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Active, not recruitingNCT04988386AG10Updated Oct 14, 2025

Open-Label Safety Study of Acoramidis (AG10) in Symptomatic ATTR Participants

A Phase 3 interventional study of Acoramidis (AG10) in Amyloid Cardiomyopathy, Transthyretin-Related, sponsored by Eidos Therapeutics, a BridgeBio company. Active, not recruiting at 72 sites in 17 countries. Open to participants aged 18 Years to 90 Years. Per ClinicalTrials.gov, last updated 2025-10-14.

Sponsored by Eidos Therapeutics, a BridgeBio company · Phase 3, Interventional, and Treatment

Phase
Phase 3
Study type
Interventional
Enrollment
389
Allocation
Not applicable
Ages
18 Years to 90 Years
Sex
All
01

Study summary

Open-Label Extension and Safety Monitoring Study of Acoramidis (AG10) in Participants with Symptomatic Transthyretin Amyloid Cardiomyopathy Who Completed the Phase 3 ATTRibute-CM Trial (AG10-301)

Read the detailed description

The primary objective of this prospective, multi-center, open-label study is to evaluate the long-term safety and tolerability of acoramidis in patients with an established diagnosis of ATTR-CM and heart failure who are concomitantly treated with currently recommended heart failure therapies. Secondary efficacy and PD objectives will be assessed. Exploratory objectives may also be assessed.

All participants who complete 30 months of blinded study treatment and the Month 30 assessments of the double-blind treatment period of the Phase 3 ATTRibute-CM trial (AG10-301) may be eligible to participate in this Open Label Extension (OLE) study of acoramidis. The Day 1 visit in Study AG10-304 may be the same as the Month 30 visit in Study AG10-301. Under these circumstances, the last dose of Investigational Medicinal Product (IMP) in Study AG10-301 (either blinded acoramidis or matching placebo) will be the night before the day of the Month 30 visit. The first dose of open-label acoramidis in Study AG10-304 will be during the Study AG10-304 Day 1 visit after baseline assessments have been completed.

Currently, tafamidis is approved for the treatment of ATTR-CM in some regions. Participants are not allowed to be treated with tafamidis or any other ATTR-CM-specific approved or investigational treatment, or therapies used off-label or as non-prescription supplements for ATTR-CM at any time during the study. If participants choose treatment with an alternative treatment as described above, they will be asked to discontinue acoramidis and complete an End of Treatment (EoT) form, and they may be asked to discontinue/withdraw from the study. If a participant discontinues/withdraws from the study, the participant will be asked to complete an early termination visit and a safety follow-up visit.

Participants are not permitted to participate in another interventional clinical trial (except Study AG10-301) within 30 days prior to dosing and throughout Study AG10-304. Participants who choose to participate in another interventional clinical trial will be asked to withdraw from acoramidis and complete an EoT form, and they may be asked to discontinue/withdraw from the study. If a participant discontinues/withdraws from the study, the participant will be asked to complete an early termination visit and a safety follow-up visit. Participation in observational and/or registry studies should be discussed with the Medical Monitor.

02

Conditions studied

  • Amyloid Cardiomyopathy, Transthyretin-Related

Keywords

  • ATTR-CM
03

Who can participate

Ages eligible
18 Years to 90 Years
Sexes eligible
All
Accepts healthy volunteers
No

Inclusion criteria

  1. Completed 30 months of the blinded study treatment in Study AG10-301 and the Study AG10-301 Month 30 visit including assessments and procedures.
  2. Have the ability to understand and sign a written informed consent form, which must be obtained prior to initiation of study procedures.
  3. Female participants of childbearing potential who engage in heterosexual intercourse must agree to use a highly effective method of contraception beginning with enrollment and continuing for 30 days after the last dose of acoramidis. Female participants using oral contraceptives must agree to use an additional birth control method. While not considered highly effective, a double-barrier method is acceptable. A male participant who is sexually active with a female of childbearing potential and has not had a vasectomy must agree to use a double-barrier method of birth control.

