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Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
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The natural history of hypertrophic cardiomyopathy

Camillo Autore1, Maria Beatrice Musumeci1

  • 1Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, Italy.

European Heart Journal Supplements : Journal of the European Society of Cardiology
|March 3, 2021
PubMed

Insights

Hypertrophic cardiomyopathy (HCM) prognosis has significantly improved due to advanced diagnostics and treatments. Contemporary management has reduced annual mortality to less than 1%, enhancing patient longevity and quality of life.

Area of Science:

  • Cardiology
  • Genetics
  • Internal Medicine

Background:

  • Historically, hypertrophic cardiomyopathy (HCM) was considered a severe condition with high mortality (4-6% annually).
  • Early studies relied on limited data from specialized centers, potentially skewing the perceived prognosis.
  • The advent of echocardiography allowed for broader patient identification and a more accurate understanding of HCM's natural history.

Purpose of the Study:

  • To review the evolving understanding and prognosis of hypertrophic cardiomyopathy.
  • To highlight the impact of diagnostic and therapeutic advancements on HCM outcomes.
  • To describe the changes in the natural history of HCM over time.

Main Methods:

  • Literature review of studies on hypertrophic cardiomyopathy from its recognition to contemporary management.
  • Analysis of mortality rates and prognostic factors based on diagnostic tools and treatment availability.
  • Comparison of outcomes before and after the introduction of echocardiography and modern therapies.

Main Results:

  • Echocardiography revealed a more benign HCM profile with annual mortality below 1.5%.
  • Therapeutic interventions including ICDs, heart transplantation, and myectomy further improved outcomes.
  • Current treatments achieve an annual mortality rate under 1%, with increased longevity and improved quality of life.

Conclusions:

  • The prognosis of hypertrophic cardiomyopathy has dramatically improved over recent decades.
  • Modern diagnostic and therapeutic strategies have transformed HCM into a manageable condition.
  • Contemporary management offers patients extended lifespan and enhanced quality of life.

Keywords:
Hypertrophic cardiomyopathyManagementMortalityNatural historyPrognosis

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