Efficacy and Safety of AG10 in Subjects With Transthyretin Amyloid Cardiomyopathy

NCT ID: NCT03860935

Last Updated: 2024-06-27

Study Results

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Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE3

Total Enrollment

632 participants

Study Classification

INTERVENTIONAL

Study Start Date

2019-03-19

Study Completion Date

2023-05-11

Brief Summary

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Phase 3 efficacy and safety study to evaluate acoramidis (AG10) HCl 800 mg administered orally twice a day compared to placebo in subjects with symptomatic Transthyretin Amyloid Cardiomyopathy (ATTR-CM).

Detailed Description

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Transthyretin amyloid cardiomyopathy (ATTR-CM) is an underdiagnosed condition believed to affect more than 400,000 people worldwide. In ATTR-CM, the accumulation of transthyretin (TTR) amyloid results in thickening and stiffening of the heart, which often leads to heart failure or even death.

There are two forms of ATTR-CM:

* Wild Type\* This form of the condition primarily develops in older individuals who do not carry gene mutations.
* Hereditary\* This form of the condition comes from gene mutations passed down in families.

In this study we are researching the investigational drug acoramidis HCl 800 mg administered orally twice a day. Through the study, we want to evaluate the efficacy and safety of acoramidis in patients with ATTR-CM versus placebo.

This is a 30 month, randomized, double-blind, placebo-controlled study. This means that, during the 30 month study, investigators conducting the research and study participants will not know whether the study participant is receiving acoramidis or placebo.

The primary outcomes of the study are:

1. The impact of acoramidis versus placebo on the change in distance walked on the 6 minute walk test (6MWT) after 12 months of treatment compared to baseline.
2. The impact of acoramidis versus placebo on the hierarchical combination of All-Cause mortality, cumulative frequency of cardiovascular-related hospitalizations, change from baseline in NT-proBNP, and change in from baseline in 6MWT over a 30-month fixed treatment duration.

At the end of 30 months, participants may be eligible to receive investigational acoramidis, and there is no placebo. This is called an "open label extension." This separate study may help us better understand the safety related to taking acoramidis over a longer period of time.

Conditions

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Amyloidosis Amyloid Cardiomyopathy Transthyretin Amyloidosis Cardiomyopathies Heart Diseases

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors

Study Groups

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acoramidis HCl 800 mg

Subjects will receive acoramidis HCl 800 mg twice daily. 6MWT primary outcome will be assessed at the end of 12 months. The hierarchical combination of All-Cause mortality, cumulative frequency of cardiovascular-related hospitalizations, change from baseline in NT-proBNP levels, and change from baseline in distance walked on the 6MWT will be assessed after 30 months of treatment.

Group Type EXPERIMENTAL

acoramidis

Intervention Type DRUG

TTR stabilizer administered orally twice daily (BID)

Placebo

Subjects will receive placebo to match twice daily. 6MWT primary outcome will be assessed at the end of 12 months. The hierarchical combination of All-Cause mortality, cumulative frequency of cardiovascular-related hospitalizations, change from baseline in NT-proBNP levels, and change from baseline in distance walked on the 6MWT will be assessed after 30 months of treatment.

Group Type PLACEBO_COMPARATOR

Placebo Oral Tablet

Intervention Type DRUG

Non-active control administered orally twice daily (BID)

Interventions

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acoramidis

TTR stabilizer administered orally twice daily (BID)

Intervention Type DRUG

Placebo Oral Tablet

Non-active control administered orally twice daily (BID)

Intervention Type DRUG

Other Intervention Names

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AG10 ALXN2060

Eligibility Criteria

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Inclusion Criteria

* Have an established diagnosis of ATTR-CM with either wild-type TTR or variant TTR genotype
* Have a history of heart failure evidenced by at least one prior hospitalization for heart failure or clinical evidence of heart failure without prior heart failure hospitalization manifested by signs or symptoms of volume overload or elevated intracardiac pressures or heart failure symptoms that required or require ongoing treatment with a diuretic.
* New York Heart Association (NYHA) Class I-III symptoms due to ATTR cardiomyopathy.
* On stable doses of cardiovascular medical therapy
* Completed ≥150 m on the 6MWT on 2 tests that are within 15% of total distance walked prior to randomization
* Biomarkers of myocardial wall stress, NT-proBNP level ≥300 pg/mL at screening
* Have left ventricular wall (interventricular septum or left ventricular posterior wall) thickness ≥12 mm