Exclusion criteria

Exclusion Criteria:

  1. Acute myocardial infarction, acute coronary syndrome or coronary revascularization within 90 days prior to Day 1 stroke or transient ischemic attack (TIA) within 90 days prior to Day 1.
  2. Has hemodynamic instability, that in the judgment of the Investigator, would pose too great a risk for participation in the study.
  3. Has had a heart and/or liver transplant or is on the heart transplantation list within the year prior to Day 1
  4. Has had implantation of a cardiac mechanical assist device (CMAD) or is scheduled for implantation of a CMAD
  5. Has confirmed diagnosis of light-chain (AL) amyloidosis at any time during Study AG10-301.
  6. Has estimated glomerular filtration rate (eGFR) by modification of diet for renal disease (MDRD) formula \< 15 mL/min/1.73 m2 at Month 27 of Study AG10-301 or at any subsequent central lab value prior to Day 1.
  7. Known hypersensitivity to acoramidis, its metabolites, or formulation excipients.
  8. At the end of Study AG10-301 or at Day 1 of Study AG10-304 (or any time during the study), participant is on prohibited medication.
  9. Females who are pregnant or breastfeeding. A negative urine pregnancy test at the Day 1 visit and at each study visit are required for female participants of childbearing potential.
  10. In the judgment of the Investigator or Medical Monitor, has any clinically important ongoing medical condition or laboratory abnormality or condition that might jeopardize the participant's safety, increase their risk from participation, or interfere with the study.
  11. Participation in another interventional clinical trial (with the exception of Study AG10-301) within 30 days prior to dosing. Participation in observational and/or registry studies should be discussed with the Medical Monitor.
  12. Has any condition that in the opinion of the Investigator or Medical Monitor would preclude compliance with the study protocol such as a history of substance abuse, alcoholism, or a psychiatric condition.
04

Study design

Phase
Phase 3
Primary purpose
Treatment
Allocation
Not applicable
Intervention model
Single group
Masking
None (open label)
Enrollment
389 participants (actual)

Study arms

  • Experimental
    AG10

    Open-label study all participants will receive AG10 during this study.

    Drug: Acoramidis (AG10)

Interventions

  • DrugAcoramidis (AG10)

    Acoramidis (AG10) twice daily

    Also known as: AG10, ALXN 2060

05

What researchers measure

Primary outcomes

  1. Incidence of treatment-emergent Adverse Events [Safety and Tolerability]

    Incidence of each treatment-emergent adverse events measured over 60 Months or study completion

    Time frame: 60 months

Secondary outcomes

  1. Evaluate all-cause mortality and cardiovascular mortality

    Assessment of All-cause mortality and CV mortality during study period

    Time frame: 60 months

  2. Evaluate the effect of acoramidis on the 6-minute walk test (6MWT)

    Change from Baseline in distance walked during the 6MWT during study period

    Time frame: 60 months

  3. Evaluate the effect of acoramidis on health-related quality of life Kansas City Cardiomyopathy Questionnaire

    Change from Baseline in Kansas City Cardiomyopathy Questionnaire Overall Score during study period \[scale is scored from 0-100, where 0=Poor, 100=Excellent\]

