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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. Limits on pediatric donor availability appear to be the reason for the limitation on transplants performed.

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Heart transplantation is performed in cases of end-stage heart failure. The primary causes are described in Chapter 497.1 Dilated cardiomyopathy (DCM) with heart failure is the most common indication for heart transplant in children less than a year old. Congenital heart disease is the most common diagnosis in children age 1 and older listed for heart transplant, accounting for 64%, whereas cardiomyopathy accounts for 30%. 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).2 As staged palliative surgery for HLHS and other complicated lesions with single ventricle physiology has improved, and the relatively high death rate for these infant candidates while awaiting heart transplantation has become apparent, referral for infants with congenital heart disease has become more selective. Other indications include rare cases of malignancy, and repeat transplantation (see below).

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In 2007, the American Heart Association issued a scientific statement on indications for heart transplantation in pediatric heart disease.3,4 Because of the lack of randomized pediatric clinical trials to inform a strictly evidence-based practice, the recommendations shown in Table 132-1.

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Table 132-1. Indications for Pediatric Heart Transplantation, American Heart Association Consensus Statement 2007 

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