Outcomes of hypertrophic cardiomyopathy in Japanese children: a retrospective cohort study

There has been no multicenter study on the prognosis of pediatric hypertrophic cardiomyopathy (HCM) in Japan. Therefore, we conducted a retrospective multicenter observational study on the long-term survival rate in patients diagnosed with HCM under the age of 18 between 1990 and 2014. Twenty instit...

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Bibliographic Details
Published in:Heart and vessels 2022-06, Vol.37 (6), p.1075-1084
Main Authors: Mori, Hiroki, Yoshikawa, Tadahiro, Kimura, Hitomi, Ono, Hiroshi, Kato, Hitoshi, Ono, Yasuo, Nii, Masaki, Shindo, Takahiro, Inuzuka, Ryo, Horigome, Hitoshi, Miura, Masaru, Hirono, Keiichi, Kobayashi, Tomio, Kogaki, Shigetoyo, Furutani, Yoshiyuki, Nakanishi, Toshio
Format: Article
Language:English
Subjects:
Arrhythmia
Biomedical Engineering and Bioengineering
Cardiac arrhythmia
Cardiac Surgery
Cardiology
Cardiomyopathy
Cohort analysis
Congestive heart failure
Death
Heart failure
Heart transplantation
Medical prognosis
Medicine
Medicine & Public Health
Mortality
Original Article
Patients
Pediatrics
Population studies
Population-based studies
Prognosis
Risk analysis
Risk factors
Survival
Transplantation
Vascular Surgery
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Summary:There has been no multicenter study on the prognosis of pediatric hypertrophic cardiomyopathy (HCM) in Japan. Therefore, we conducted a retrospective multicenter observational study on the long-term survival rate in patients diagnosed with HCM under the age of 18 between 1990 and 2014. Twenty institutions participated. A total of 180 patients were identified. The median age at diagnosis was 5.8 years old and median duration of observation was 8.3 years. Although six patients (3%) deteriorated into the dilated phase of HCM, no patient received heart transplantation. Freedom from death at 1, 5, 10, and 20 years were 97%, 92%, 84%, and 80%, respectively. There were 26 deaths. Among them, 11 patients died suddenly, presumably due to arrhythmia, and 15 patients died of heart failure. The presence of heart failure symptoms and a greater cardiothoracic ratio were significant risk factors for heart failure-related death. There were no significant risk factors identified for arrhythmia-related death. In conclusion, the prognosis of pediatric HCM in Japan is good and similar to those reported in population-based studies in the United States and Australia. Significant risk factors for heart failure-related death were identified in pediatric patients with HCM in Japan.
ISSN:0910-8327
1615-2573
DOI:10.1007/s00380-021-01989-7