Exclusion Criteria

* Had acute myocardial infarction, acute coronary syndrome or coronary revascularization, or experienced stroke or transient ischemic attack within 90 days prior to screening
* Has hemodynamic instability
* Likely to undergo heart transplantation within a year of screening
* Confirmed diagnosis of primary (light chain) amyloidosis
* Biomarkers of myocardial wall stress, NT-proBNP level ≥8500 pg/mL at screening
* Measure of kidney function, eGFR by MDRD formula \<15 mL/min/1.73 m2
* Current treatment with marketed drug products and other investigational agents for the treatment of ATTR-CM
* Current treatment with calcium channel blockers with conduction system effects (e.g. verapamil, diltiazem). The use of dihydropyridine calcium channel blockers is allowed. The use of digitalis will only be allowed if required for management of atrial fibrillation with rapid ventricular response
Minimum Eligible Age

18 Years

Maximum Eligible Age

90 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Eidos Therapeutics, a BridgeBio company

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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Cedars-Sinai Medical Center

Los Angeles, California, United States

Site Status

Pacific Heart Institute

Santa Monica, California, United States

Site Status

University of Colorado Hospital - Anschutz Medical Campus

Aurora, Colorado, United States

Site Status

Yale School of Medicine

New Haven, Connecticut, United States

Site Status

MedStar Washington Hospital Center

Washington D.C., District of Columbia, United States

Site Status

University of Miami - Sylvester Comprehensive Cancer Center

Miami, Florida, United States

Site Status

Piedmont Heart Institute Athens

Athens, Georgia, United States

Site Status

Emory Heart and Vascular Center

Atlanta, Georgia, United States

Site Status

Northwestern University

Chicago, Illinois, United States

Site Status

The University of Chicago Medical Center

Chicago, Illinois, United States

Site Status

NorthShore University Health System

Evanston, Illinois, United States

Site Status

Indiana University

Indianapolis, Indiana, United States

Site Status

MedStar Medical Group Cardiology at Franklin Square Medical Center

Baltimore, Maryland, United States

Site Status

Boston University School of Medicine

Boston, Massachusetts, United States

Site Status

Saint Elizabeth's Medical Center

Boston, Massachusetts, United States

Site Status

Mayo Clinic

Rochester, Minnesota, United States

Site Status

Saint Luke's Hospital - Kansas City

Kansas City, Missouri, United States

Site Status

Washington University School of Medicine

St Louis, Missouri, United States

Site Status

Newark Beth Israel Medical Center

Newark, New Jersey, United States

Site Status

North Shore University Hospital

Manhasset, New York, United States

Site Status

New York University Langone Health

New York, New York, United States

Site Status

Mount Sinai Hospital

New York, New York, United States

Site Status

Columbia University Medical Center

New York, New York, United States

Site Status

Laurelton Heart Specialist

Rosedale, New York, United States

Site Status

Montefiore Medical Center

The Bronx, New York, United States

Site Status

University of North Carolina - Chapel Hill

Chapel Hill, North Carolina, United States

Site Status

Duke University Health System

Durham, North Carolina, United States

Site Status

Cleveland Clinic

Cleveland, Ohio, United States

Site Status

Oregon Health and Science University

Portland, Oregon, United States

Site Status

Penn Presbyterian Medical Center

Philadelphia, Pennsylvania, United States

Site Status

Allegheny General Hospital

Pittsburgh, Pennsylvania, United States

Site Status

University of Pittsburgh

