Abstract
Since the first heart transplantation procedure was performed almost 50 years ago, heart transplantation has undergone a startling transformation from an experimental, high-risk intervention to being offered as standard of care for children and adults with heart failure refractory to medical and surgical therapy.1,2 The potential for heart transplantation to treat complex congenital heart disease was recognized early in the transplantation experience. In 1967, the first heart transplantation procedure in the United States was performed in an infant with Ebstein anomaly and subvalvular right ventricular outflow tract obstruction.3 Shortly thereafter, heart transplantation was adopted to treat the most complex forms of congenital heart disease in the most critical situations.4–9 Over the past 3 decades, the indications for transplantation in adults and children have evolved as new medical and device therapies have been developed, the number of available donors has plateaued, and risk factors for better and worse outcomes have been identified.10,11 In the 1980s, survival after heart transplantation was significantly better than survival after the Norwood procedure, and unrepaired hypoplastic left heart syndrome was the major indication for infant heart transplantation.12–15 As survival after the Norwood procedure improved to >80% and the shortage of infant donor hearts continued to result in a high incidence of death while waiting (20%–25%), the indications for transplantation in the neonate with hypoplastic left heart syndrome have narrowed to the point where transplantation is rarely considered in the initial management of this lesion.16–18 This has resulted in a significant decrease in the proportion of infants with the diagnosis of congenital heart disease undergoing transplantation, as shown in Figure 1.18,19 Figure 1. The distribution of number of patients listed for heart transplantation in the Pediatric Heart Transplant Study …
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