[III-TSPCJS-2] 小児心臓移植 日本の現状 -なぜ成績が良いのか-
キーワード：pediatric heart transplantation, coronary vasculopathy, Evelolimus
We started pediatric heart transplantation (HTx) in 2011. Before 2011, we didn't have a choice other than sending patients abroad, and thanks to the good faith and cooperation from several institutes, 123 patients received HTx in other countries. There are 6 licensed centers in Japan where pediatric heart transplantation can be carried out. Fifty-five patients received HTx in Japan. Among those 55 patients, age at HTx was 10±5.8 years, and waiting time was 668±502 days. Ventricular assist device was placed in 44 patients (76%). Survival rate up to 10 years is 97.5% which is significantly better than ISHLT data. One of the reasons we could achieve this good result is patient selection. 38/55 (69%) patients were dilated cardiomyopathy, 8/55(15%) patients were restrictive cardiomyopathy, and only 1 patient had congenital heart disease. Because HTx is scarce and has to wait a long time in Japan, patients' compliance is very good and patient care is tailored precisely. Also, donor risks such as smoking and drug use among children are very low in Japan which could explain relatively small number of post-transplant coronary vasculopathy (CAV), thus low mortality. Including patients transplanted overseas, CAV was diagnosed in about 5% and suspected in 4% of the patients followed in Japan. Tacrolimus and MMF are used as immune-suppressants in more than half of the patients, and Tacrolimus and Evelolimus are used in about 30% of the patients. Although number of patients transplanted in Japan is small, we could achieve good long term outcome.