    Time frame: 60 months

  4. Evaluate the effect of acoramidis on the frequency of CV-related hospitalization

    assess CV-related hospitalization during the study period

    Time frame: 60 months

06

Study locations

72 sites
  • Cedars-Sinai Medical Center
    Los Angeles, California 90048, United States
  • University of Colorado Hospital - Anschutz Medical Campus
    Aurora, Colorado 80045, United States
  • Northwestern University
    Chicago, Illinois 60611, United States
  • University of Chicago Medical Center
    Chicago, Illinois 60637, United States
  • NorthShore University Health System
    Evanston, Illinois 60201, United States
  • MedStar Medical Group Cardiology at Franklin Square
    Baltimore, Maryland 21237, United States
  • Boston University Medical Center General Clinical Research Unit
    Boston, Massachusetts 02118, United States
  • Mayo Clinic - Rochester
    Rochester, Minnesota 55905, United States
  • Saint Luke's Hospital of Kansas City
    Kansas City, Missouri 64111, United States
  • Washington University
    St Louis, Missouri 63110, United States
  • New York University Langone Health
    New York, New York 10016, United States
  • Columbia University Irving Medical Center
    New York, New York 10032, United States
  • Laurelton Heart Specialists
    Rosedale, New York 11422, United States
  • Montefiore Medical Center at Hutchinson
    The Bronx, New York 10461, United States
  • Duke University Health System - Duke Clinic
    Durham, North Carolina 27710, United States
  • Cleveland Clinic Main Campus
    Cleveland, Ohio 44195, United States
  • Oregon Health & Science University
    Portland, Oregon 97239, United States
  • Penn Presbyterian Medical Center
    Philadelphia, Pennsylvania 19104, United States
  • University of Pittsburgh Medical Center (UPMC)
    Pittsburgh, Pennsylvania 15213, United States
  • Medical University of South Carolina
    Charleston, South Carolina 29425, United States
  • University of Texas Southwestern Medical Center
    Dallas, Texas 75390, United States
  • University of Utah
    Salt Lake City, Utah 84132, United States
  • Virginia Commonwealth University Health System
    Richmond, Virginia 23219, United States
  • Roanoke Heart Institute
    Roanoke, Virginia 24014, United States
  • University of Washington School of Medicine
    Seattle, Washington 98195, United States
  • Providence Sacred Heart Children's Hospital
    Spokane, Washington 99204, United States
  • Saint Vincent's Hospital Sydney
    Darlinghurst, New South Wales 2010, Australia
  • Princess Alexandra Hospital
    Woolloongabba, Queensland 4102, Australia
  • Royal Adelaide Hospital
    Adelaide, South Australia 5000, Australia
  • Royal Hobart Hospital
    Hobart, Tasmania 7000, Australia
  • Box Hill Hospital
    Box Hill, Victoria 3128, Australia
  • Fiona Stanley Hospital
    Murdoch, Western Australia 6150, Australia
  • Ziekenhuis Oost-Limburg
    Genk, Limburg 3600, Belgium
  • Algemeen Ziekenhuis Sint-Jan Brugge-Oostende - Campus Sint-Jan
    Bruges, West Vlaanderen 8000, Belgium
  • Instituto de Cardiologia do Rio Grande do Sul
    Porto Alegre, Rio Grande do Sul 90620-001, Brazil
  • Centro Avançado de Pesquisa e Estudos para o Diagnóstico CAPED
    Ribeirão Preto, São Paulo 14026-900, Brazil
  • Instituto do Coração de São Paulo
    São Paulo, São Paulo 05403-000, Brazil
  • Vancouver General Hospital
    Vancouver, British Columbia V5Z 1M9, Canada
  • Saint Boniface Hospital
    Winnipeg, Manitoba R2H 2A6, Canada
  • Queen Elizabeth II Health Sciences Centre - Halifax Infirmary
    Halifax, Nova Scotia B3H 3A7, Canada
  • Toronto Heart Centre
    Toronto, Ontario M4P IE4, Canada
  • L' Institut de Cardiologie de Montréal
    Montreal, Quebec H1T 1C8, Canada
  • Centre de Recherche du Centre Hospitalier de l'Université de Montréal (CHUM)
    Montreal, Quebec H2X 0A9, Canada
  • Hôpital du Sacré-Coeur de Montréal
    Montreal, Quebec H4J 1C5, Canada
  • Hôpital régional de Rimouski
    Rimouski, Quebec G5L 5T1, Canada
  • Vseobecna Fakultni Nemocnice v Praze
    Prague, Prague 128 08, Czechia
  • Institut Klinické a Experimentální Medicíny
    Prague, Prague 140 21, Czechia
  • Fakultní Nemocnice u sv. Anny v Brně
    Brno, South Moravian 656 91, Czechia
  • Aarhus Universitets Hospital
    Aarhus N, Palle Juul-Jensens 8200, Denmark
  • Alexandra General Hospital of Athens
    Athens, Attica 11528, Greece
  • Mater Misericordiae University Hospital
    Dublin, Dublin 7, Ireland
  • The Chaim Sheba Medical Center
    Ramat Gan, Tel Aviv 5265601, Israel
  • Hadassah University Hospital Ein Kerem
    Jerusalem, 9112001, Israel
  • Ospedale San Donato
    Arezzo, Arezzo 52100, Italy
  • Azienda Ospedaliero - Universitaria Careggi
    Florence, Florence 50134, Italy
  • Fondazione Toscana Gabriele Monasterio per la Ricerca Medica e di Sanità Pubblica
    Pisa, Pisa 56124, Italy
  • Fondazione IRCCS Policlinico San Matteo
    Pavia, 27100, Italy
  • Universitair Medisch Centrum Groningen
    Groningen, Provincie Groningen 9713 GZ, Netherlands
  • Universitair Medisch Centrum Utrecht
    Utrecht, Utrecht 3584 CX, Netherlands
  • Middlemore Clinical Trials - Middlemore Hospital
    Papatoetoe, Auckland 2025, New Zealand
  • Waikato Hospital
    Hamilton, Waikato Region 3240, New Zealand
  • Centro Hospitalar Universitário Lisboa Norte EPE- Hospital Santa Maria
    Lisbon, Lisbon District 1649-035, Portugal
  • Seoul National University Bundang Hospital
    Seongnam-si, Gyeonggi-do 13620, South Korea
  • Seoul National University Hospital
    Seoul, Gyeonggi-do 3080, South Korea
  • Hospital Son Llàtzer
    Palma de Mallorca, Balearic Islands 7198, Spain
  • Complejo Hospitalario Universitario de Santiago (CHUS)
    Santiago de Compostela, La Coruña 15706, Spain
  • Clinica Universidad de Navarra Madrid
    Madrid, Madrid 28027, Spain
  • Hospital Universitario Puerta de Hierro - Majadahonda
    Majadahonda, Madrid 28222, Spain
  • Clínica Universidad de Navarra
    Pamplona, Navarre 31008, Spain
  • Hospital Clínico Universitario de Valencia
    Valencia, Valencia 46010, Spain
  • National Amyloidosis Centre, Division of Medicine - Royal Free
    London, England NW3 2PF, United Kingdom
  • Richmond Pharmacology Ltd
    London, England SE1 1YR, United Kingdom
07

