Pediatric heart transplantation.

Heart transplantation is now a treatment option with good outcome for infants and children with end-stage heart failure or complex, inoperable congenital cardiac defects. One-year and 5-year actuarial survival rates are high, approximately 75% and 65%, respectively, with overall patient survival half-life greater than 10 years. To date, survival has been improving as a result of reducing early mortality. Further reductions in late mortality, in part because of graft coronary artery disease and rejection, will allow achievement of the goal of decades-long survival. Quality of life in surviving children, as judged by activity, is usually "normal." Somatic growth is usually at the low normal range but linear growth can be reduced. Of infant recipients, 85% evaluated at 6 years of age or older were in an age-appropriate grade level. Long-term management of childhood heart recipients requires the collaboration of transplant physicians, given the increasing number of immunosuppressive agents and the balance between rejection and infection. Currently, recipients are maintained on immunosuppressive medications that target calcineurin (eg, cyclosporine, tacrolimus), lymphocyte proliferation (eg, azathioprine, mycophenolate mofetil [MMF], sirolimus) and, in some instances antiinflammatory corticosteroids. Emerging evidence now suggests a favorable immunologic opportunity for transplantation in childhood and, conversely, a higher mortality rate in children who have had prior cardiac surgery. Further studies are needed to define age-dependent factors that are likely to play a role in graft survival and possible graft-specific tolerance (eg, optimal conditions for tolerance induction and how immunosuppressive regimens should be changed with maturation of the immune system). As late outcomes continue to improve, the need for donor organs likely will increase, as transplantation affords a better quality and duration of life for children with complex congenital heart disease, otherwise facing a future of multiple palliative operations and chronic heart failure.