Heart failure & cardiomyopathies

Long-Term Favorable Cardiac Remodeling in Obstructive Hypertrophic Cardiomyopathy Patients Treated With Mavacamten for Up to 128 Weeks: Insights From the VALOR-HCM Trial

JACC Cardiovasc ImagingPublished Secondary analysis of the VALOR-HCM randomised trial

Authors' conclusion

In the VALOR-HCM trial, treatment with mavacamten resulted in sustained favorable cardiac remodeling, including improvement in LVOT gradients, cardiac volumes, cardiac hypertrophy, diastolic function, and markers of LA and LV strain from baseline through week 128, suggesting disease modification.

Study context

Population
Adults with symptomatic obstructive hypertrophic cardiomyopathy (HCM) referred for septal reduction therapy (mean age 60.3 years; 50% men; 94% NYHA III/IV)
Participants
112 adults
Comparison
Mavacamten, change from baseline to week 128
Primary endpoint
Long-term echocardiographic cardiac remodeling (LVOT gradients, LV and LA structure, function and strain)
Follow-up
128 weeks
Methodological detailsplacebo-controlled for 16 weeks, then all on mavacamten, long-term extension to 128 weeks
  • Placebo-controlled for 16 weeks, then all on mavacamten
  • Long-term extension to 128 weeks

Primary result

As reported in the abstract

Mean % change from baseline, all patients · week 128
Left ventricular outflow tract (LVOT) gradient, resting
−61%
LVOT gradient, post-Valsalva
−72%
LVOT gradient, postexercise
−53%
LV mass index
−11%
Septal E/e'
−18%
LV global longitudinal strain
4.5%
LA volume index
−6%
LA conduit strain
16%
LA contraction strain
35%
LA reservoir strain
32%

Further findings

Events

Outcome≥5 point KCCQ-23-CSS improvement<5 point KCCQ-23-CSS improvement
LA and LV strain by KCCQ-23-CSS response (subgroup) · week 1287125
LA and LV strain by KCCQ-23-CSS response (subgroup) — ≥5 point improvement: significant and sustained improvement
all P < 0.05

LA and LV strain by KCCQ-23-CSS response (subgroup) · week 128

<5 point improvement: no significant improvement in various LA and LV strain values

Original publication

Long-Term Favorable Cardiac Remodeling in Obstructive Hypertrophic Cardiomyopathy Patients Treated With Mavacamten for Up to 128 Weeks: Insights From the VALOR-HCM Trial. JACC Cardiovasc Imaging. Published 16 September 2025. doi:10.1016/j.jcmg.2025.07.019

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