References and documents

Publications

  • Testani JM, Judge DP, Borlaug BA, Cherney D, Cox ZL, Gillmore JD, Lewis JB, Adler SH, Cao X, Castano A, Fox JC, Katz L, Mathur V, Xiong K, Butler J, Masri A. Effects of Acoramidis on Kidney Function in Transthyretin Amyloid Cardiomyopathy. Circ Heart Fail. 2026 Jul 2:e014656. doi: 10.1161/CIRCHEARTFAILURE.126.014656. Online ahead of print. PubMed 42389794 ↗
  • Soman P, Cuddy SAM, Gillmore JD, Sarswat N, Judge DP, Cappelli F, Stern LK, Wright R, Akinboboye O, Obici L, Bhatt K, Xiong K, Castano A, Chen C, Falvey H, Pecoraro ML, Tamby JF, Sinha U, Fox JC, Shah K, Grodin JL, Ambardekar AV, Alexander KM, Khouri MG, Cheng RK. Long-Term Durability of Acoramidis Efficacy in Transthyretin Amyloid Cardiomyopathy: Open-Label Extension of the ATTRibute-CM Randomized Clinical Trial. JAMA Cardiol. 2026 May 1;11(5):446-454. doi: 10.1001/jamacardio.2026.0819. PubMed 41911489 ↗
  • Alexander KM, Davis MK, Akinboboye O, Berk J, Bhatt K, Cappelli F, Cuddy SAM, Fontana M, Garcia-Pavia P, Gillmore JD, Griffin JM, Grodin JL, Judge DP, Khouri MG, Lam K, Masri A, Maurer MS, Obici L, Ruberg FL, Sarswat N, Shah K, Soman P, Stern L, Wright R, Xiong K, Cao X, Lystig T, Tamby JF, Castano A, Katz L, Sinha U, Fox JC, Solomon SD, Grogan M. Efficacy of Acoramidis in Wild-Type and Variant Transthyretin Amyloid Cardiomyopathy: Results From ATTRibute-CM and Its Open-Label Extension. JAMA Cardiol. 2026 Jan 1;11(1):57-67. doi: 10.1001/jamacardio.2025.4477. PubMed 41205147 ↗
  • Judge DP, Gillmore JD, Alexander KM, Ambardekar AV, Cappelli F, Fontana M, Garcia-Pavia P, Grodin JL, Grogan M, Hanna M, Masri A, Nativi-Nicolau J, Obici L, Hvitfeldt Poulsen S, Sarswat N, Shah K, Soman P, Lystig T, Cao X, Wang K, Pecoraro ML, Tamby JF, Katz L, Sinha U, Fox JC, Maurer MS. Long-Term Efficacy and Safety of Acoramidis in ATTR-CM: Initial Report From the Open-Label Extension of the ATTRibute-CM Trial. Circulation. 2025 Mar 4;151(9):601-611. doi: 10.1161/CIRCULATIONAHA.124.072771. Epub 2024 Nov 18. PubMed 39556242 ↗

Individual participant data

Plan to share: Undecided

08

Registry details

Key details

Study ID
NCT04988386
Lead sponsor
Eidos Therapeutics, a BridgeBio company
Responsible party
Sponsor
First posted
Aug 3, 2021
Start date
Oct 27, 2021
Primary completion
Apr 15, 2028 (estimated)
Completion
May 15, 2028 (estimated)
Last update
Oct 14, 2025

Oversight

Data monitoring committee
Yes
FDA-regulated drug
Yes
FDA-regulated device
No
View the source record on ClinicalTrials.gov ↗

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