Pittsburgh, Pennsylvania, United States

Site Status

The Medical University of South Carolina

Charleston, South Carolina, United States

Site Status

Prisma Health - Greenville Memorial Hospital

Greenville, South Carolina, United States

Site Status

University of Texas Southwestern Medical Center

Dallas, Texas, United States

Site Status

University of Utah

Salt Lake City, Utah, United States

Site Status

Virginia Commonwealth University Medical Center

Richmond, Virginia, United States

Site Status

Carilion Clinic Roanoke Heart Institute

Roanoke, Virginia, United States

Site Status

University of Washington School of Medicine

Seattle, Washington, United States

Site Status

Providence Sacred Heart Medical Center

Spokane, Washington, United States

Site Status

Royal Adelaide Hospital

Adelaide, , Australia

Site Status

Box Hill Hospital

Box Hill, , Australia

Site Status

Royal Hobart Hospital

Hobart, , Australia

Site Status

Fiona Stanley Hospital

Murdoch, , Australia

Site Status

Saint Vincent's Hospital Sydney

Sydney, , Australia

Site Status

Princess Alexandra Hospital

Woolloongabba, , Australia

Site Status

Ziekenhuis Oost-Limburg - Campus Sint-Jan

Genk, Limburg, Belgium

Site Status

Jessa Ziekenhuis - Campus Virga Jesse

Hasselt, Limburg, Belgium

Site Status

Algemeen Ziekenhuis Sint-Jan Brugge-Oostende

Bruges, West Vlaanderen, Belgium

Site Status

Onze-Lieve-Vrouw Ziekenhuis Aalst

Aalst, , Belgium

Site Status

Universitair Ziekenhuis Leuven

Leuven, , Belgium

Site Status

Hospital Cárdio Pulmonar

Salvador, Estado de Bahia, Brazil

Site Status

Santa Casa de Misericordia - Porto Alegre

Porto Alegre, Rio Grande do Sul, Brazil

Site Status

CAPED - Centro Avançado de Pesquisa e Estudos para o Diagnóstico

Ribeirão Preto, São Paulo, Brazil

Site Status

INCOR

São Paulo, São Paulo, Brazil

Site Status

Instituto de Cardiologia do Rio Grande do Sul

Porto Alegre, , Brazil

Site Status

University of Calgary

Calgary, Alberta, Canada

Site Status

University of British Columbia

Vancouver, British Columbia, Canada

Site Status

CancerCare Manitoba - St. Boniface

Winnipeg, Manitoba, Canada

Site Status

Halifax Infirmary

Halifax, Nova Scotia, Canada

Site Status

Toronto Heart Centre

Toronto, Ontario, Canada

Site Status

University of Toronto

Toronto, Ontario, Canada

Site Status

Centre Hospitalier de L'Universite de Montreal - Hôpital Notre-Dame

Montreal, Quebec, Canada

Site Status

Hôpital du Sacré-Coeur de Montréal

Montreal, Quebec, Canada

Site Status

Institut Universitaire de Cardiologie et de Pneumologie de Québec

Québec, Quebec, Canada

Site Status

Hôpital régional de Rimouski

Rimouski, Quebec, Canada

Site Status

Montreal Heart Institute

Montreal, , Canada

Site Status

St. Anne´s University Hospital

Brno střed, , Czechia

Site Status

General University Hospital in Prague

New Town, , Czechia

Site Status

Institute for Clinical and Experimental Medicine

Prague, , Czechia

Site Status

Aarhus Universitetshospital

Aarhus, Dinamarca, Denmark

Site Status

Alexandra General Hospital of Athens

Athens, Attica, Greece

Site Status

Mater Misericordiae University Hospital

Dublin, , Ireland

Site Status

Saint Vincents University Hospital

Dublin, , Ireland

Site Status

Hadassah University Hospital Ein Kerem

Jerusalem, , Israel

Site Status

The Chaim Sheba Medical Center

Tel Litwinsky, , Israel

Site Status

Ospedale San Donato

Arezzo, , Italy

Site Status

Azienda Ospedaliero Universitaria di Bologna Policlinico Sant'Orsola-Malpighi

Bologna, , Italy

Site Status

Azienda Ospedaliero - Universitaria Careggi

Florence, , Italy

Site Status

Fondazione IRCCS Policlinico San Matteo

Pavia, , Italy

Site Status

Fondazione Toscana Gabriele Monasterio per la Ricerca Medica e di Sanità Pubblica

