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Cardiology, Inside Pediatrics

Harmony Device Saves Children from Open-Heart Surgery

Harmony transcatheter pulmonary valve

In July 2021, interventional cardiologist William McMahon, M.D., Mark Law, M.D., and the Pediatric Cardiac Catheterization Lab team at Children’s of Alabama, snaked a catheter device through a vein in a 16-year-old girl’s leg up to her heart and replaced the valve. She went home the next day with just a small scar on her leg that will eventually become invisible. The U.S. Food and Drug Administration approved the device, called the Harmony transcatheter pulmonary valve, in March. Drs. McMahon and Law were the first cardiology specialists in a 10-state region to use it.

Fewer than 20,000 children are born each year with Tetralogy of Fallot, a condition marked by four major heart defects: ventricular septal defect, or a hole in the two lower chambers of the heart; a narrowing of the pulmonary valve and main pulmonary artery; malaligned aortic valve; and ventricular hypertrophy, or thickening, of the right ventricle. These children often need surgery soon after birth and a pulmonary valve replacement by the time they’re adolescents or young adults.

In the past, that meant another open-heart surgery and time spent on cardiopulmonary bypass, which carries significant risks of complications; a week or more in the hospital; scarring; weeks of recovery at home; and a low but real risk of death. In addition, because the children have already had major heart surgery, scar tissue from the previous procedure makes the valve replacement even more difficult.

In July 2021, however, interventional cardiologist William McMahon, M.D., and his colleague Mark Law, M.D., together with the Pediatric Cardiac Catheterization Lab team at Children’s of Alabama, snaked a catheter device through a vein in a 16-year-old girl’s leg up to her heart and replaced the valve. She went home the next day with just a small scar on her leg that will eventually become invisible.

The U.S. Food and Drug Administration had just approved the device, called the Harmony transcatheter pulmonary valve, in March. Drs. McMahon and Law were the first cardiology specialists in a 10-state region to use it. While similar devices have been available for pulmonary valve replacement, few children with Tetralogy of Fallot qualified because of their previous surgeries. Now, Dr. McMahon estimates that four out of five children with the condition will qualify.

The new procedure is a game-changer, he said. “We have many patients who live in fear of that surgery because they’ve been told they need another open-heart surgery since they were 8 or 10. We certainly have some patients who reasonably don’t want to do it and some who put it off. That becomes a problem because it means their heart is working harder.”

Dr. McMahon says the advantages to the Harmony device are obvious: “There’s a quicker recovery; lower risk of major complications and death; and they’re able to get on with their lives sooner.” The team has completed 10 procedures so far with no complications other than some arrhythmia that resolved with treatment.

Some patients returned to work or school three days after the procedure. And while the valve won’t last forever, Dr. McMahon expects a new valve could be inserted within the old one in the same manner. “That’s the overall goal of these valves,” he said. “To reduce the total number of heart surgeries that our patients need during their lifetime.”

William McMahon, M.D.

William McMahon, M.D., is an interventional cardiologist at Children’s of Alabama and a professor in the Division of Pediatric Cardiology, University of Alabama at Birmingham Department of Pediatrics.

Cardiology, Inside Pediatrics

Improving Quality and Outcomes in Cardiology

Ashley Moellinger, RN, CRNP, Cardiovascular Services, Children's of Alabama

Ashley Moellinger, RN, CRNP, Cardiovascular Services, Children’s of Alabama

Children’s of Alabama is deeply committed to continual improvement in every part of the care pathway. Two quality-improvement projects in cardiology are already showing the results.

Handoff of Care

Medical errors are the third-leading cause of death in the United States.[1] The Joint Commission reports that two-thirds of serious medical errors, or “sentinel events,” are tied to poor communication, and half involve communication during care handoff, such as when a patient is transferred from the intensive care unit (ICU) to surgery or back.[2]

The handoff is an important faultline for miscommunication that can lead to patient harm, said Children’s of Alabama cardiovascular intensivist Hayden Zaccagni, M.D. It’s not just communication between the intensivist and the surgeon; it involves the pediatric anesthesiologist, bedside and surgical nurses, advanced practice practitioners, and respiratory therapists.

