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A Study to Evaluate Mavacamten in Adolescents With Symptomatic Obstructive Hypertrophic Cardiomyopathy

Phase 3
Recruiting
Conditions
Cardiomyopathy, Hypertrophic
Interventions
Drug: Placebo
Registration Number
NCT06253221
Lead Sponsor
Bristol-Myers Squibb
Brief Summary

The purpose of this study is to evaluate the efficacy, safety, and pharmacokinetics of mavacamten in adolescent patients with symptomatic obstructive hypertrophic cardiomyopathy (HCM).

Detailed Description

Not available

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
40
Inclusion Criteria
  • Diagnosis of HCM
  • Presence of LVOT obstruction
  • Presence of symptoms
Exclusion Criteria
  • Phenocopy diseases resulting in myocardial hypertrophy not related to sarcomere dysfunction
  • Evidence of LVEF <50% in prior 6 months
  • Planned escalation in HCM therapy or upcoming intervention (eg, major cardiac surgery, HCM medication dose increase)

Other protocol-defined Inclusion/Exclusion criteria apply.

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
PlaceboPlaceboParticipants assigned to this arm will receive mavacamten (1 mg to 15 mg) from week 28 to end of treatment at week 200.
MavacamtenMavacamtenParticipants assigned to this arm will receive mavacamten (1 mg to 15 mg) from day 1 to end of treatment at week 200.
Primary Outcome Measures
NameTimeMethod
Change from baseline in Valsalva left ventricular outflow tract (LVOT) (VLVOT) gradientAt Week 28
Secondary Outcome Measures
NameTimeMethod
Change from baseline in resting LVOT gradientAt Week 28
Change from baseline in post-exercise peak LVOT gradientAt Week 28
Change from baseline in maximal wall thicknessAt Week 28
Change from baseline in ratio between early mitral inflow velocity and mitral annular early diastolic velocity (E/e')At Week 28
Proportion of participants achieving an increase from baseline to Week 28 in peak oxygen uptake test (pVO2)From baseline up to Week 28
Proportion of participants achieving a reduction from baseline to Week 28 in maximal LVOT gradient to < 30 mmHgFrom baseline up to Week 28
Proportion of participants with at least 1 class improvement in New York Heart Association (NYHA) class from baseline to Week 28From baseline up to Week 28
Proportion of participants with at least 1 grade improvement in mitral regurgitation at Week 28From baseline up to Week 28
Number of participants with treatment-emergent adverse events (TEAEs)Up to Week 218
Number of participants with treatment-emergent serious adverse events (TESAEs)Up to Week 218
Change from baseline in electrocardiogram (ECG) (QT interval)At Week 28
Number of participants with left ventricular ejection fraction (LVEF) ≤ 30%Up to Week 200
Number of participants with LVEF < 50%Up to Week 200
Trough observed plasma concentration (Ctrough)Up to Week 200
Post-dose plasma concentration of mavacamtenUp to Week 200
Maximum observed concentration (Cmax)Up to Week 200
Area under the concentration-time curve (AUC)Up to Week 200
Proportion of participants who evaluate taste and swallowability as neutral or better using taste and swallowability scalesAt Day 1 and Week 11
Change from baseline in the Hypertrophic Cardiomyopathy Symptom Questionnaire - Shortness of Breath (HCMSQ SoB) domainAt Week 28

Trial Locations

Locations (55)

Rady Children's Hospital-San Diego

🇺🇸

San Diego, California, United States

Children's Hospital of Philadelphia-Center for Childhood Cancer Research

🇺🇸

Philadelphia, Pennsylvania, United States

The Hospital for Sick Children (SickKids)

🇨🇦

Toronto, Ontario, Canada

Royal Belfast Hospital for Sick Children

🇬🇧

Belfast, BFS, United Kingdom

The University of Alabama at Birmingham

🇺🇸

Birmingham, Alabama, United States

Phoenix Children's Cardiology

🇺🇸

Phoenix, Arizona, United States

Children's Hospital Los Angeles

🇺🇸

Los Angeles, California, United States

University of California Los Angeles (UCLA) - Mattel Children's Hospital

🇺🇸

Los Angeles, California, United States

Local Institution - 0004

🇺🇸

Oakland, California, United States

Lucile Packard Children's Hospital (LPCH)

