Related Experiment Video
Updated: Apr 26, 2026

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix
Published on: June 14, 2016
Long-Term Real-World Impact of Cardiac Myosin Inhibitors on Diastolic Function in Hypertrophic Cardiomyopathy
Aakash Bavishi1, John Fritzlen2, Marybeth Soutar3
1Division of Cardiology, University of Illinois, Chicago, Illinois.
Insights
Long-term treatment with mavacamten significantly improved diastolic function in hypertrophic cardiomyopathy (HCM) patients. These positive effects on diastolic function, left atrial remodeling, and pulmonary pressures were durable over 24 months in a real-world setting.
Area of Science:
- Cardiology
- Pharmacology
Background:
- Diastolic dysfunction is a primary driver of symptoms and adverse cardiac remodeling in hypertrophic cardiomyopathy (HCM).
- Limited data exist on the long-term durability of diastolic improvements achieved with cardiac myosin inhibitor therapy in real-world clinical practice.
Purpose of the Study:
- To evaluate the real-world durability of diastolic function improvements in patients with hypertrophic cardiomyopathy (HCM) treated with mavacamten for at least 18 months.
- To assess changes in left ventricular (LV) structure, diastolic indices, and pulmonary pressures over a 24-month period.
Main Methods:
- A real-world cohort study involving adults with HCM receiving mavacamten therapy for a minimum of 18 months.
- Serial echocardiographic assessments were conducted at approximately 3-month intervals up to 24 months.
- Analysis included paired baseline and end-point measurements of LV structure, diastolic function (e.g., e', E/e'), left atrial volume index (LAVI), and right ventricular systolic pressure (RVSP).
Main Results:
- Significant reductions in LAVI (Δ -12.0 mL/m²), LV wall thickness (Δ -3.5 mm), and LV mass index (Δ -23.4 g/m²) were observed by 24 months (all p<0.001).
- Improvements in diastolic function were evidenced by increased lateral e' (p<0.001) and medial e' (p<0.001), and decreased lateral E/e' (p<0.001) and medial E/e' (p<0.001).
- A significant decrease in RVSP (p=0.01) and a shift toward lower grades of diastolic dysfunction were noted over the study period.
Conclusions:
- Mavacamten treatment in a real-world HCM cohort led to significant improvements in echocardiographic measures of diastolic function.
- These improvements in diastolic function, left atrial remodeling, and pulmonary pressures were sustained and durable through 24 months.
- The findings support the long-term efficacy of mavacamten in managing diastolic dysfunction in HCM patients outside of clinical trial settings.
Abstract:
Diastolic dysfunction is a key determinant of symptoms and adverse remodeling in hypertrophic cardiomyopathy (HCM). Data describing the durability of diastolic improvement with long-term cardiac myosin inhibitor therapy in real-world settings remain limited. We performed a real-world cohort study of adults with HCM treated with at least 18 months of mavacamten with serial echocardiography at approximately 3-month intervals through 24 months. Left ventricular (LV) structure, standard diastolic indices and diastolic dysfunction grade were assessed. Paired baseline and end-point measures were compared. Fifty-one patients were included (mean age 58 ± 14 years; 45% men). From baseline to 24 months, left atrial volume index decreased from 42.2 ± 14.4 to 30.2 ± 11.7 ml/m² (Δ -12.0, p <0.001); LV wall thickness decreased from 18.7 ± 3.0 to 15.2 ± 2.3 mm (Δ -3.5, p <0.001); and LV mass index decreased from 121.6 ± 33.5 to 98.1 ± 22.3 g/m² (Δ -23.4, p <0.001). Lateral e' increased from 0.08 ± 0.03 to 0.09 ± 0.03 m/s (p <0.001) and medial e' increased from 0.05 ± 0.02 to 0.07 ± 0.02 m/s (p <0.001). Lateral E/e' decreased from 14.3 ± 8.9 to 9.8 ± 3.9 (p <0.001) and medial E/e' decreased from 18.8 ± 11.3 to 13.3 ± 4.7 (p <0.001). RVSP decreased from 30.0 ± 12.0 to 26.5 ± 8.9 mm Hg (p = 0.01). Diastolic dysfunction grade distribution shifted toward lower grades over time. In conclusion, in a real-world HCM cohort treated with mavacamten, echocardiographic measures of diastolic function, LA remodeling, and pulmonary pressures improved and remained durable through 24 months.
Related Concept Videos
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Heart Failure II: Pathophysiology
Cardiomyopathy V: Interprofessional Care
Myocarditis I: Introduction
Cardiomyopathy II: Dilated Cardiomyopathy
Heart Failure Drugs: Inhibitors of Renin-Angiotensin System