“It’s a big team that cares for these patients,” Dr. Zaccagni said. Research shows that standardizing the handoff from the ICU to the operating room increases communication without delaying surgery and increased provider satisfaction and patient readiness for surgery while reducing errors.[3],[4]

The cardiology service didn’t have standardized protocol for handoffs, so Dr. Zaccagni, together with Ashley Moellinger, RN, CRNP, leda quality-improvement (QI) project to develop a process that prioritized clear, concise, and consistent communication from the cardiac ICU to the operating room or catheterization lab.

They started with a survey of 82 staff members, which found that 69 percent had experienced a safety event related to inadequate handoff. The survey also showed that communication was the primary barrier to transition followed by organizational barriers.

The team developed a tool and process for handoffs that involved all clinicians who interacted with the patient. “This multidisciplinary approach is so important,” said Moellinger.

Now, the night prior to surgery, the nurse practitioner, bedside nurse, and respiratory therapist complete a data form on the patient. The next day, the entire team meets at the bedside to review the form and bring up any concerns. “A big part of this is around situational awareness, or concerns we have about the patient that might not be obvious from reading through the chart or notes,” said Moellinger.When the patient is transferred, the team verbally goes through the tool again to ensure there are no outstanding questions or changes in condition.

The team is also tracking what it calls “moments of clarity”—when the process unveiled a potentially problematic issue such as a difficult airway, unavailability of vasoactive drip, patient cardiac arrest the prior night, or airway management for a patient with worsening oxygen levels.

The goal, or “smart aim,” was to demonstrate a standardized handoff in 80 percent of transition interactions, with 80 percent completion of patient data points by December 2021, and 95 percent compliance by July 2022.

Reintervention Reduction

This reintervention reduction QI project focuses on the most complex cardiothoracic surgery performed in newborns. Called the Norwood procedure, the surgery involves constructing a new, larger aorta for babies born with hypoplastic left heart syndrome. Nationally, patients who don’t require an intervention after their surgery have a mortality rate of about 6 percent compared to the 26 percent mortality rate in those who require another surgery or catheterization procedure.

The project, which is part of the National Pediatric Cardiac Quality Improvement Collaborative, was designed to understand why reinterventions occurred and identify opportunities to recognize the warning signs early in the post-operative period.

The Children’s team first performed a root-cause analysis of the 69 patients who required additional interventions between January 2015 and June 2020. That involved identifying what triggered the complication and how it could have been prevented. Of the 69 patients, 23 (34 percent) required an unplanned cardiac surgery or catheterization while hospitalized after the first-stage operation. Half of the surgical interventions were to explore unexplained bleeding, and half of the catheterization interventions were for conduit stenting to improve pulmonary blood flow. Fewer than five patients (12.5 percent) who required a reintervention died compared to none in the other group.

Reviewing the entire care pathway from the cardiovascular ICU to the operating room and back, including rates of post-operative bleeding and the timing for administering blood products, “we were essentially able to come up with a solution that we should communicate more effectively between team members in the operating room,” said Dr. Zaccagni. One way to improve communication is to wait at least 30 minutes in the operating room after closing the sternum to estimate chest tube output. Another is to standardize blood work when a patient is bleeding in case it’s due to a rebound effect of blood thinners given during the surgery. In addition, the team developed a standardized tool for the post-operative debriefing with the entire team.

The efforts are already paying off, said Moellinger, with fewer reinterventions since they began in 2020. “Standardization and, thus, reducing variation in everything we do is an important component for the best outcomes,” she said.


[1] Makary MA, Daniel M. Medical error—the third leading cause of death in the US. BMJ. 2016;353:i2139.

[2] The Joint Commission. Inadequate hand-off communication. Sentinel Event Alert. September 12, 2017. Issue 58.

[3] Caruso TJ, Marquez S, ,Luis J, et al. Standardized ICU to OR handoff increases communication without delaying surgery. Int J Health Care Qual. 2017;30(4):304-311.

[4] Joy BF, Elliott E, Hardy C, Sullivan C, Backer CL, Kane JM. Standardized multidisciplinary protocol improves handover of cardiac surgery patients to the intensive care unit. Pediatr Crit Care Med. 2011 May;12(3):304-8.

