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A Study of Sotatercept in Participants With PAH WHO FC III or FC IV at High Risk of Mortality (MK-7962-006/ZENITH)

Phase 3
Completed
Conditions
Pulmonary Arterial Hypertension
Interventions
Other: Placebo
Registration Number
NCT04896008
Lead Sponsor
Acceleron Pharma, Inc., a wholly-owned subsidiary of Merck & Co., Inc., Rahway, NJ USA
Brief Summary

The objective of this study is to evaluate the effects of sotatercept (MK-7962, formerly called ACE-011) treatment (plus maximum tolerated background pulmonary arterial hypertension (PAH) therapy) versus placebo (plus maximum tolerated background PAH therapy) on time to first event of all cause death, lung transplantation, or PAH worsening-related hospitalization of ≥24 hours, in participants with World Health Organization (WHO) functional class (FC) III or FC IV PAH at high risk of mortality.

Detailed Description

This is a phase 3, randomized, double-blind, placebo-controlled study to evaluate sotatercept when added to maximum tolerated background PAH therapy on time to first event of all-cause death, lung transplantation, or PAH worsening related hospitalization of ≥24 hours, in participants with WHO FC III PAH or WHO FC IV PAH at high risk of mortality.

Participants with symptomatic PAH (WHO FC III or FC IV at high risk of mortality) who present with idiopathic or heritable PAH, PAH associated with connective tissue diseases (CTD), drug- or toxin-induced, post-shunt correction PAH, or PAH presenting at least 1 year following the correction of congenital heart defect. Participants must have a Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) Lite 2 risk score of ≥9 and be on maximum tolerated combination background PAH therapy.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
173
Inclusion Criteria
  • Documented diagnostic right heart catheterization prior to screening confirming the diagnosis of World Health Organization (WHO) pulmonary arterial hypertension (PAH) Group 1 in any of the following subtypes:

    • Idiopathic PAH
    • Heritable PAH
    • Drug/toxin-induced PAH
    • PAH associated with connective tissue diseases (CTD)
    • PAH associated with simple, congenital systemic to pulmonary shunts at least 1 year following repair
  • Symptomatic PAH classified as WHO functional class (FC) III or IV

  • Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) Lite 2.0 risk score of ≥9

  • Right heart catheterization performed during screening (or within 2 weeks prior to screening, if done at the clinical study site) documenting a minimum pulmonary vascular resistance (PVR) of ≥5 Wood units and a pulmonary capillary wedge pressure (PCWP) or left ventricular end-diastolic pressure (LVEDP) of ≤15 mmHg

  • Clinically stable and on stable doses of maximum tolerated (per investigator's judgment) double or triple background PAH therapies for at least 30 days prior to screening

  • Females of childbearing potential must:

    • Have 2 negative urine or serum pregnancy tests as verified by the investigator prior to starting study therapy; must agree to ongoing urine or serum pregnancy testing during the course of the study and until 8 weeks after the last dose of the study drug
    • If sexually active with a male partner, have used, and agree to use highly effective contraception without interruption per protocol; for at least 28 days prior to starting the investigational product, during the study (including dose interruptions), and for 16 weeks (112 days) after discontinuation of study treatment
    • Refrain from breastfeeding a child or donating blood, eggs, or ovum for the duration of the study and for at least 16 weeks (112 days) after the last dose of study treatment
  • Male participants must:

    • Agree to use a condom, defined as a male latex condom or nonlatex condom NOT made out of natural (animal) membrane (e.g., polyurethane), during sexual contact with a pregnant female or a female of childbearing potential while participating in the study, during dose interruptions, and for at least 16 weeks (112 days) following investigational product discontinuation, even if he has undergone a successful vasectomy
    • Refrain from donating blood or sperm for the duration of the study and for 16 weeks (112 days) after the last dose of study treatment
  • Ability to adhere to study visit schedule and understand and comply with all protocol requirements

