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Updated: Oct 12, 2025

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
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Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

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

Hiroki Mori1, Tadahiro Yoshikawa2, Hitomi Kimura2

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

Heart and Vessels
|November 20, 2021
PubMed

Insights

Pediatric hypertrophic cardiomyopathy (HCM) in Japan has a good long-term survival rate, with 80% of patients surviving 20 years. Heart failure symptoms and cardiothoracic ratio predict heart failure-related death in pediatric HCM.

Area of Science:

  • Cardiology
  • Pediatric Medicine
  • Genetics

Background:

  • Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
  • Pediatric HCM prognosis data is limited, especially from multicenter studies in Japan.

Purpose of the Study:

  • To evaluate the long-term survival rate and identify risk factors for pediatric hypertrophic cardiomyopathy (HCM) in Japan.
  • To provide crucial data for managing pediatric HCM patients.

Main Methods:

  • Retrospective multicenter observational study.
  • Involved 20 institutions and 180 patients diagnosed with HCM under 18 years old (1990-2014).
  • Analyzed survival rates, causes of death, and risk factors.

Main Results:

  • Long-term survival rates at 1, 5, 10, and 20 years were 97%, 92%, 84%, and 80%, respectively.
  • 26 deaths occurred: 11 sudden (arrhythmia) and 15 heart failure-related.
  • Heart failure symptoms and higher cardiothoracic ratio were significant risk factors for heart failure-related death.

Conclusions:

  • The prognosis for pediatric hypertrophic cardiomyopathy in Japan is favorable and comparable to international studies.
  • Identified key risk factors for heart failure-related mortality in this population.
  • Further research is needed to identify risk factors for arrhythmia-related death.

Keywords:
ChildrenHypertrophic cardiomyopathyMortalityRisk factors

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