Cardiology, Inside Pediatrics, Pulmonology

Saving Children with Pulmonary Hypertension – One Patient at a Time

Bennett_Pearce_MD_600x284

Children’s of Alabama cardiologist Frank Bennett Pearce, M.D.

When the cardiology team at Children’s of Alabama heard the family history of a 6-year-old boy who presented with an episode of syncope, they knew immediately what was wrong. His father had undergone a double lung transplant at the University of Alabama at Birmingham to cure his pulmonary hypertension (PH). Now his son had been diagnosed with the same thing.  

But that wasn’t the only problem. The boy had also developed a supraventricular tachycardia requiring radiofrequency ablation, which was successful. 

“So we cured that,” said Children’s cardiologist Frank Bennett Pearce, M.D., the boy’s cardiologist. But then the patient continued having episodes of syncope, particularly during exertion. “When that happens in patients with PH, it’s because the blood can’t get through the lungs to the left side of the heart, limiting cardiac output,” said Dr. Pearce. To address that problem, Dr. Pearce and his team performed an atrial septostomy, creating a tiny hole between the atria in the atrial septum. Second problem fixed. 

Discharged on oral medications, the child did well with close follow up for several years, said Dr. Pearce, although he was vulnerable to pneumonia and other infections.  

Then in 2020, at age 13, he took a turn for the worse. “There are three principal metabolic pathways involved in treatment of PH,” said Dr. Pearce. Two—endothelin and phosphodiesterase—have effective oral drugs for treatment. The third, the prostaglandin pathway, is more difficult to address, he said. In the past, it required a central line for IV infusions of treprostinil, a prostaglandin pathway medication. “Most families are very reluctant to go to the central line because it creates major problems in their lifestyle and is a quantum leap in terms of the negative effects on these children,” he said. 

However, treprostinil can also be administered subcutaneously through a small catheter and external pump, much like an insulin pump. Unfortunately, the day the teen was scheduled for cardiac cath and initiation of subcutaneous treprostinil, he became very cyanotic. “We didn’t think it was safe,” Dr. Pearce said. Instead, the boy was admitted to the CVICU on inhaled and oral prostacyclin inhibitors. Despite increasing the dosage, his disease progressed. Finally, the team put him on the intravenous form of treprostinil, and he improved. Eventually, they were able to transition him to the subcutaneous form of the drug via the pump, and he became the first patient at Children’s to be initiated onto subcutaneous treprostinil. 

He’s now home and undergoing evaluation for a lung transplant. “He’s a typical teenage kid but able to deal with all these challenges and keep a pretty good attitude, thanks to support from his family,” said Dr. Pearce. “He just hangs in there.” 

Cardiology, Inside Pediatrics

T3: Clinical Decision Support Platform in the CVICU Improves Monitoring; Reduces Problems

Doctors-Cardiovascular-ICU-Monitoring-Etiometry-Resized

Right, Santiago Borasino, M.D., and left, Hayden J. Zaccagni, M.D.

An enduring issue in pediatrics is that so much of the technology used was developed for adults and must be retrofitted for children. That’s why the new software the cardiovascular ICU (CVICU) at Children’s of Alabama received for its monitoring devices is such a big deal. “It’s unique to the pediatric cardiovascular ICU population because it was developed initially for that population,” said cardiac intensivist Hayden J. Zaccagni, M.D.  

The software, called T3 (trajectory, target and trigger), was developed by ICU software development company Etiometry with input from some of the top children’s hospitals in the country. Dr. Zaccagni calls it a “situational awareness tool,” one that pulls information from the telemetry unit, ventilator and other machines and displays all the data on one screen. It also provides past data and trends and uses algorithms to warn staff of the probability of low oxygen levels in the venous blood (a surrogate of a lower-functioning cardiac pump). 

With 20 beds in the CVICU that are nearly always filled, anything that makes it easier for the team to track potential problems and potentially improve outcomes is a boon. 

