Current status and challenges of Pediatric heart transplantation in Japan.

It has been about 10 years since the revised Japanese Organ Transplant Law came into action. Organ donation from brain dead donors has increased in the past decade, but the number of recipients waiting for transplant is increasing more rapidly. Implantable continuous flow ventricular assist device, such as Jarvik 2000 and HeartWare, available from 2011, and Berlin heart EXCOR, available from 2015, has changed the scene for children with severe heart failure. About 80% of the patients who received heart transplantation (HTx) were supported by one of these devices, and average waiting time is 688±502 days. Despite small number of patients transplanted in Japan, patient survival at 10 years was 97.5%, which is superior to that of the International Registry of Heart and Lung Transplantation. Immunosuppression regimen included calcineurin inhibitor (mostly tacrolimus), mycophenolate mofetil, and steroid at the time of HTx. Major complications after HTx include rejection, infection, renal failure, cardiac allograft vasculopathy, and post-transplant lymphoproliferative disorders. Reasons for survival of Japanese pediatric HTx includes selections: limited number of patients with congenital heart disease, low donor risks, and good compliance. Further effort is required to achieve self-sufficiency of organ donors and transplantation, and reduce morbidity and mortality after HTx.