- 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.
- 著者
- Koichiro Miura Katsuhisa Matsuura Yu Yamasaki Itoyama Daisuke Sasaki Takuma Takada Yoshiyuki Furutani Emiko Hayama Masamichi Ito Seitaro Nomura Hiroyuki Morita Masashi Toyoda Akihiro Umezawa Kenji Onoue Yoshihiko Saito Hiroyuki Aburatani Toshio Nakanishi Nobuhisa Hagiwara Issei Komuro Tatsuya Shimizu
- 出版者
- International Heart Journal Association
- 雑誌
- International Heart Journal (ISSN:13492365)
- 巻号頁・発行日
- vol.63, no.2, pp.338-346, 2022-03-30 (Released:2022-03-30)
- 参考文献数
- 35
- 被引用文献数
- 5
Dilated cardiomyopathy (DCM) is caused by various gene variants and characterized by systolic dysfunction. Lamin variants have been reported to have a poor prognosis. Medical and device therapies are not sufficient to improve the prognosis of DCM with the lamin variants. Recently, induced pluripotent stem (iPS) cells have been used for research on genetic disorders. However, few studies have evaluated the contractile function of cardiac tissue with lamin variants. The aim of this study was to elucidate the function of cardiac cell sheet tissue derived from patients with lamin variant DCM. iPS cells were generated from a patient with lamin A/C (LMNA) -mutant DCM (LMNA p.R225X mutation). After cardiac differentiation and purification, cardiac cell sheets that were fabricated through cultivation on a temperature-responsive culture dish were transferred to the surface of the fibrin gel, and the contractile force was measured. The contractile force and maximum contraction velocity, but not the maximum relaxation velocity, were significantly decreased in cardiac cell sheet tissue with the lamin variant. A qRT-PCR analysis revealed that mRNA expression of some contractile proteins, cardiac transcription factors, Ca2+-handling genes, and ion channels were downregulated in cardiac tissue with the lamin variant.Human iPS-derived bioengineered cardiac tissue with the LMNA p.R225X mutation has the functional properties of systolic dysfunction and may be a promising tissue model for understanding the underlying mechanisms of DCM.