The software’s greatest value comes from the historical information it provides, said Dr. Zaccagni. Physicians and nurses can then use objective data to review events and improve their knowledge about what happened and why, he said. For instance, they use the data during extensive reviews of all cardiac arrest occurrences. “It shows the baseline, what happened before the arrest and what happened that led to the physiology failure,” he said. And that can identify opportunities for improvement. 

“It provides two things we didn’t have before,” said cardiac intensivist Santiago Borasino, M.D. “The ability to see multiple vital signs trend at the same time on the same screen and the ability to see trends over time right at the bedside without running a report on a different computer.” 

“Sometimes the vital signs might look normal, but that might be very abnormal compared to what the patient was experiencing six hours ago,” Dr. Borasino said.  

It also improves rounding communication and efficiency, enabling the entire team, including the attending physician, fellow, bedside nurse, dietician, respiratory therapist and pharmacist, to view the patient’s history for the past 12-24 hours at the same time. “We want everyone on the same page,” Dr. Zaccagni said. “The goal is that everyone is aware of patients at risk for having an untoward event.” 

The other advantage is the software’s ability to continually recalculate the algorithm based on the changes in vital signs. “As intensivists, we’ve been trained to put together information from different sources to create a picture of whether the patient is fine or not fine,” Dr. Borasino said. But those mental algorithms are fraught by memory and sometimes wrong. The software, however, provides robust data analyses and assigns a probability of a problem. 

“The ability to view the history, see multiple vital signs at the same time and see data trends, I think, provides better monitoring and helps us find problems when they’re small rather than when they’re big,” said Dr. Borasino. “Everyone knows when the patient is crashing; the algorithm and the way the information is laid out in front of us might help us act before the patient is crashing.” 

Cardiology, Inside Pediatrics

Children’s of Alabama Receives Prestigious Accreditation from Children’s Cardiomyopathy Foundation

Cardiomyopathy_WEB

Livie Wheeler of Pell City, Alabama was diagnosed with dilated cardiomyopathy and underwent a successful heart transplant at Children’s of Alabama. Livie was discharged home just in time for her second birthday.

Children’s of Alabama was recently named an accredited center of care by the Children’s Cardiomyopathy Foundation (CCF), a national nonprofit committed to improving the health outcomes and quality of life for children with cardiomyopathy.

“We’re glad to be able to participate,” said F. Bennett Pearce, M.D., outgoing medical director of the Pediatric Advanced Heart Failure and Transplant Program at Children’s of Alabama. “CCF promotes education and helps families connect and choose centers with experience in these kinds of patients.”

The Pediatric and Congenital Heart Center of Alabama at Children’s of Alabama is one of the largest pediatric cardiovascular programs in the Southeast. In 2018, its team of 250 dedicated professionals treated more than 230 patients with pediatric cardiomyopathy. The team includes surgeons, intensivists, cardiologists and many others, including nurses, social workers, child life specialists, genetic counselors, nutritionists, occupational and physical therapists, and chaplains.

“We have traditionally had very strong clinical abilities and success with treating a variety of these conditions,” Pearce said. “Over the 25 years that I’ve worked in the program, I feel we have been among the finest centers, but we have not been quite so active in getting the word out. That needs to change because we want to make families aware that there’s a good resource for them here.”

The CCF offers a plethora of educational and supportive programs for families, as well as a research grant program for basic, clinical, population/epidemiologic, or translational studies focused on primary pediatric cardiomyopathy.

Its accreditation program was established in 2017 to recognize excellence in diagnosing and treating pediatric cardiomyopathy and provide families with standardized center information to help them find an expert treatment center in their area. To date, 39 centers in the U.S. and Canada, including Children’s of Alabama, have been accredited.

