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Phase 3 Completed N=251 Randomized Quadruple-blind Treatment

Clinical Study to Evaluate Mavacamten (MYK-461) in Adults With Symptomatic Obstructive Hypertrophic Cardiomyopathy

Source: ClinicalTrials.gov NCT03470545 ↗
Enrolled (actual)
251
Serious AEs
10.4%
Results posted
Oct 2021
Primary outcomePrimary: Percentage of Participants Achieving A Clinical Response — 45; 22 Participants
◆ Published Evidence
Established
96citations · ~48 / year
Long-term effect of mavacamten in obstructive hypertrophic cardiomyopathy.
European heart journal · 2024 · Open access · Likely link

Summary

This is a multicenter, international, double-blind study of the administration of mavacamten in participants with symptomatic obstructive HCM (oHCM). Approximately 220 participants will be randomized to receive placebo or mavacamten.

Linked Publications (5)

  • Long-term effect of mavacamten in obstructive hypertrophic cardiomyopathy.
    European heart journal · 2024 · 96 citations · Open access · Likely link
  • The Kansas City Cardiomyopathy Questionnaire in Relation to New York Heart Association Class.
    Journal of cardiac failure · 2025 · 11 citations · Open access · Likely link
  • Association Between age or Duration of Diagnosis in Obstructive Hypertrophic Cardiomyopathy and Response to Mavacamten Treatment: Exploratory Analysis of the EXPLORER-HCM Trial.
    Journal of cardiac failure · 2025 · 9 citations · Likely link
  • The Psychometric Performance of the Kansas City Cardiomyopathy Questionnaire-12 in Symptomatic Obstructive Hypertrophic Cardiomyopathy.
    Journal of cardiac failure · 2025 · 6 citations · Open access · Likely link
  • Association of Echocardiographic Parameters and Health Status in Patients With Obstructive Hypertrophic Cardiomyopathy: Insights From EXPLORER-HCM.
    Circulation · 2024 · 5 citations · Open access · Likely link

Outcome Measures

OutcomeResultp-value
PRIMARY
Percentage of Participants Achieving A Clinical Response
45; 22
SECONDARY
Changes From Baseline to Week 30 in Post Exercise in LVOT Peak Gradient.
-47; -10
SECONDARY
Change From Baseline to Week 30 in pVO2 as Assessed by CPET
1.4; -0.05
SECONDARY
Proportion of Participants With at Least 1 Class Improvement in NYHA Functional Class From Baseline to Week 30
80; 40
SECONDARY
Change From Baseline to Week 30 in Participant-reported Health-related Quality of Life as Assessed by the KCCQ Score
13.6; 4.2
SECONDARY
Change From Baseline to Week 30 in Participant-reported Severity of HCM Symptoms as Assessed by the HCMSQ Score
-2.8; -0.9

Eligibility Criteria

Key Inclusion Criteria

  • Age 18 and greater, body weight ≥ 45kg
  • Has adequate acoustic windows to enable accurate transthoracic echocardiograms (TTEs)
  • Diagnosed with oHCM consistent with current American College of Cardiology Foundation/American Heart Association and European Society of Cardiology guidelines and satisfy both criteria:
  • Has documented left ventricular ejection fraction (LVEF) ≥55%
  • NYHA Class II or III
  • Has documented oxygen saturation at rest ≥90% at Screening
  • Is able to perform an upright CPET and has a respiratory exchange ratio (RER) ≥1.0 at Screening per central reading

Key Exclusion Criteria

  • Known infiltrative or storage disorder causing cardiac hypertrophy that mimics oHCM, such as Fabry disease, amyloidosis, or Noonan syndrome with LV hypertrophy
  • History of syncope or sustained ventricular tachyarrhythmia with exercise within 6 months prior to Screening
  • History of resuscitated sudden cardiac arrest (at any time) or known history of appropriate implantable cardioverter defibrillator (ICD) discharge for life-threatening ventricular arrhythmia within 6 months prior to Screening
  • Paroxysmal, intermittent atrial fibrillation with atrial fibrillation present at Screening
  • Persistent or permanent atrial fibrillation not on anticoagulation for at least 4 weeks prior to Screening and/or not adequately rate controlled within 6 months prior to Screening
  • Treatment (within 14 days prior to Screening) or planned treatment during the study with disopyramide or ranolazine
  • Treatment (within 14 days prior to Screening) or planned treatment during the study with a combination of β-blockers and calcium channel blockers
  • LVOT gradient with Valsalva maneuver <30 mmHg at Screening
  • Has been successfully treated with invasive septal reduction (surgical myectomy or percutaneous alcohol septal ablation [ASA]) within 6 months prior to Screening or plans to have either of these treatments during the study
  • ICD placement within 2 months prior to Screening or planned ICD placement during the study
  • Has a history or evidence of any other clinically significant disorder, condition, or disease that, in the opinion of the investigator, would pose a risk to participant safety or interfere with the study evaluation, procedures, or completion
  • Prior treatment with cardiotoxic agents such as doxorubicin or similar
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT03470545) and the linked publication. Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication. Informational only — not medical advice.

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