Pediatric heart transplantation and heart failure: Early referrals save lives
by Steven Zangwill, MD
Steven Zangwill, MD, is program director of Pediatric Heart Failure and Heart Transplantation and a pediatric cardiologist at Children's Hospital of Wisconsin. He also is an associate professor of Pediatrics (Cardiology) at The Medical College of Wisconsin and a member of Children's Specialty Group.
In the last 10 years, tremendous strides have been made in cardiac transplantation and heart failure, especially in the pediatric population. This is true for the state of the art in medical care as a whole, and perhaps even more so for our practice here at Children's Hospital of Wisconsin. Globally, heart transplantation and heart failure are far more prevalent in the adult population, so new evidence and innovation tends to trickle down from adult experiences to pediatric ones. It is the challenge of the pediatric subspecialist to decide when and how to apply adult findings to our pediatric practices. In this environment, collaboration between pediatric centers of excellence, partnership with adult congenital heart disease experts and the development of subspecialty programs and clinics are crucial steps to moving pediatric practices forward in a data-driven way. This has been our path at Children's Hospital. It has allowed us to improve the care for children with heart failure, to judiciously integrate adult practices and to lead the way in pediatric care. In some ways we have moved past standard adult paradigms by recognizing the very real anatomic and physiologic differences in children and the often unique circumstances that bring them to the heart failure/heart transplant specialist.
Heart transplantation
The pediatric heart transplant program at Children's Hospital was started in 1991 and has seen tremendous growth over the years. In the first 10 years, 16 heart transplants were performed. By comparison, in the last five years (2005-2010), we have performed more than 60. While volume has increased significantly and the children who have come to transplantation have, as a group, been sicker and more vulnerable, the outcomes have continued to substantially improve. In the past five years, 30 percent of the children have required some type of mechanical support pretransplant, 22 percent have been ventilator dependent, more than 50 percent have had some form of congenital heart disease and nearly 60 percent have had some level of antibody sensitization. All of these factors are known to increase the risks associated with transplantation. Despite these challenges, our overall survival during the past five years has been 83 percent with a conditional survival (past 6 months) of 95 percent. Academically, we have participated in the Pediatric Heart Transplant Study Group. Through this national consortium of the 36 largest pediatric transplant centers, we have participated in numerous prospective and retrospective research projects that have resulted in significantly improved outcomes for the field as a whole. Additionally, in collaboration with the BloodCenter of Wisconsin, we have led the way in the management of sensitized children who require heart transplantation. Following in vitro research at the BloodCenter in 2004, we applied this approach in our own practice. In 2007, we reported our experience in the pediatric population wherein we moved historically from having 7 of 8 sensitized children not surviving to transplant with a prospective crossmatch strategy to successfully transplanting 10 of 11 consecutive children with our virtual crossmatch strategy. In that original abstract, we coined the term Òvirtual crossmatch,Ó which has since become ubiquitous, recognized by UNOS, and adopted nationally as a preferred strategy by pediatric and adult programs.
Heart failure
The growth in the pediatric heart transplant program would not have been possible without the recognition that pediatric heart failure is a subspecialty that is best served by having resources and expertise brought together in a programmatic fashion. We formally started the pediatric heart failure program at Children's Hospital in 2005 and have seen steady growth since that time. Initially sharing resources with the transplant program, the heart failure program has grown to become a dedicated practice with its own outpatient clinic, subspecialty-experienced advanced practice nursing support and collaboration with support services including experts in genetics, metabolic disease, electrophysiology and psychosocial challenges. In essence, our initial goals in the heart failure program are to identify alternatives to heart transplantation and/or to look for opportunities to safely improve functionality and quality of life in children with heart failure while delaying or eliminating the need for transplant. Early referral is recommended as it allows us to judiciously counsel families 
as to the timing of consideration for transplantation. In numerous cases, we have been able to manage heart failure medically, improve dysrhythmia therapy or utilize cardiac resynchronization, provide support for the failing fontan or recommend surgical palliation in ways that allow patients to delay or avoid the need for transplantation. We offer heart failure consultative services either as an adjunct to primary cardiology care or as the primary service for these patients.
Going forward, we can anticipate continued growth in these programs and better alternatives over time for children with late-stage cardiac failure. Innovations in mechanical support, the development of tissue valves and the promise of stem cell therapies, along with improvements in immunomodulation with monoclonal antibodies and genetically guided personalized pharmacology, all hold the promise of near-term benefits for our patients. We believe we have organized our resources in transplantation and heart failure, and we recognize the incredible infrastructure of the Herma Heart Center at Children's Hospital that will allow us to take full advantage of these developments to improve the lives of our patients and families.
For outcomes from our Herma Heart Center, visit chw.org/quality.
Heart Matters
Children's Hospital of Wisconsin's Herma Heart Center is one of the nation's top programs for medical and surgical treatment of congenital heart defects and heart disease in children. As one of the largest pediatric cardiac programs in the United States, we have set national benchmarks for surgical outcomes. For more information, visit chw.org/hermaheartcenter.
Steven Zangwill, MD, catches up with 13-year-old heart transplant patient Anna Schmidt. Children's Hospital of Wisconsin is among the nation's top hospitals for pediatric heart transplant volumes. In 2010, we performed more heart transplants than ever before.