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Obstructive Hypertrophic CardiomyopathyPhase 3EXPLORER-HCMNCT03470545

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

A Phase 3 study testing mavacamten in people with Obstructive Hypertrophic Cardiomyopathy, run by MyoKardia, Inc. at 71 sites in 13 countries.

Completed

Finished. The study has ended and is not enrolling. Results may be posted on the official record.

Official record
Search all trials

Trial 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. Read more about this study

Who is it for?
Ages 18 years and older
Conditions the study is for
Obstructive Hypertrophic Cardiomyopathy
What is being tested?
mavacamten
Type of treatment
Pill or oral medicine
Could I receive a placebo?
Yes, some participants may receive a placebo
How many people
251 participants
Where
71 sitesin 13 countries
Study dates
29 May 2018to 6 May 2020
Phase
Phase 3
Are there results?
Yes, posted in the registrySummary in the doctors' view

Key requirements

Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.

  • Ages 18 years and older
  • All sexes
  • No healthy volunteers
  • Adult, Older adult

Who may be able to join

  • 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

Who cannot join

  • 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
Show all 12Show fewer
  • 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

Trial locations and contacts

71 sites in 13 countries.

71 sites in 13 countries

United States29 sites
  • University of Michigan

    Ann Arbor, Michigan, 48109

  • St. Luke's Cardiology Associates

    Bethlehem, Pennsylvania, 18018

  • Brigham and Women's Hospital

    Boston, Massachusetts, 02115

  • Carolinas Medical Center

    Charlotte, North Carolina, 28203

  • University of Virginia Health System

    Charlottesville, Virginia, 22903

  • Northwestern University

    Chicago, Illinois, 60611

  • Cincinnati Children's Hospital Medical Center

    Cincinnati, Ohio, 45229

  • University of Texas Southwestern Medical Center

    Dallas, Texas, 75235

  • Duke Cardiology at Southpoint

    Durham, North Carolina, 27710

  • Spectrum Health

    Grand Rapids, Michigan, 49512

  • Houston Methodist Hospital

    Houston, Texas, 77030

  • University of Texas Houston Medical School

    Houston, Texas, 77030

  • University Of Iowa Hospitals And Clinics

    Iowa City, Iowa, 52242

  • Mayo Clinic Jacksonville - PPDS

    Jacksonville, Florida, 32224

  • Cedars-Sinai Medical Center (Smidt Heart Institute)

    Los Angeles, California, 90048

  • Methodist University Hospital

    Memphis, Tennessee, 38163

  • Yale New Haven Hospital

    New Haven, Connecticut, 16511

  • NYU Langone Medical Center

    New York, New York, 10017

  • Columbia University Medical Center

    New York, New York, 10032

  • University of Pennsylvania (Penn Heart and Vascular Center)

