Abstract
Children under 15 years of age could not donate their hearts after brain death until the Japanese Organ Transplantation Act was revised on 17th July in 2010, because only persons who had a written consent for organ donation after brain death could donate their organs in Japan. Therefore, small children could not undergo heart transplantation （HTx） in Japan and many Japanese children had been abroad to undergo HTx. After revision of the Act, small children could be able to donate organs if their family accepted and, in fact, finally six children （one younger than 6 years, three between 10 and 14 years, and two between 15 and 17 years） donated his or her heart until the end of 2013. In this review, current status and issues of pediatric HTx in Japan and indication of pediatric HTx and management before and after HTx were described. Briefly, most indication of HTx was dilated cardiomyopathy （DCM） and restrictive cardiomyopahty in Japan and many candidates with DCM required left ventricular assist device （LVAS） for bridge to HTx in Japan. Immunosuppressive regimen was calcineurin inhibitor and mycophenolate mofetil and steroid was discontinued within 6 months in most children. Patient survival at 10 years after HTx was 100% in children transplanted in Japan and 87.6% in children transplanted abroad. Post transplant lymphoproliferative disorder and various infections were major morbidity and mortality. Non-adherence should be carefully observed while taking care of adolescent patients.