Pisa, , Italy

Site Status

Ospedale degli Infermi

Rimini, , Italy

Site Status

Universita degli Studi di Roma La Sapienza - Umberto I Policlinico di Roma

Roma, , Italy

Site Status

Maastricht Universitair Medisch Centrum

Maastricht, Limburg, Netherlands

Site Status

Universitair Medisch Centrum Groningen

Groningen, , Netherlands

Site Status

Universitair Medisch Centrum Utrecht

Utrecht, , Netherlands

Site Status

Middlemore Hospital

Otahuhu, Auckland, New Zealand

Site Status

Waikato Hospital

Hamilton, Waikato Region, New Zealand

Site Status

National Institute of Cardiology

Warsaw, , Poland

Site Status

Centro Hospitalar de Lisboa Norte EPE- Hospital Santa Maria

Lisbon, , Portugal

Site Status

Centro Hospitalar do Porto

Porto, , Portugal

Site Status

Seoul National University Bundang Hospital

Seongnam-si, Gyeonggi-do, South Korea

Site Status

Seoul National University Hospital

Seoul, Gyeonggi-do, South Korea

Site Status

Clínica Universidad de Navarra

Pamplona, Navarre, Spain

Site Status

Hospital Universitari Germans Trias i Pujol

Barcelona, , Spain

Site Status

Hospital Juan Ramón Jiménez

Huelva, , Spain

Site Status

Hospital Juan Ramón Jiménez

Huelva, , Spain

Site Status

Clinica Universidad de Navarra Madrid

Madrid, , Spain

Site Status

Hospital Universitario Puerta de Hierro

Madrid, , Spain

Site Status

Hospital Son Llàtzer

Palma de Mallorca, , Spain

Site Status

Hospital Clínico Universitario de Santiago de Compostela

Santiago de Compostela, , Spain

Site Status

Hospital Clínico Universitario de Valencia

Valencia, , Spain

Site Status

Royal Free Hospital

London, England, United Kingdom

Site Status

Richmond Pharmacology

London, , United Kingdom

Site Status

Countries

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United States Australia Belgium Brazil Canada Czechia Denmark Greece Ireland Israel Italy Netherlands New Zealand Poland Portugal South Korea Spain United Kingdom

References

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Dasgupta N, Poulsen SH, Emdin M, Ambardekar AV, Shah KB, Hennum L, Marwah R, Allison M, Shivanna P, Siddhanti S, Tamby JF, Falvey H, Grodin JL. Contemporary Oral Medication Use and Frequency in Patients with Transthyretin Amyloid Cardiomyopathy. Am J Cardiovasc Drugs. 2025 Aug 1. doi: 10.1007/s40256-025-00752-x. Online ahead of print.

Reference Type DERIVED
PMID: 40750720 (View on PubMed)

Maurer MS, Judge DP, Gillmore JD, Garcia-Pavia P, Masri A, Cappelli F, Alexander KM, Sarswat N, Grogan M, Ambardekar AV, Ducharme A, Poulsen SH, Lam K, Obici L, Soman P, Rao S, Tamby JF, Castano A, Fox JC, Adam B, Chepyala SR, Poland B, Sinha U, Fontana M. Early Increase in Serum Transthyretin by Acoramidis Independently Predicts Improved Survival in TTR Amyloid Cardiomyopathy. J Am Coll Cardiol. 2025 May 27;85(20):1911-1923. doi: 10.1016/j.jacc.2025.03.542.

Reference Type DERIVED
PMID: 40398971 (View on PubMed)

Judge DP, Alexander KM, Cappelli F, Fontana M, Garcia-Pavia P, Gibbs SDJ, Grogan M, Hanna M, Masri A, Maurer MS, Obici L, Soman P, Cao X, Lystig T, Tamby JF, Siddhanti S, Castano A, Katz L, Fox JC, Mahaffey KW, Gillmore JD. Efficacy of Acoramidis on All-Cause Mortality and Cardiovascular Hospitalization in Transthyretin Amyloid Cardiomyopathy. J Am Coll Cardiol. 2025 Mar 18;85(10):1003-1014. doi: 10.1016/j.jacc.2024.11.042.

Reference Type DERIVED
PMID: 40074465 (View on PubMed)

Soman P, Khouri MG, Lenihan D, Reyentovich A, Sperry BW, Sowalsky K, Bai Y, Du J, Katz L, Siddhanti S, Fox JC. Comparison of in-clinic assessment of 6MWT by conventional method and using wearable sensors for patients with ATTR-CM. Future Cardiol. 2025 Feb;21(2):75-81. doi: 10.1080/14796678.2025.2457881. Epub 2025 Jan 29.

Reference Type DERIVED
PMID: 39878480 (View on PubMed)

Gillmore JD, Judge DP, Cappelli F, Fontana M, Garcia-Pavia P, Gibbs S, Grogan M, Hanna M, Hoffman J, Masri A, Maurer MS, Nativi-Nicolau J, Obici L, Poulsen SH, Rockhold F, Shah KB, Soman P, Garg J, Chiswell K, Xu H, Cao X, Lystig T, Sinha U, Fox JC; ATTRibute-CM Investigators. Efficacy and Safety of Acoramidis in Transthyretin Amyloid Cardiomyopathy. N Engl J Med. 2024 Jan 11;390(2):132-142. doi: 10.1056/NEJMoa2305434.

Reference Type DERIVED
PMID: 38197816 (View on PubMed)

Stern LK, Kittleson MM. Updates in Cardiac Amyloidosis Diagnosis and Treatment. Curr Oncol Rep. 2021 Mar 16;23(4):47. doi: 10.1007/s11912-021-01028-8.

Reference Type DERIVED
PMID: 33725199 (View on PubMed)

Provided Documents

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Document Type: Study Protocol

View Document

Document Type: Statistical Analysis Plan

View Document

Other Identifiers

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2018-004280-32

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

AG10-301

Identifier Type: -

Identifier Source: org_study_id

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