    Philadelphia, Pennsylvania, 19104

  • University of Pittsburgh Medical Center Presbyterian

    Pittsburgh, Pennsylvania, 15213

  • Oregon Health & Science University

    Portland, Oregon, 97239

  • Intermountain Medical Center

    Salt Lake City, Utah, 84107

  • University of Utah

    Salt Lake City, Utah, 84132

  • UCSF School of Medicine

    San Francisco, California, 94143

  • Mayo Clinic Arizona

    Scottsdale, Arizona, 85259

  • University of Washington Medical Center

    Seattle, Washington, 98195

  • Washington University School of Medicine

    St Louis, Missouri, 63110

  • Stanford University

    Stanford, California, 94305

Other trials in United States

Germany7 sites
  • Kerckhoff-Klinik-Forschungs-GmbH

    Bad Nauheim, 61231

  • Charité Campus Buch - Experimental and Clinical Research Center

    Berlin, 13125

  • Charité - Universitätsmedizin Berlin

    Berlin, 13353

  • Cardiologicum Dresden und Pirna

    Dresden, 01277

  • University Medicine Göttingen

    Göttingen, Neidersachsen

  • University Clinic Heidelberg - PPDS

    Heidelberg, 69120

  • Universitatsklinikum Schleswig-Holstein

    Kiel, 24105

Other trials in Germany

Israel7 sites
  • Barzilai Medical Center

    Ashkelon, 78278

  • Hadassah Medical Center PPDS -

    Jerusalem, 91120

  • Rabin Medical Center - PPDS

    Petah Tikva, 49100

  • The Chaim Sheba Medical Center - The Edmond and Lily Safra Children's Hospital

    Ramat Gan, 52621

  • Kaplan Medical Center

    Rehovot, 76100

  • ZIV Medical Center

    Safed, 13100

  • Tel Aviv Sourasky Medical Center

    Tel Aviv, Tel Aviv, 62431

Other trials in Israel

Spain5 sites
  • Hospital Universitario A Coruña

    A Coruña

  • Hospital Universitario Virgen de La Arrixaca

    El Palmar, Murcia, 30120

  • Hospital Universitario Puerta de Hierro - Majadahonda

    Madrid

  • Hospital Universitario Ramon y Cajal

    Madrid

  • Hospital Universitario Virgen Macarena

    Seville, 41009

Other trials in Spain

France4 sites
  • CHRU Nantes

    Nantes, Loire-Atlantique, 44805

  • Groupe Hospitalier Pitié Salpétrière

    Paris, 75013

  • Hôpital Européen Georges Pompidou

    Paris, 75015

  • Hôpital de Rangueil

    Toulouse, 31403

Other trials in France

Poland4 sites
  • Kardio Klinika Brynów

    Katowice, Silesian Voivodeship

  • Collegium Medicum Uniwersytetu Jagiellonskiego

    Krakow, Lesser Poland Voivodeship, 31-501

  • Szpital Kliniczny Przemienienia Panskiego Uniwesytetu Medycznego im. Karola Marcinkowskiego

    Poznan, 61-848

  • Instytut Kardiologii im Prymasa Tysiaclecia Kardynala Stefana Wyszynskiego

    Warsaw, 04-628

Other trials in Poland

Belgium3 sites
  • Onze-Lieve-Vrouwziekenhuis

    Aalst, Oost-Vlaanderen, 9300

  • Hôpital Erasme

    Brussels, 1070

  • UZ Antwerpen

    Edegem, Antwerpen, 2650

Other trials in Belgium

Denmark3 sites
  • Aarhus Universitetshospital

    Aarhus N, 8200

  • Bispebjerg Hospital

    København NV, 2400

  • Odense Universitetshospital

    Odense, 5000

Other trials in Denmark

Czechia2 sites
  • Vseobecna fakultni nemocnice v Praze

    Prague, 128 08

  • Institut Klinicke a Experimentalni Mediciny

    Prague

Other trials in Czechia

Netherlands2 sites
  • Maastricht University Medical Center

    Maastricht, Limburg, 6229 HX

  • Erasmus MC

    Rotterdam, South Holland, 3015GD

Other trials in Netherlands

Portugal2 sites
  • Hospital Garcia de Orta

    Almada, 2805-267

  • Hospital da Luz

    Lisbon, 1500-650

Other trials in Portugal

United Kingdom2 sites
  • University Hospital of Wales

    Cardiff, South Glamergon, CF14 4XW

  • St Bartholomew's Hospital

    London, W1G 8PH

Other trials in United Kingdom

Italy1 site
  • Azienda Ospedaliera Universitaria Careggi

    Florence

Other trials in Italy

About this study

Official title: A Randomized, Double Blind, Placebo Controlled Clinical Study to Evaluate Mavacamten (MYK-461) in Adults With Symptomatic Obstructive Hypertrophic Cardiomyopathy

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.

Being tested
mavacamten, Placebo
Keywords
Symptomatic, left ventricular outflow tract gradient
Medical subject headings
Cardiomyopathy, Hypertrophic

What participants receive

What participants receive. In a randomised study each person is assigned to one group by chance.

  • mavacamtenDrug

    Also called MYK-461

    mavacamten capsules

  • PlaceboDrug

    placebo oral capsule

Study groups in NCT03470545
Study groupRoleWhat they receive
mavacamten (MYK-461)ExperimentalNot described
Receives: Drug: mavacamten
PlaceboPlacebo comparatorNot described
Receives: Drug: Placebo

What is being measured

The primary outcome is the main question the study is designed to answer.

  1. Percentage of Participants Achieving A Clinical Response

    A positive clinical response (value="YES") is defined as having achieved either an improvement of at least 1.5 mL/kg/min in peak oxygen consumption (pVO2) as determined by cardiopulmonary exercise testing (CPET) and a reduction of one or more class in New York Heart Association (NYHA) functional classification (e.g.I, II, III, or IV) -OR- an improvement of 3.0 mL/kg/min or more in pVO2 with no worsening in NYHA Functional Class.

