Hypertrophic cardiomyopathy management: a systematic review of the clinical practice guidelines and recommendations
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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.
Aims:
In light of recent advances in imaging techniques, molecular understanding and therapeutic options in hypertrophic cardiomyopathy (HCM), we performed a systematic review of current guidelines for the diagnosis and management of HCM in order to identify consensus and discrepant areas in the clinical practice guidelines.
Methods And Results:
We systematically reviewed the English language guidelines and recommendations for the management of HCM in adults. MEDLINE and EMBASE databases were searched for guidelines published in the last 10 years. Following a systematic search, three guidelines on the diagnosis and management of HCM were identified, all of which were robustly developed (AGREE rigour of development score ≥50%). These guidelines were authored by the major European (European Society of Cardiology; 2023), American (American Heart Association /American College of Cardiology/American Medical Society for Sports Medicine /Heart Rhythm Society/Pediatric and Congenital Electrophysiology Society/Society for Cardiovascular Magnetic Resonance; 2024), and Japanese [Japanese Circulation Society (JCS)/Japanese Heart Failure Society (JHFS); 2018] cardiovascular societies. There was broad consensus on echocardiographic recommendations, the medical and invasive management of HCM, the application of genetic testing and family screening, and exercise and reproductive recommendations in HCM. There were areas of variability in the definition and diagnostic criteria for HCM, cardiovascular magnetic resonance imaging recommendations, and assessment of sudden cardiac death (SCD) risk and prevention strategies. Due to the JCS/JHFS guidelines being older, there are no recommendations on the use of cardiac myosin ATPase inhibitors.
Conclusion:
Contemporary guidelines for HCM achieve consensus across a broad range of criteria and recommendations concerning diagnosis and management. However, variations in the approach towards risk assessment for SCD exist between the guidelines. There are also more subtle differences concerning diagnostic criteria and the utility of late gadolinium enhancement for risk stratification, which will likely evolve as the evidence-base broadens.
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