  • Ability to understand and provide written informed consent

Exclusion Criteria
  • Diagnosis of pulmonary hypertension (PH) WHO Groups 2, 3, 4, or 5
  • Diagnosis of the following PAH Group 1 subtypes: human immunodeficiency virus-associated PAH and PAH associated with portal hypertension
  • Diagnosis of pulmonary veno-occlusive diseases or pulmonary capillary hemangiomatosis or overt signs of capillary and/or venous involvement
  • Hemoglobin at screening above gender-specific upper limit of normal (ULN), per local laboratory test
  • Baseline platelet count <50,000/mm3 (<50.0 x 109/L) at screening
  • Baseline systolic blood pressure <85 mmHg at screening
  • Pregnant or breastfeeding women
  • Serum alanine aminotransferase or aspartate aminotransferase levels or total bilirubin >3.0×ULN
  • Currently enrolled in or have completed any other investigational product study within 30 days for small molecule drugs or within 5 half-lives for biologics prior to the date of signed informed consent
  • Prior exposure to sotatercept or known allergic reaction to sotatercept, its excipients or luspatercept
  • History of pneumonectomy
  • Untreated more than mild obstructive sleep apnea
  • History of known pericardial constriction
  • History of restrictive or congestive cardiomyopathy
  • Electrocardiogram (ECG) with Fridericia's corrected QT interval (QTcF) >500 ms during the screening period
  • Personal or family history of long QT syndrome or sudden cardiac death
  • Left ventricular ejection fraction <45% on historical echocardiogram within 1 year prior to the screening visit
  • Any current or prior history of symptomatic coronary disease (prior myocardial infarction, percutaneous coronary intervention, coronary artery bypass graft surgery, or cardiac anginal chest pain) in the past 6 months prior to the screening visit
  • Cerebrovascular accident within 3 months prior to the screening visit
  • Significant (≥ 2+ regurgitation) mitral regurgitation or aortic regurgitation valvular disease
  • Currently on dialysis or anticipated need for dialysis within the next 12 months

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Placebo plus background PAH therapyPlaceboPlacebo administered subcutaneously (SC) every 21 days plus background PAH therapy
Sotatercept plus background PAH therapySotaterceptSotatercept at a starting dose of 0.3 mg/kg, with a target dose of 0.7 mg/kg, SC every 21 days plus background PAH therapy
Primary Outcome Measures
NameTimeMethod
Time to First Confirmed Morbidity or Mortality EventUp to approximately 31 months

Events are defined as all-cause death, lung transplantation, or PAH worsening-related hospitalization of ≥ 24 hours. All events will be adjudicated by a blinded, independent committee of clinical experts.

Secondary Outcome Measures
NameTimeMethod
Overall survival (OS)Up to approximately 31 months

OS is defined as the time from randomization to death due to any cause.

Transplant-Free SurvivalUp to approximately 31 months

Transplant-free survival is defined as the time from randomization to the first lung transplantation or death due to any cause.

Percentage of Participants Who Experienced a Mortality EventUp to approximately 31 months

Mortality event is defined as death due to any cause throughout the study.

Change From Baseline in REVEAL Lite 2 Risk Score at Week 24Baseline and Week 24

The REVEAL Lite 2 uses renal insufficiency (by estimated glomerular filtration rate (eGFR)), World Health Organization (WHO) functional class (FC), systolic blood pressure (SBP) and heart rate, 6-Minute Walk Distance (6-MWD), and N-terminal prohormone B-type natriuretic peptide (NT-proBNP) to determine the total risk score. The scores (range: 1-14) can be defined as: low risk as a score of ≤5, intermediate risk as a score of 6 or 7, and high risk as a score of ≥8 for the survival rates.

Percentage of Participants Achieving a Low or Intermediate (≤7) REVEAL Lite 2 Risk Score at Week 24Week 24

The REVEAL Lite 2 uses renal insufficiency (eGFR), WHO FC, SBP and heart rate, 6-MWD, and NT-proBNP to determine the total risk score. The scores (range: 1-14) can be defined as: low risk as a score of ≤5, intermediate risk as a score of 6 or 7, and high risk as a score of ≥8 for the survival rates.

Change From Baseline in NT-proBNP levels at Week 24Baseline and Week 24

Blood samples will be collected at baseline and at Week 24 to measure NT-proBNP levels.

Change From Baseline in Mean Pulmonary Artery Pressure (mPAP) at Week 24Baseline and Week 24

mPAP was measured by right heart catheterization (RHC) at baseline and at Week 24. mPAP is a hemodynamic parameter used to diagnose PAH.