    Measured: 30 weeks

Secondary outcomes (5)
  1. Changes From Baseline to Week 30 in Post Exercise in LVOT Peak Gradient.

    The post-exercise LVOT gradient was measured from echocardiograms obtained at baseline and week 30 following a study-specified exercise protocol and read by the Cardiovascular Imaging Core Laboratory (CICL, Boston MA). Change from baseline was determined as per the study statistical analysis plan and compared between treatment arms.

    Measured: 30 weeks

  2. Change From Baseline to Week 30 in pVO2 as Assessed by CPET

    Cardiopulmonary exercise testing (CPET) was performed at baseline and week 30 following a study-specified protocol and peak oxygen consumption (pVO2) was determined by the Cardiovascular Metabolic Disease Research Institute (CMDRI, Palo Alto, CA). Change from baseline was determined as per the study statistical analysis plan and compared between treatment arms.

    Measured: 30 weeks

  3. Proportion of Participants With at Least 1 Class Improvement in NYHA Functional Class From Baseline to Week 30

    New York Heart Association (NYHA) functional classification was determined by the principal investigator at baseline and at specified timepoints in the study. At baseline, all subjects were NYHA Class II or III. For the secondary outcome, NYHA class at Week 30 was compared to baseline and the proportion of subjects with an improvement of at least one class was determined, and the difference between treatment groups was analyzed. The proportion was also multiplied by 100 to provide the result as a percent.

    Measured: 30 weeks

  4. Change From Baseline to Week 30 in Participant-reported Health-related Quality of Life as Assessed by the KCCQ Score

    The Kansas City Cardiomyopathy Questionnaire (KCCQ) is a patient reported outcome instrument with minimum score = 0 and maximum score = 100 where higher score indicates better health status. There are no units to the score. The instrument utilizes a recall period of 2 weeks over which patients describe the frequency and severity of their symptoms, their physical and social limitations, and how they perceive their heart failure symptoms to affect their quality of life. The KCCQ clinical summary (KCCQ-CS) score, a prespecified secondary outcome of EXPLORER-HCM, combines the physical limitation and total symptom scores.

    Measured: 30 weeks

  5. Change From Baseline to Week 30 in Participant-reported Severity of HCM Symptoms as Assessed by the HCMSQ Score

    The Hypertrophic Cardiomyopathy Symptom Questionnaire (HCMSQ) is a patient reported outcome instrument that is a daily self-administered 11-item questionnaire. The HCMSQ assesses the core symptoms of HCM (tiredness/fatigue, heart palpitations, chest pain, dizziness, and shortness of breath). The Shortness of Breath domain score, a pre-specified secondary outcome of EXPLORER-HCM, assesses the frequency and severity of shortness of breath. The minimum score = 0 and maximum score = 18 where lower score indicates better health status. There are no units to the score.

    Measured: 30 weeks

Timeline

From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.

  1. First posted20 Mar 2018
  2. Study start29 May 2018
  3. Primary completion14 Mar 2020
  4. Study completion6 May 2020

Resources

More details

For doctors and researchers: how the study is designed, who runs it, and its registry IDs.

Study design
Study type
Interventional
Phase
Phase 3
Primary purpose
Treatment
Allocation
Randomised
Intervention model
Parallel groups
Masking
Quadruple blind
Who is masked
Participant, Care provider, Investigator, Outcomes assessor
Enrolment
251 participants (actual)
IDs, dates and publications
ClinicalTrials.gov ID
NCT03470545
Sponsor's protocol ID
MYK-461-005
Acronym
EXPLORER-HCM
Status last verified
May 2020
First submitted
12 Mar 2018
Last updated
4 Oct 2021
Data monitoring committee
Yes
FDA-regulated drug
Yes
FDA-regulated device
No

Publications

  • Olivotto I, Garcia-Pavia P, Tian Z, Owens AT, Lakdawala NK MD, Balaratnam G, Zhong Y, Memaj A, Barrett YC, Desai M. Mavacamten monotherapy in obstructive hypertrophic cardiomyopathy: An integrated analysis of phase 3 clinical trials. Am Heart J. 2026 Aug 24;303:107587. doi: 10.1016/j.ahj.2026.107587. Online ahead of print.

