Hypertrophic cardiomyopathy management: a systematic review of the clinical practice guidelines and recommendations

Mihir M Sanghvi1,2,3, Eamon Dhall4, C Anwar A Chahal1,3,5,6

  • 1William Harvey Research Institute, Queen Mary University of London, Charterhouse Square, London EC1M 6BQ, UK.

Insights

This review compares hypertrophic cardiomyopathy (HCM) guidelines, finding consensus on diagnosis and management but differing approaches to sudden cardiac death (SCD) risk assessment. Newer guidelines address evolving treatments like myosin inhibitors.

Area of Science:

  • Cardiology
  • Genetics
  • Medical Guidelines

Background:

  • Hypertrophic cardiomyopathy (HCM) is a complex genetic heart disease with evolving diagnostic and therapeutic strategies.
  • Recent advancements in imaging, molecular understanding, and treatments necessitate updated clinical practice guidelines.

Purpose of the Study:

  • To systematically review and compare current international guidelines for HCM diagnosis and management.
  • To identify areas of consensus and discrepancy among major cardiovascular society guidelines.

Main Methods:

  • Systematic review of English-language guidelines for adult HCM published within the last 10 years.
  • Searched MEDLINE and EMBASE databases.
  • Evaluated three key guidelines from European (ESC, 2023), American (AHA/ACC/AMSSM/HRS/PACES/SCMR, 2024), and Japanese (JCS/JHFS, 2018) cardiovascular societies.

Main Results:

  • Broad consensus exists on echocardiography, medical/invasive management, genetic testing, family screening, and exercise/reproductive recommendations.
  • Variability noted in HCM definition, diagnostic criteria, cardiovascular magnetic resonance (CMR) imaging, and sudden cardiac death (SCD) risk assessment.
  • Older Japanese guidelines lack recommendations on novel cardiac myosin ATPase inhibitors.

Conclusions:

  • Contemporary HCM guidelines show significant agreement on diagnosis and management.
  • Divergent strategies for SCD risk stratification persist among guidelines.
  • Subtle differences in diagnostic criteria and the role of late gadolinium enhancement in risk stratification require ongoing evaluation.
Abstract

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