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

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Abstract

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.

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Funding

This work was supported by the Health and Labour Sciences Research Grant from the Ministry of Health and Labor (Grant number H25-102).

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Authors and Affiliations

  1. Department of Pediatric Cardiology, Tokyo Women’s Medical University, Tokyo, Japan

    Hiroki Mori, Yoshiyuki Furutani & Toshio Nakanishi

  2. Division of Pediatric Cardiology, Sakakibara Heart Institute, Tokyo, Japan

    Tadahiro Yoshikawa & Hitomi Kimura

  3. Division of Cardiology, National Center for Child Health and Development, Tokyo, Japan

    Hiroshi Ono & Hitoshi Kato

  4. Division of Cardiology, Shizuoka Children’s Hospital, Shizuoka, Japan

    Yasuo Ono & Masaki Nii

  5. Department of Pediatrics, The University of Tokyo, Tokyo, Japan

    Takahiro Shindo & Ryo Inuzuka

  6. Department of Pediatrics, University of Tsukuba, Tsukuba, Japan

    Hitoshi Horigome

  7. Division of Cardiology, Tokyo Metropolitan Children’s Medical Center, Tokyo, Japan

    Masaru Miura

  8. Department of Pediatrics, University of Toyama, Toyama, Japan

    Keiichi Hirono

  9. Division of Cardiology, Gunma Children’s Medical Center, Shibukawa, Gunma, Japan

    Tomio Kobayashi

  10. Department of Pediatrics, Osaka University, Suita, Osaka, Japan

    Shigetoyo Kogaki

  11. Sakakibara Heart Institute Clinic, Japan Research Promotion Society for Cardiovascular Diseases, 2-4-1, Nishi-Shinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan

    Toshio Nakanishi

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  1. Hiroki Mori
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  2. Tadahiro Yoshikawa
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Corresponding author

Correspondence to Toshio Nakanishi.

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The authors declare no conflicts of interest.

Ethical approval

The Medical Ethics Committee of the Tokyo Women’s Medical University, Clinical Study #2714.

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Appendix

Appendix

List of investigators and institutions participated to this study.

Hiroki Mori, Mikito Shimizu-Ishido, Toshio Nakanishi (Tokyo Women’s Medical University), Tadahiro Yoshikawa, Hitomi Kimura (Sakakibara Heart Institute), Hiroshi Ono, Hitoshi Kato (National Center for Child Health and Development), Yasuo Ono, Masaki Nii (Shizuoka Children’s Hospital), Takahiro Shindo, Ryo Inuzuka (The University of Tokyo), Hitoshi Horigome (University of Tsukuba), Fukiko Ichida, Keiichi Hirono (University of Toyama), Tomio Kobayashi (Gunma Children’s Medical Center), Shigetoyo Kogaki (Osaka University), Masaru Miura, (Tokyo Metropolitan Children’s Medical Center), Junko Shiono (Ibaraki Children’s Hospital), Motoki Takamuro (Hokkaido Medical Center for Child Health and Rehabilitation), Mamoru Ayusawa (Nihon University), Minako Hoshiai (Yamanashi University), Shunichi Ogawa (Nihon Medical School), Kiyoshi Ogawa (Saitama Children’s Medical Center), Toshiki Kobayashi (Saitama Medical Unversity International Medical Center), Hiromichi Hamada (Tokyo Women’s Medical University Yachiyo Medical Center), Hiroyuki Matsuura (Toho University Omori Medical Center), and Isao Shiraishi (National Cerebral and Cardiovascular Center).

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Mori, H., Yoshikawa, T., Kimura, H. et al. Outcomes of hypertrophic cardiomyopathy in Japanese children: a retrospective cohort study. Heart Vessels 37, 1075–1084 (2022). https://doi.org/10.1007/s00380-021-01989-7

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