    PubMed 42636950
  • Saberi S, Naidu SS, Olivotto I, Hagege AA, Zhong Y, Lam J, Wyrwich KW, Fine JT, Dolan C, Allen V, Sehnert AJ, Reaney M. Impact of Mavacamten on Disease-Related Symptoms in Patients With Obstructive Hypertrophic Cardiomyopathy: HCMSQ Outcomes in EXPLORER-HCM. J Card Fail. 2026 Jun 6:S1071-9164(26)00257-5. doi: 10.1016/j.cardfail.2026.03.036. Online ahead of print.

    PubMed 42251960
  • Wang A, Lakdawala NK, Abraham TP, Nilles EK, Wojdyla DM, Owens AT, Bach RG, Saberi S, Sehnert A, Cresci S. Association Between age or Duration of Diagnosis in Obstructive Hypertrophic Cardiomyopathy and Response to Mavacamten Treatment: Exploratory Analysis of the EXPLORER-HCM Trial. J Card Fail. 2025 Jun;31(6):901-911. doi: 10.1016/j.cardfail.2024.10.449. Epub 2024 Dec 7.

    PubMed 39653325
  • Arnold SV, Gosch KL, Dolan C, Fine JT, Masri A, Saberi S, Wang A, Elliott PM, Hegde SM, Lam J, Sehnert AJ, Cresci S, Bach RG, Spertus JA. Association of Echocardiographic Parameters and Health Status in Patients With Obstructive Hypertrophic Cardiomyopathy: Insights From EXPLORER-HCM. Circulation. 2024 Nov 5;150(19):1560-1562. doi: 10.1161/CIRCULATIONAHA.123.067470. Epub 2024 Nov 4. No abstract available.

    PubMed 39495835
  • Sauer AJ, Sherrod CF, Gosch KL, Arnold SV, Reaney M, Zhong Y, Lam J, Wyrwich KW, Spertus JA. The Psychometric Performance of the Kansas City Cardiomyopathy Questionnaire-12 in Symptomatic Obstructive Hypertrophic Cardiomyopathy. J Card Fail. 2025 May;31(5):813-820. doi: 10.1016/j.cardfail.2024.09.010. Epub 2024 Sep 28.

    PubMed 39349160
  • Sherrod CF 4th, Spertus JA, Gosch KL, Wang A, Elliott PM, Lakdawala NK, Reaney M, Zhong Y, Lam J, Wyrwich KW, Sauer AJ. The Kansas City Cardiomyopathy Questionnaire in Relation to New York Heart Association Class. J Card Fail. 2025 Feb;31(2):481-484. doi: 10.1016/j.cardfail.2024.08.061. Epub 2024 Sep 28.

    PubMed 39349158
  • Garcia-Pavia P, Oreziak A, Masri A, Barriales-Villa R, Abraham TP, Owens AT, Jensen MK, Wojakowski W, Seidler T, Hagege A, Lakdawala NK, Wang A, Wheeler MT, Choudhury L, Balaratnam G, Shah A, Fox S, Hegde SM, Olivotto I. Long-term effect of mavacamten in obstructive hypertrophic cardiomyopathy. Eur Heart J. 2024 Dec 16;45(47):5071-5083. doi: 10.1093/eurheartj/ehae579.

    PubMed 39217450
  • Merali S, Salinger DH, Palmisano M, Sehnert AJ, Thanneer N, Back H, Seroogy JD, Gretler DD, Roy A, Perera V. Recommendation of mavacamten posology by model-based analyses in adults with obstructive hypertrophic cardiomyopathy. CPT Pharmacometrics Syst Pharmacol. 2024 Sep;13(9):1448-1461. doi: 10.1002/psp4.13138. Epub 2024 May 2.

    PubMed 38695527
  • Cresci S, Bach RG, Saberi S, Owens AT, Spertus JA, Hegde SM, Lakdawala NK, Nilles EK, Wojdyla DM, Sehnert AJ, Wang A. Effect of Mavacamten in Women Compared With Men With Obstructive Hypertrophic Cardiomyopathy: Insights From EXPLORER-HCM. Circulation. 2024 Feb 13;149(7):498-509. doi: 10.1161/CIRCULATIONAHA.123.065600. Epub 2023 Nov 14.

    PubMed 37961906
  • Wang A, Spertus JA, Wojdyla DM, Abraham TP, Nilles EK, Owens AT, Saberi S, Cresci S, Sehnert A, Lakdawala NK. Mavacamten for Obstructive Hypertrophic Cardiomyopathy With or Without Hypertension: Post-Hoc Analysis of the EXPLORER-HCM Trial. JACC Heart Fail. 2024 Mar;12(3):567-579. doi: 10.1016/j.jchf.2023.07.030. Epub 2023 Oct 18.