The CCF has very strict criteria for accreditation. Centers must:

  • Manage 30 or more cardiomyopathy patients up to age 18 annually or 60 pediatric cardiomyopathy patient visits a year.
  • Have at least one pediatric cardiologist who treats patients with dilated, hypertrophic, restrictive arrhythmogenic and right ventricular or left ventricular non-compaction cardiomyopathy.
  • Be part of a teaching hospital affiliated with a medical school.
  • Offer the following:
    • Prenatal or fetal echocardiography
    • High quality imaging (echocardiogram, cardiac magnetic resonance imaging)
    • Interventional cardiology (catheterization, endomyocardial biopsy)
    • Pediatric cardiac electrophysiology
    • Cardiac surgical services (septal myectomy, cardiac device implantation)
    • Genetic testing and counseling
    • Pediatric neurology
    • Social work
    • Child life services
  • Centers must also have at least one of the following (Children’s of Alabama meets all four):
    • A specialized clinic or program focused on pediatric cardiomyopathy or familial hypertrophic cardiomyopathy with a dedicated team of professionals.
    • Involvement with the North American Pediatric Cardiomyopathy Registry, Pediatric Cardiomyopathy Repository or Pediatric Heart Transplant Study Group.
    • Engagement in pediatric cardiomyopathy research.
    • Advanced heart failure management and transplantation.

“We have everything needed to offer to this group of patients,” Pearce said. “And we want to make sure that when a family confronts this difficult diagnosis, they can easily find our program and be assured that it is a good resource.”

 

Cardiology, Inside Pediatrics

Discharged with an iPad: Children’s of Alabama Uses Telehealth to Monitor Complex Heart Patients at Home

Telehealth_WEB

Children’s of Alabama has partnered with Locus Health to provide a special iPad app that connects parents with nurse practitioners who treat infants who have undergone complex surgery.

Babies born with a single ventricle must undergo three major open-heart surgeries by the time they are toddlers. The first and most complex surgery occurs at 1 to 2 weeks; the second between 4 and 12 months. The months spent at home between the two can be overwhelming for parents.

Now families served at Children’s of Alabama have a new tool to help them cope – an iPad containing a special app from Locus Health, a Charlottesville, Virginia-based company that develops software to ease the discharge process and transition from hospital to home. The app forms the core of a remote monitoring system that connects parents with the nurse practitioners at Children’s of Alabama who care for their infants.

“These parents have been through a tremendous amount of stress,” said Katelyn Staley, discharge coordinator for Cardiovascular Services at Children’s of Alabama. “Not only do they have a newborn, but the baby requires major open-heart surgery in that first week or two of life. Then they are discharged home; it’s an overwhelming process,” she said.

“The Locus platform was designed specifically for the pediatric patient population with congenital heart disease,” said Sarah Blair, RN, MSN, CRNP, of Children’s of Alabama’s Hearts at Home Program. More than a dozen of the country’s leading children’s hospitals now use the system, which studies find can reduce post-discharge emergency room visits as much as 40 percent and the total hospital days by up to two weeks.

Children’s of Alabama had been using another electronic program, but it was cumbersome, not user-friendly and difficult to extract data from. Before that, all data was collected the old-fashioned way – with paper and pencil.

With the Locus app, parents enter their child’s daily weight, oxygen saturation, heart rate, number of diapers, Synagis dosing and nutritional intake, noting if there is any vomiting or diarrhea. They can also upload photos and videos.

Timely information is critical. For instance, weight gain is vitally important because if the baby stops gaining or loses weight the team needs to intervene quickly before complications occur. In addition, values can be individualized for each infant depending on their medical status. “If a parent enters an out-of-range value it creates a red flag and prompts the caregiver to call the hospital immediately,” Staley said.

The data automatically populates the congenital heart clinical dashboard, which nurse practitioners and clinical nutritionists monitor. Parents can also see current and past data and even track trends across time, Blair said. Data can also be downloaded into a PDF and emailed to physicians.

The remote monitoring is also beneficial since many patients live hours from the hospital and may be followed by a local cardiologist. “Now we can share the information with the cardiologist where they live,” she said.

“It definitely keeps us in constant communication with the families,” Blair said. “We still call and talk to them, but it relieves some of that pressure.”

“Sending families home with the reassurance that nurse practitioners are logging into the system on a daily basis and that they have 24/7 access to a provider is very reassuring,” Staley said.

