation with 3-D mapping at UI Children’s Hospital. She weighed just over 7 pounds. Using 3-D mapping technology rather than X-rays, the team was able to pinpoint the location of the abnormal area causing the elevated heart rate. With delicate precision, radiofrequency energy was applied at the tip of a catheter to “burn” the tiny bit of tissue until it no longer produced the hyperactive electrical spark.
Within seconds, Chloe’s heart returned to a normal rhythm. A few days later, her heart function was back to normal, and she was cleared to go home.
The Simpsons received another scare about five weeks later when Chloe’s heart rate spiked again. Tests revealed an entirely different, yet relatively common, tachycardia, which may have been blocked (and thus, undetected) by medications administered during Chloe’s initial hospitalization.
Fortunately, this time medications worked to control the tachycardia. Chloe continued drug therapy for nearly a year until she literally outgrew the disruptive signal. Since August 2013, it’s been smooth sailing and no meds for the toddler.
“We no longer need to listen to her heart every day,” Kate says, “but I still check it a lot. I probably always will.”
Are sports OK for ICD patients?
By David Pedersen
Should people with implantable cardioverter defibrillators (ICDs) participate in competitive sports or strenuous physical activity? It’s a question that’s challenged clinicians, researchers, parents, and athletes ever since the devices were developed more than three decades ago.
An ICD delivers a jolt of electricity to the heart of an arrhythmia patient when it detects an irregular or rapid heartbeat. Hence, the consensus among medical professionals—and a 2005 recommendation from the American College of Cardiology/American Heart Association-sponsored 36th Bethesda Conference on eligibility guidelines for athletes with cardiovascular abnormalities—has been that individuals with ICDs should not play sports or engage in physical activity more strenuous than golf, bowling, or billiards.
For these patients, the risks associated with high-exertion activity are not fully known. Another concern is that devices could become damaged, malfunction, shock the heart inappropriately for a very fast (but otherwise normal) heart rate, or fail to convert an abnormal rhythm due to high adrenaline levels or other physiologic changes that can occur during sports.
For children with ICDs, not being allowed to play sports can be devastating, according to Ian Law, MD, director of the pediatric EP program at UI Children’s Hospital.
“If you take any competitive high school athlete and tell them they can no longer play basketball or run track, for example, their whole world shrinks,” he says. “Now imagine what it’d be like for a child with an ICD. It’s one more factor that can add to their stigma about being ‘different’—having a heart condition or looking or feeling different because they have a device.”
Despite the recommendations, some people with ICDs—adults and teenagers—continue to take part in vigorous, and even competitive, sports. To learn more about the safety and risks of sports participation for these patients, the UI is part of an international research study called the ICD Sports Registry.
Now in its eighth year and led by Rachel Lambert, MD, at Yale University, the multi-center study does not endorse competitive sports (other than golf) or less rigorous activities for ICD patients. However, it does encourage ICD patient-athletes to join the registry so researchers may gather information that ultimately helps ICD patients, their parents or family members, and their health care providers face play-or-don’t-play decisions. To date, more than 400 ICD patients worldwide, ages 10 to 60, who play sports or take part in strenuous exercise, have joined the registry.
Among the centers taking part in the research study, the UI site has enrolled the most pediatric patients in the registry, Law notes.
The study already has provided important information that supports people with ICDs who play sports. In a paper published in May 2013 in the journal Circulation, study authors report that athletes with ICDs can engage in vigorous sports without physical injury or risk of ICD failure to stop an arrhythmia, despite ICD shocks that may occur during these activities. Over a follow-up period lasting 21 to 46 months, there were no reported occurrences of death, resuscitated cardiac arrest, arrhythmia, or shock-related injury to study participants.
These results suggest reassessing guidelines that prohibit strenuous sports for all patients with ICDs, Law acknowledges, but he cautioned against advising patients to take up any sport they wish. “We’ll learn more as this study continues,” he says, “but for patients with ICDs who choose to play sports, the findings are encouraging.”
Patients, families bond at annual conference
By David Pedersen
Patient and family involvement is key to the “Young Hearts With ICDs” conference, held each fall in Iowa City and sponsored by the UI Children’s Hospital EP program.
In fact, a group of young patients was instrumental in getting the event off the ground more than a decade ago, says UI pediatric EP nurse clinical specialist Jean Gingerich, one of the conference’s coordinators.
“Several of our patients, mostly teenage girls, expressed a strong interest in meeting other patients their own age,” Gingerich recalls. “I suggested they draft a mission statement, not thinking much would come of it. About a week later, they responded, and my jaw dropped. Their statement showed such wisdom and sincerity—we knew right away we had to make this happen.”