    PubMed 37855754
  • Wheeler MT, Olivotto I, Elliott PM, Saberi S, Owens AT, Maurer MS, Masri A, Sehnert AJ, Edelberg JM, Chen YM, Florea V, Malhotra R, Wang A, Oreziak A, Myers J. Effects of Mavacamten on Measures of Cardiopulmonary Exercise Testing Beyond Peak Oxygen Consumption: A Secondary Analysis of the EXPLORER-HCM Randomized Trial. JAMA Cardiol. 2023 Mar 1;8(3):240-247. doi: 10.1001/jamacardio.2022.5099.

    PubMed 36652223
  • Nassif M, Fine JT, Dolan C, Reaney M, Addepalli P, Allen VD, Sehnert AJ, Gosch K, Spertus JA. Validation of the Kansas City Cardiomyopathy Questionnaire in Symptomatic Obstructive Hypertrophic Cardiomyopathy. JACC Heart Fail. 2022 Aug;10(8):531-539. doi: 10.1016/j.jchf.2022.03.002. Epub 2022 May 4.

    PubMed 35902155
  • Reaney M, Addepalli P, Allen V, Spertus JA, Dolan C, Sehnert AJ, Fine JT. Longitudinal Psychometric Analysis of the Hypertrophic Cardiomyopathy Symptom Questionnaire (HCMSQ) Using Outcomes from the Phase III EXPLORER-HCM Trial. Pharmacoecon Open. 2022 Jul;6(4):575-586. doi: 10.1007/s41669-022-00340-8. Epub 2022 Jun 20.

    PubMed 35718845
  • Hegde SM, Lester SJ, Solomon SD, Michels M, Elliott PM, Nagueh SF, Choudhury L, Zemanek D, Zwas DR, Jacoby D, Wang A, Ho CY, Li W, Sehnert AJ, Olivotto I, Abraham TP. Effect of Mavacamten on Echocardiographic Features in Symptomatic Patients With Obstructive Hypertrophic Cardiomyopathy. J Am Coll Cardiol. 2021 Dec 21;78(25):2518-2532. doi: 10.1016/j.jacc.2021.09.1381.

    PubMed 34915982
  • Xie J, Wang Y, Xu Y, Fine JT, Lam J, Garrison LP. Assessing health-related quality-of-life in patients with symptomatic obstructive hypertrophic cardiomyopathy: EQ-5D-based utilities in the EXPLORER-HCM trial. J Med Econ. 2022 Jan-Dec;25(1):51-58. doi: 10.1080/13696998.2021.2011301.

    PubMed 34907813

Questions about this trial

What is this clinical trial studying?

A Phase 3 study testing mavacamten in people with Obstructive Hypertrophic Cardiomyopathy, run by MyoKardia, Inc. at 71 sites in 13 countries.

The full registered description is in the About this study section and the official record.

Who may be eligible to take part?

The sponsor lists 7 inclusion and 12 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.

Is this clinical trial currently recruiting?

The record says: finished, as of the sponsor's last update on 4 Oct 2021. Status can change between updates, so confirm with the study contact before making plans.

Where is the study taking place?

The record lists 71 sites in United States, Germany, Israel, Spain, France and 8 more countries. Whether a site is taking part right now should be confirmed with that site. See every site.

What are the possible benefits and risks?

They depend on the study and the treatment, and no study can promise a benefit. Some participants in this study may receive a placebo. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.

What does this phase mean?

Phase 3 studies compare the treatment with the current standard treatment or a placebo in a large group, to confirm how well it works and watch for side effects.

Not every study follows the same path, and some studies (observational studies, device studies) have no phase.

Are results available for this study?

Yes. Results are posted in the registry (first posted 4 Oct 2021). A summary is in the For doctors view, and the full results are on ClinicalTrials.gov.

How can a doctor refer a patient or contact the research team?

The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.

What does the primary outcome measure?

Percentage of Participants Achieving A Clinical Response (time frame: 30 weeks). It is what the study was designed to measure, not a finding.

Where can I read the original study information?

On the ClinicalTrials.gov record for NCT03470545, which also lists 19 linked publications.

Does a listing mean the treatment is approved or proven to work?

No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.

How often is this information updated?

AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 4 Oct 2021.

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