Cardiology

Thanks to Team-Oriented Approach, Heart Transplant Program Leads Country in Outcomes

transplant_team

The Pediatric Advanced Heart Failure and Transplant Team at Children’s Alabama, seated, left to right: Sally Smith, DNP, CRNP, CCTC; Meloneysa Hubbard, MSN, CRNP, CCTC; and Kimberly Sullivan, MSN, CCTC, CRNP. Standing, left to right: Mariah Strickland, MSN, CRNP; Waldemar F. Carlo, M.D.; David C. Mauchley, M.D.; F. Bennett Pearce, M.D; and Robert J. Dabal, M.D.

The numbers tell the story of the heart transplant program at Children’s of Alabama.

• 176 pediatric heart transplants since 1981
• 59 transplants since 2012
• Zero deaths since 2014
• A 97 percent one-year survival rate over the last decade — considerably higher than the national rate of 90.2 percent

One major reason? “We have a very cohesive, experienced, stable team,” said Medical Director F. Bennett Pearce, M.D. That team includes surgical director Robert J. Dabal, M.D., pediatric cardiologist Waldemar F. Carlo, M.D., and pediatric surgeon David C. Mauchley, M.D., as well as four cardiac nurse practitioners, three of whom are certified clinical transplant coordinators.

Until 2012, the pediatric transplant program was housed at the University of Alabama at Birmingham (UAB). But with the opening of the new children’s hospital that year, the transplant team was able to create a separate entity and move into a state-of-the-art cardiac care facility at Children’s. Today, it serves as a regional referral center and is the only pediatric heart transplant program in the state. The program also provides comprehensive care for patients with advanced heart failure using evidence-based medical management and mechanical circulatory support. Its surgeons also perform heart transplants, including ABO incompatible transplants, in infants.

Although the United Network of Organ Sharing (UNOS) considers the Children’s program to be a relatively young program, Pearce said, “the reality is far different” given the team’s depth and length of experience. The team is also diverse, he said, bringing different viewpoints and interests, which expands the program’s potential.

Moving to Children’s brought several advantages, he said, particularly access to pediatric specialists. “We can provide better multidisciplinary care for patients,” he said. The program also now has its own administrative structure and identity, providing families with a central place to call with questions or concerns.

The center also features dedicated social workers, child life therapists, physical/occupational therapists, dietary counselors, psychosocial counselors, specialty pharmacists and pastoral care. “We realize that when a patient is listed for transplant that you’re entering into a relationship the family as well as the patient,” Pearce said, “and we do all we can to optimize that relationship.”

Research is a major part of the program, he said, with ongoing studies on cardiomyopathy, pulmonary hypertension and heart transplantation. For instance, one major study is evaluating alternative immune suppression techniques for post-transplant children. The center also participates in numerous quality initiatives with other transplant programs around the country in order to identify best practices.

Largest Pediatric Heart Transplant Registry in the World

UAB houses the international Pediatric Heart Transplant Society, which maintains the largest registry in the world on heart transplantation. Data from the registry is used to encourage and stimulate basic and clinical research in the field of pediatric heart transplantation and to promote new therapeutic strategies. Since its founding in 1993, data from the registry has been used to produce more than 100 abstracts and presentations, and 87 publications. Today, 56 centers participate in the registry, which contains information on more than 6,542 transplants. James Kirklin, M.D., who was surgical director of Adult and Pediatric Heart Transplant program at UAB and Children’s until his retirement from clinical work in 2017, initiated the registry.

Cardiology Heart Transplant Chart

The Heart of It All
Visit www.childrensal.org/advanced-heart-failure-and-transplant to learn more about the Pediatric Advanced Heart Failure and Transplant team at Children’s of Alabama.

Cardiology

‘Not-So-Stressful’ Stress Testing Evaluates Pediatric Congenital Heart Disease and Exercise-related Complaints

exercise_labPut a seemingly timid child with congenital heart disease (CHD) on a treadmill, attach electrodes to their chest and place a mask over their mouth and nose to measure gas exchange, and it’s amazing what you can learn about their cardiovascular and pulmonary health.  That’s the premise behind Children’s of Alabama’s new Cardiometabolic Exercise Testing Lab, which opened this summer. The lab was made possible through the generosity of Gene and Leslie Cash in loving memory of their daughter Kelly Cash.