The 11th annual “Young Hearts With ICDs” was held last September in Iowa City, The event is open to ICD patients and their families, with meals and hotel accommodations provided.
While the half-day conference includes physician-facilitated breakout sessions on topics like cardiomyopathies or the intricacies of device technology, it’s also purposely “low pressure,” Gingerich says, with plenty of time for informal discussions. There are short presentations by young patients who share their own medical histories, challenges, and triumphs.
“It’s so moving to hear them put into words their personal journeys,” Gingerich says. “When you’re in that room, you can hear a pin drop.”
The event also includes a guest attendee who offers motivational perspective and support. This year’s speaker was storyteller and performer Beth Horner. Past presenters have included the acclaimed “Armless Archer” and 2012 Paralympic silver medalist Matt Stutzman and Michaela Gagne, a former Miss Massachusetts who received an ICD when she was 17.
“We’ve had kids who’d become socially isolated after receiving an ICD come to this conference and by the next year, their parents say it’s been a complete turnaround—getting good grades and participating in activities again,” Gingerich says. “That’s why we do this. It’s possibly the most important thing we provide for our patients.”
In rhythm with Dianne Atkins
As a clinician, researcher, and a policymaker, Dianne Atkins, MD (’83 F), has helped shape scientific and public understanding of cardiac resuscitation, particularly as it relates to defibrillation and children. Earlier this year, Atkins received the Meritorious Achievement Award from the American Heart Association for three decades of service, which includes serving the AHA’s Council on Cardiovascular Disease in the Young, CPR guidelines committee, and as chair of the Science Subcommittee of the Emergency Cardiovascular Care Committee supporting an AHA goal to double cardiac arrest survival rates for children and adults by 2020.
Q: Briefly describe your work with automated external defibrillators (AEDs).
A: When AEDs were first developed, they were designed primarily for adults. As awareness about these devices grew—as did the calls for placing more AEDs in public places like schools, sports arenas and shopping malls—we needed to make sure these were also safe for children. One issue was whether the algorithms in the machine that decides to shock or not shock would work with children. That was a central question for several research papers I was involved in…we found that generally, they do. Another issue was how much energy should be delivered to a child? Our work, in collaboration with colleagues around the country and device manufacturers themselves, ultimately helped lead to advancements where you could use the same machine but reduce the amount of shock administered to a child. And this helped pave the way for AEDs to be placed in many different kinds of public settings.
Q: When did AEDs really take off?
A: I remember attending a conference around 1995 where people were saying that AEDs would soon be as prevalent and easy to use as fire extinguishers. That’s not quite been the case, but around the early 2000s, you started seeing greater awareness overall. There was a public access defibrillation trial supported by AHA, NIH, and the manufacturers demonstrating that people did better in communities that provided easy access to AEDs in addition to CPR. That’s important to note: You don’t want to simply distribute AEDs everywhere. You still need to know how to do CPR.
Q: Speaking of CPR, you’re on the AHA committee charged with reviewing the guidelines.
A: Yes. We revise the guidelines every five years. The last version came out in 2010, and we are heavy into the process of reviewing the scientific literature for the next set of guidelines, which will be published in October 2015.
Q: As for the AHA goal to double cardiac arrest survival rates by 2020?
A: If you look nationally, the survival for out-of-hospital cardiac arrest in most settings is about 10 percent—for adults and children. About seven years ago, the UI participated in a large, NIH-sponsored trial called the Resuscitation Outcomes Consortium; for our research group, I oversaw the database so we could develop a baseline. When we looked at our data—10 centers total, eight in the U.S. and two in Canada—there was a broad range of survival rates. Seattle/King County was one of the sites, and they really set the curve; Iowa was in the middle of the pack.
Q: What can we learn from Seattle?
A: If you have an out-of-hospital cardiac arrest in Seattle, your chances of being discharged from the hospital are 50 to 60 percent. That’s really impressive. How do they do it? They have a well-organized emergency medical services (EMS) system, a very active educational implementation system, and excellent coordination of care between EMS providers, ERs, and intensive care units. With trauma, you often hear about the “golden hour,” but with cardiac arrest, we’re talking minutes, really. Even though cardiac arrest is about 1 percent of their total EMS calls, if you call 911 in the Seattle area, the dispatcher will ask you two questions: “Is this patient awake?” and “If not, is the victim breathing?” If the answer to the second question is no, they talk you through the CPR steps over the phone. It’s the best place in the world to get bystander CPR, which we know is a huge factor in survival.