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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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