“Exercise capacity can be highly predictive of the risk of complications related to CHD, including developing heart failure,” said pediatric cardiologist Camden Hebson, M.D., who runs the lab. “Given that the heart and lungs are usually the limiting factors on the extent of exercise capacity, stress testing is a wonderful way to evaluate how well patients are supported.”

In contrast, evaluating a child at rest, as with an EKG and echocardiogram, “is like evaluating the quality of a car when it’s in the shop versus taking it out on the road and seeing how well it drives,” he said.

While the test mimics the traditional stress test, the use of the mask to measure oxygen consumption (VO2) and CO2 elimination is particularly important, Hebson said.

“These become the key variables to say from a quantitative standpoint how well the heart and lungs support you as you exercise,” he said.

Children who have been living with a heart condition for years may underappreciate the severity of their symptoms, he said. The exercise test, however, provides objective data that can demonstrate problems and allow intervention.

“Many patients become accustomed to how they feel with exercise, yet with stress testing we can identify limitations early on and thus intervene in a timelier manner,” he said.

The test also enables serial assessments to assess function over time.

In some instances, he said, a patient’s symptoms are attributed to cardiovascular function, but the stress test can show it was related to pulmonary function instead.

“Sometimes that’s hard to tease out because we’re always focused on the heart,” he said. “Exercise testing offers a way to make that connection.”

Children as young as 7 years old can complete the test, he said, and those that can’t walk or run on the treadmill can use a bike. The key point is that “they need to be comfortable enough to come in and wear a mask, EKG leads, etc., while exercising,” he said.

To take some of the fear and anxiety out of the experience, the team has decorated the room to look more inviting with posters of sports figures and decals. They even added a scoreboard where kids can write their “code name” and see how they compare to others taking the test. The goal,  Hebson said, is to make the lab look less like a clinic or hospital setting and more like a place to have fun.

The test itself takes about 15 minutes, with another 30 minutes for the setup and explanations. It is typically covered by insurance.

Hebson, nursing staff, and parents are always in the room. As he noted, “We try to make it the least stressful as possible, even though it’s a stress test.”

Interested in a Pediatric Cardiology Fellowship at Children’s of Alabama?
Learn more at childrensal.org/pediatric-cardiology-fellowship.

Cardiology

Kylin’s Brave Heart

kylin_heart

Hearts are full at Children’s of Alabama because Kylin Harris’ heart is well. Inside a hospital conference room in June 2018, the banner was hung, the tablecloth was draped and the cake was cut in celebration of Kylin, the first-ever patient at Children’s to be successfully weaned from a pediatric ventricular assist device without the need for a heart transplant. The 1-year-old guest of honor arrived in what is by far Children’s most popular mode of transportation – a red Radio Flyer wagon – a stark contrast from her arrival just four months prior.

Kylin was flown by helicopter to Children’s after her mother, Keianna Harris, came home to find her daughter uncharacteristically lethargic. Kylin hadn’t slept well the past two nights and a startled Harris, a nurse by trade, took Kylin to their local emergency room. While en route to the hospital, Kylin had a seizure and fell limp.  Doctors at Children’s intubated Kylin and later diagnosed her with advanced heart failure due to myocarditis. Kylin experienced cardiac arrest while being transferred to the Cardiovascular Intensive Care Unit. Doctors performed chest compressions and CPR, and placed her on extracorporeal membrane oxygenation therapy (ECMO), which pumps and oxygenates a patient’s blood outside the body allowing the heart and lungs to rest for a limited time.

The Pediatric Advanced Heart Failure and Transplant Team at Children’s discussed every detail of Kylin’s care with Harris. It was apparent ECMO would not provide long enough support for Kylin’s heart. First, doctors would convert Kylin from ECMO to the Berlin Heart® EXCOR, a long-term cardiac assist device that functions as a heart outside of the body. The Berlin Heart acts as a bridge to transplantation for children in heart failure, allowing the patient mobility and freedom to rehabilitate in preparation for transplant surgery. Available in several sizes, the Berlin Heart is not totally implanted inside the body. Doctors insert cannulas, or flexible tubes, in the heart and they extend through the skin and connect to a small pump located outside the body. That pump, along with its computerized drive unit, maintains blood flow.

