Outcomes of Dilated Cardiomyopathy in Japanese Children - A Retrospective Cohort Study
Related Experiment Video
Updated: Oct 18, 2025
A Doxorubicin-Induced Murine Model of Dilated Cardiomyopathy In Vivo
Published on: May 16, 2020
Hiroki Mori1, Tadahiro Yoshikawa2, Hitomi Kimura2
1Department of Pediatric Cardiology, Tokyo Women's Medical University.
Insights
Pediatric dilated cardiomyopathy (DCM) in Japan has a poor prognosis. This study found low survival rates and limited heart transplantation for pediatric DCM patients, highlighting a critical need for improved care.
Area of Science:
- Cardiology
- Pediatric Medicine
- Public Health
Background:
- No nationwide data existed on pediatric dilated cardiomyopathy (DCM) prognosis in Japan.
- Pediatric DCM significantly impacts long-term survival and requires urgent investigation.
Purpose of the Study:
- To investigate the long-term survival rate of pediatric patients diagnosed with DCM in Japan.
- To establish a baseline for pediatric DCM prognosis in the Japanese population.
Main Methods:
- A retrospective, multicenter observational study was conducted.
- Data from 106 pediatric patients (<18 years) diagnosed with DCM between 1990 and 2014 were analyzed.
- Survival and heart transplantation rates were tracked over 20 years post-diagnosis.
Main Results:
- The median age at diagnosis was 2.0 years, with most cases presenting with heart failure symptoms.
- Freedom from death or transplantation at 20 years was 43%, with freedom from death at 53%.
- Heart transplantation incidence was low, with only 15% of patients receiving a transplant within 5 years.
Conclusions:
- Pediatric DCM in Japan is associated with a poor long-term prognosis.
- The rate of heart transplantation for pediatric DCM patients in Japan is notably low.
- Findings underscore the need for enhanced management strategies and access to transplantation for pediatric DCM.
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.
Related Concept Videos
01:30Cardiomyopathy II: Dilated Cardiomyopathy
01:29Cardiomyopathy III: Hypertrophic Cardiomyopathy
01:29Cardiomyopathy IV: Restrictive Cardiomyopathy
01:29Cardiomyopathy V: Interprofessional Care
01:22Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies
01:25Cardiomyopathy I: Introduction and Classification