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 (ESC; 2023), American (AHA/ACC/AMSSM/HRS/PACES/SCMR; 2024) and Japanese (JCS/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 (CMR) imaging recommendations and assessment of sudden cardiac death (SCD) risk and prevention strategies. Due to the JCS/JHFS guideline 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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http://dx.doi.org/10.1093/ehjqcco/qcae117 | DOI Listing |
Eur Heart J Qual Care Clin Outcomes
January 2025
William Harvey Research Institute, Queen Mary University of London, Charterhouse Square, London EC1M 6BQ, UK.
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.
Catheter Cardiovasc Interv
January 2025
Division of Cardiovascular Diseases, Bridgeport Hospital, Yale New Haven Health, Bridgeport, Connecticut, USA.
Background: The co-existence of severe aortic stenosis (AS) and hypertrophic cardiomyopathy (HCM) is not uncommon. Surgical intervention is the gold standard management. Patients with high surgical risk might undergo transcatheter aortic valve replacement (TAVR).
View Article and Find Full Text PDFCardiol Rev
October 2024
Department of Cardiology, Royal Devon University Healthcare National Health Service Foundation Trust, Exeter, United Kingdom.
Hypertrophic cardiomyopathy (HCM) is a genetic cardiac disorder characterized by structural and functional abnormalities. Current management strategies, such as medications and septal reduction therapies, have significant limitations and risks. Recently, cardiac myosin inhibitors (CMIs) like mavacamten and aficamten have shown promise as noninvasive treatment options.
View Article and Find Full Text PDFJ Cardiovasc Dev Dis
December 2024
3rd Department of Cardiology, "Sotiria" Chest Diseases Hospital, Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece.
Hypertrophic cardiomyopathy (HCM) is a complex and heterogeneous cardiac disorder, often complicated by cardiogenic shock, a life-threatening condition marked by severe cardiac output failure. Managing cardiogenic shock in HCM patients presents unique challenges due to the distinct pathophysiology of the disease, which includes dynamic left ventricular outflow tract obstruction, diastolic dysfunction, and myocardial ischemia. This review discusses current and emerging therapeutic strategies tailored to address the complexities of HCM-associated cardiogenic shock and other diseases with similar pathophysiology that provoke left ventricular outflow tract obstruction.
View Article and Find Full Text PDFGlob Heart
December 2024
Instituto do Coração, Faculdade de Medicina Universidade de São Paulo, São Paulo, Brazil.
Background: Sudden cardiac death (SCD) is a major concern in patients with hypertrophic cardiomyopathy (HCM). The American College of Cardiology/American Heart Association (ACC/AHA) and the European Society of Cardiology (ESC) have different guidelines for SCD risk stratification. Their comparative performance in diverse populations remains uncertain.
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