“It was a lot to take in … It all happened so fast,” Harris said. “My biggest fear was losing her. It was scary not knowing whether she was going to make it through.”

The Pediatric Advanced Heart Failure and Transplant Team, a partnership between Children’s and the University of Alabama at Birmingham (UAB),  is among the first in the U.S. to use the Berlin Heart in children and reported the first successful Berlin Heart bridge to transplantation for a child with a single ventricle. Children’s and UAB first used the device in 2005, when UAB was home to the pediatric cardiac unit. The following year, the team weaned its first patient, now an adult, from the Berlin Heart without the need for transplant at UAB. In 2012, with the opening of the Benjamin Russell Hospital for Children and Bruno Pediatric Cardiac Unit, all pediatric cardiac care to relocated to Children’s.

The team’s success with the Berlin Heart led to a landmark study published in the New England Journal of Medicine in 2012 featuring two team members as co-authors – pediatric cardiologist F. Bennett Pearce, M.D., and David Naftel, Ph.D, professor in the UAB Division of Cardiothoracic Surgery. The research team, known as the Berlin Heart Study Investigators, evaluated ECMO and the Berlin Heart to see which offered children the best chance of survival until they could receive a new heart or recover enough heart function not to need a support device or transplant.

Pearce says 46 percent of children diagnosed with heart failure die or receive a heart transplant within the first five years after diagnosis. A heart transplant is a child’s best hope of survival, with the survival rate after a transplant estimated at 83 percent at three years. However, with limited donor hearts available, the wait is often long.

“Children on waiting lists for heart transplants experience the highest waiting-list mortality for any age or organ,” Pearce says. “This research demonstrates that the Berlin Heart, available in variety of appropriate sizes for children, has the potential to effectively and safely bridge children from diagnosis to transplantation or in some cases recovery, for long periods of time.”

Before the study and subsequent approval of the Berlin Heart by the U.S. Food and Drug Administration, ECMO was the mainstay for mechanical circulatory support as a bridge to transplantation. The effective period of support with ECMO is typically limited to 10 to 20 days before serious complications such as bleeding and major organ system failure, often prohibiting transplantation. The short duration of support afforded by ECMO is often inadequate, the study reads, citing only 40 to 60 percent of children requiring support with ECMO survive long enough to undergo heart transplantation. Researchers found that the longest duration of support for the younger, smaller children was 174 days for the Berlin Heart and 21 days for ECMO therapy. The longest duration of support for the larger, older children was 192 days for the Berlin Heart and 28 days for ECMO.

“The Berlin Heart provided better survival over longer support periods than ECMO therapy,” Pearce says. “These longer support periods on the Berlin Heart allow for patient rehabilitation, improved nutrition and weaning from ventilator support to use of the patient’s own respiratory system so that the children may be better candidates for successful transplantation or so that long-term cardiac recovery can occur.”

Kylin lived with the Berlin Heart for 83 days. In that time span, doctors noted signs of cardiac recovery and further testing gave doctors the green light to again operate on Kylin to remove the device. Pearce credits fellow pediatric cardiologist Waldemar F. Carlo, M.D., who was the first to see hints of Kylin’s recovery.

“He was the strongest advocate for us to explore whether her degree of recovery was enough for her to be off the device,” said Pearce, adding Kylin’s echocardiogram and catheterization data yielded positive results. “We followed the protocol of the Berlin Heart Clinical Support Team. We were in daily communication with the team from the moment we considered weaning her off.

“She has continued to show us normal cardiac function. We removed her from the transplant list because she had improved so much. We’re following her closely, but our hope is she won’t require a heart transplant,” Pearce said.

And much to Harris’ delight and relief, her baby girl did make it through. Kylin is her old self again, she says, smiling from ear to ear.

“When Kylin was in the hospital, she wouldn’t smile. She felt miserable,” Harris said. “But now her personality is back. She’s been grinning ever since we left.”

Joseph S. Bruno Pediatric Heart Center
Learn more about the services provided by the Bruno Pediatric Heart Center at Children’s of Alabama at https://www.childrensal.org/heart-inpatient-services.