🇺🇸

Palo Alto, California, United States

Children's Hospital Colorado

🇺🇸

Aurora, Colorado, United States

Johns Hopkins All Children's Hospital

🇺🇸

Saint Petersburg, Florida, United States

Children's Healthcare of Atlanta - Arthur M. Blank Hospital

🇺🇸

Atlanta, Georgia, United States

The University of Chicago Medical Center

🇺🇸

Chicago, Illinois, United States

Riley Hospital for Children - Pediatric Cardiology

🇺🇸

Indianapolis, Indiana, United States

Boston Children's Hospital

🇺🇸

Boston, Massachusetts, United States

University of Michigan - C.S. Mott Children's Hospital

🇺🇸

Ann Arbor, Michigan, United States

Washington University School of Medicine in St. Louis - St. Louis Children's Hospital

🇺🇸

Saint Louis, Missouri, United States

Atlantic Medical Group Hypertrophic Cardiomyopathy Associates

🇺🇸

Morristown, New Jersey, United States

The Children's Hospital at Montefiore

🇺🇸

Bronx, New York, United States

Cohen Children's Medical Center of New York

🇺🇸

New Hyde Park, New York, United States

Icahn School of Medicine at Mount Sinai

🇺🇸

New York, New York, United States

Morgan Stanley Children's Hospital of NewYork-Presbyterian

🇺🇸

New York, New York, United States

Sanger Heart & Vascular Institute Heart Failure & Transplant Kenilworth

🇺🇸

Charlotte, North Carolina, United States

Duke University Hospital

🇺🇸

Durham, North Carolina, United States

Cincinnati Children's Hospital Medical Center

🇺🇸

Cincinnati, Ohio, United States

Cleveland Clinic

🇺🇸

Cleveland, Ohio, United States

Nationwide Children's Hospital

🇺🇸

Columbus, Ohio, United States

UPMC Childrens Hospital of Pittsburgh

🇺🇸

Pittsburgh, Pennsylvania, United States

Le Bonheur Children's Hospital

🇺🇸

Memphis, Tennessee, United States

Dell Children's Medical Center of Central Texas

🇺🇸

Austin, Texas, United States

Local Institution - 0055

🇺🇸

Dallas, Texas, United States

Texas Children's Hospital

🇺🇸

Houston, Texas, United States

Primary Children's Hospital

🇺🇸

Salt Lake City, Utah, United States

University Of Virginia School of Medicine

🇺🇸

Charlottesville, Virginia, United States

Local Institution - 0035

🇺🇸

Madison, Wisconsin, United States

The Children's Hospital at Westmead

🇦🇺

Sydney, New South Wales, Australia

Monash Medical Centre Clayton

🇦🇺

Clayton, Victoria, Australia

Local Institution - 0028

🇦🇺

Parkville, Victoria, Australia

Stollery Children's Hospital

🇨🇦

Edmonton, Alberta, Canada

Hôpital Necker - Enfants Malades

🇫🇷

Paris, France

CHU Hopitaux de Bordeaux - Hopital Haut-Leveque

🇫🇷

Pessac, France

Charite - UB - CVK - Medizinische Klinik

🇩🇪

Berlin, BE, Germany

Herzzentrum München

🇩🇪

Munich, BY, Germany

Children's Health Ireland

🇮🇪

Dublin 12, D12 N512, Ireland

Azienda Ospedaliero Universitaria Meyer

🇮🇹

Firenze, FI, Italy

Istituto Giannina Gaslini

🇮🇹

Genova, GE, Italy

AORN - Ospedali dei Colli Ospedale V. Monaldi

🇮🇹

Napoli, Italy

Hospital Sant Joan de Déu Barcelona

🇪🇸

Barcelona, B, Spain

Local Institution - 0021

🇪🇸

Granada, GR, Spain

Local Institution - 0048

🇪🇸

Madrid, M, Spain

Local Institution - 0011

🇪🇸

A Coruna, Spain

Local Institution - 0023

🇪🇸

Madrid, Spain

Hospital Universitario La Paz

🇪🇸

Madrid, Spain

Great Ormond Street Hospital for Children NHS Foundation Trust - Great Ormond Street Hospital

🇬🇧

London, United Kingdom

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