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Updated: Jun 22, 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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Lifetime Clinical Course of Hypertrophic Cardiomyopathy: Outcome of the Historical Florence Cohort Over 5 Decades

Niccolò Maurizi1,2, Iacopo Olivotto1,3,4, Martin S Maron5

  • 1Cardiomyopathy Unit, Careggi University Hospital, Florence, Italy.

JACC. Advances
|June 28, 2024
PubMed

Insights

Hypertrophic cardiomyopathy (HCM) patients experienced a lifetime burden of disease, with heart failure (HF) progression increasing over time. Despite this, overall HCM-related mortality remained low across five decades.

Area of Science:

  • Cardiology
  • Internal Medicine
  • Genetics

Background:

  • Current understanding of hypertrophic cardiomyopathy (HCM) long-term outcomes relies on limited follow-up data (<10 years).
  • Extended assessments are needed to understand the full lifetime burden of HCM.

Purpose of the Study:

  • To report the lifetime clinical course and long-term outcomes of patients with hypertrophic cardiomyopathy (HCM).

Main Methods:

  • Analysis of 202 HCM patients diagnosed between 1970-1992, with annual follow-up up to 50 years.
  • Utilized cumulative incidence functions to estimate HCM-related mortality (heart failure/stroke, sudden cardiac death) and non-HCM related mortality.

Main Results:

  • Over 50 years, 52% of patients died; 42% of HCM-related deaths occurred before modern therapies.
  • HCM-related mortality increased over time, from 0.7% in the first decade to 1.8% in subsequent decades, primarily due to heart failure (HF) and stroke.
  • Sudden cardiac death (SCD) rates remained constant, with 16 SCDs reported.

Conclusions:

  • Hypertrophic cardiomyopathy (HCM) is associated with significant long-term morbidity, particularly progressive heart failure (HF).
  • Sudden cardiac death (SCD) events were less frequent and stable over time.
  • Despite evolving treatment eras, overall HCM-related mortality remained low at 1.3% annually.
Abstract

Keywords:
heart failurehypertrophic cardiomyopathylong-term outcomesudden cardiac death

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