Inside Pediatrics Winter 2018

H earts 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, into the heart, which 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.

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 a 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 occur, 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

“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 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 Kylin was diagnosed with advanced heart failure with myocarditis. She was first placed on ECMO and then later the Berlin Heart once it was established ECMO would not provide long enough support. Photo submitted.

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