- 著者
- 
             
             Hiroki Mori
             
             Tadahiro Yoshikawa
             
             Hitomi Kimura
             
             Hiroshi Ono
             
             Hitoshi Kato
             
             Yasuo Ono
             
             Masaki Nii
             
             Takahiro Shindo
             
             Ryo Inuzuka
             
             Hitoshi Horigome
             
             Masaru Miura
             
             Shunichi Ogawa
             
             Junko Shiono
             
             Yoshiyuki Furutani
             
             Mikiko Ishido
             
             Toshio Nakanishi
             
          
- 出版者
- The Japanese Circulation Society
- 雑誌
- Circulation Journal (ISSN:13469843)
- 巻号頁・発行日
- vol.86, no.1, pp.109-115, 2021-12-24 (Released:2021-12-24)
- 参考文献数
- 21
- 被引用文献数
- 
             
             1
             
             
             4
             
             
          
        
        Background:There has been no nationwide survey on the prognosis of pediatric dilated cardiomyopathy (DCM) in Japan. Therefore, we designed this retrospective multicenter study to investigate the long-term survival rate in pediatric patients with DCM in Japan.Methods and Results:In this multicenter retrospective observational study, data were reviewed for 106 patients aged <18 years who had been diagnosed with DCM at any 1 of 18 Japanese institutions between 1990 and 2014. The median age at diagnosis was 2.0 years and the median duration of observation was 3.3 years. Most DCM patients were diagnosed because of symptoms of heart failure. On echocardiography, the median left ventricular end-diastolic dimension z score was 5.4 and fractional shortening was 0.10. Freedom from death or transplantation rates at 1, 3, 5, 10, and 20 years after diagnosis were 76%, 66%, 64%, 58%, and 43%, respectively. Freedom from death rates at 1, 5, 10, and 20 years after diagnosis were 81%, 75%, 72%, and 53%, respectively. The incidence of heart transplantation at 1, 5, 10, and 20 years after diagnosis was 6%, 15%, 20%, and 20%, respectively, suggesting that only 15% of patients in Japan underwent heart transplantation within 5 years of diagnosis.Conclusions:In Japan, the prognosis of pediatric DCM is poor and the rate of heart transplantation is low.