Hypertrophic Cardiomyopathy Today: Integrating Myosin Inhibition with Established Strategies

Area of Science:

• Cardiology
• Genetics
• Pharmacology

Background:

• Hypertrophic cardiomyopathy (HCM) is a significant cause of heart disease.
• Advances in understanding HCM's genetic basis and pathophysiology are ongoing.
• Current management strategies aim to improve patient outcomes and quality of life.

Purpose of the Study:

• To synthesize current knowledge on hypertrophic cardiomyopathy (HCM).
• To integrate recent progress in imaging, genetics, and therapeutics, focusing on cardiac myosin inhibitors.
• To emphasize individualized care approaches for both obstructive and nonobstructive HCM.

Main Methods:

• This narrative review analyzes contemporary literature on HCM.
• Key areas include epidemiology, disease mechanisms, diagnostic tools, and management strategies.
• Emphasis is placed on multimodality imaging (echocardiography, cardiac magnetic resonance), risk stratification, and clinical trial data for mavacamten and aficamten.

Main Results:

• Earlier diagnosis and improved risk assessment have reduced HCM-related mortality over the past two decades.
• Cardiac myosin inhibitors demonstrate consistent benefits in symptoms, left ventricular outflow tract gradients, and functional capacity in obstructive HCM.
• Nonobstructive HCM remains challenging to treat, with some patients progressing to advanced heart failure; biomarkers and imaging may aid monitoring.

Conclusions:

• Myosin inhibition represents a therapeutic shift for obstructive HCM, potentially offering benefits beyond symptom relief, though long-term safety and applicability require further study.
• Advanced imaging and biomarkers are essential for personalized decision-making and risk stratification in HCM.
• While outcomes are improving with multidisciplinary care, unmet needs in nonobstructive HCM and equitable access to therapies remain critical considerations.