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From its inception in the early 1980s, heart transplantation had seen a steady rise in the numbers of operations performed. However, about 15 years ago, the procedure reached a worldwide plateau of approximately 400 pediatric transplants being performed each year. This has recently increased to a record high of 577 pediatric transplants in 2013 reported to the International Society for Heart and Lung Transplantation (ISHLT) International Registry of Heart and Lung Transplantation, which composes 13% of all heart transplants performed. Limited availability of infant and pediatric organ donors remains the limitation on transplants performed.
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Heart transplantation is performed primarily in cases of end-stage heart failure, although it may also be indicated in children with failed palliation of congenital heart disease (CHD), for example, in patients with severe cyanosis or Fontan circulation failure that does not involve ventricular dysfunction. Diagnoses of recipients vary by age; CHD remains the most common indication for heart transplant in the infant age group (53%), which has decreased from 75% since the earlier era (before 2000), whereas only 25% of teens transplanted have CHD (Fig. 494-1). The majority of teenage recipients (66%) have cardiomyopathy (most commonly dilated cardiomyopathy). In the early period, heart transplant was indicated as primary therapy for congenital heart lesions that were not amenable to surgical repair, including hypoplastic left heart syndrome (HLHS). Although staged palliative surgery for HLHS and other complicated lesions with single ventricle physiology has significantly improved over time, those who develop contraindications for further surgical single ventricle palliation account for the continued high incidence of transplantations in this age group.
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In 2007, the American Heart Association issued a scientific statement on indications for heart transplantation in pediatric heart disease. Because of the lack of randomized pediatric clinical trials to inform a strictly evidence-based practice, the recommendations shown in Table 494-1 are mainly level of evidence B or C, based on nonrandomized studies or expert consensus opinion, respectively.
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