Change From Baseline in Pulmonary Vascular Resistance (PVR)Baseline and Week 24

PVR is a hemodynamic variable measured by RHC at baseline and at Week 24.

Percentage of Participants Who Improve in WHO FCUp to approximately 31 months

The severity of an individual's PAH symptoms was graded using the WHO FC system. WHO functional classification for PAH range from Class I (no limitation in physical activity, no dyspnea with normal activity), Class II (slight limitation of physical activity), Class III (marked limitation of physical activity) and Class IV (cannot perform a physical activity without any symptoms, dyspnea at rest). The change from baseline in WHO FC is classified into "Improved", "No change" and "Worsened". Improvement = reduction in FC, worsened = increase in FC and no change = no change in FC.

Change From Baseline in 6-MWD at Week 24Baseline and Week 24

6-MWD is a measure of exercise capacity.

Change From Baseline in Cardiac Output (CO) at Week 24Baseline and Week 24

CO is the volume of blood pumped by the heart per minute.

Change From Baseline in EuroQoL-5 Dimensions Scale 5 Levels (EQ-5D-5L) Index Score at Week 24Baseline and Week 24

EQ-5D-5L measures health outcome. It consists of of descriptive system and EQ visual analogue scale (EQ-VAS). EQ-5D-5L system has 5 dimensions: mobility, self-care, usual activities, pain/discomfort and anxiety/depression. Each dimension is divided into 5 levels of perceived problems (Level 1-no problem, Level 2-slight problems, Level 3-moderate problems, Level 4-severe problems, Level 5-extreme problems). Participant selects answer for each of 5 dimensions considering response that best matches his/her health "today". Responses will be used to generate an index score.

Trial Locations

Locations (57)

Uniklinik Köln ( Site 1511)

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Koln, Nordrhein-Westfalen, Germany

Thoraxklinik-Heidelberg gGmbH ( Site 1509)

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Heidelberg, Baden-Wurttemberg, Germany

Hôpital Louis Pradel ( Site 1317)

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Bron, Rhone, France

Universitaetsklinikum Giessen und Marburg GmbH ( Site 1512)

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Giessen, Hessen, Germany

Medizinische Hochschule Hannover ( Site 1505)

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Hannover, Niedersachsen, Germany

Krankenhaus Neuwittelsbach ( Site 1510)

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München, Bayern, Germany

CHU de Nancy - Hôpital de Brabois Adultes ( Site 1308)

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Vandœuvre-lès-Nancy, Meurthe-et-Moselle, France

La Sapienza-Università di Roma-Policlinico Umberto I ( Site 2402)

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Roma, Italy

Universitätsklinikum Carl Gustav Carus an der TU Dresden. ( Site 1501)

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Dresden, Sachsen, Germany

University of Rochester Medical Center - PPDS ( Site 1039)

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Rochester, New York, United States

Lady Davis Carmel Medical Center ( Site 1705)

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Haifa, Israel

Statcare Pulmonary Consultants - Knoxville ( Site 1031)

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Knoxville, Tennessee, United States

St Vincent's Hospital Sydney ( Site 1102)

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Darlinghurst, New South Wales, Australia

University Of Texas Southwestern Medical Center ( Site 1038)

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Dallas, Texas, United States

University of California Irvine ( Site 1086)

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Orange, California, United States

University of New Mexico Health Sciences Center ( Site 1048)

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Albuquerque, New Mexico, United States

Instituto Nacional De Cardiologia Dr. Ignacio Chavez ( Site 2503)

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Ciudad de Mexico, Distrito Federal, Mexico

Royal Brompton Hospital ( Site 1206)

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London, London, City Of, United Kingdom

David Geffen School of Medicine at UCLA ( Site 1068)

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Los Angeles, California, United States

Mayo Clinic Jacksonville - PPDS ( Site 1045)

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Jacksonville, Florida, United States

Northside Hospital ( Site 1073)

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Atlanta, Georgia, United States

University Of Iowa Hospitals and Clinics ( Site 1050)

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Iowa City, Iowa, United States

Washington University School of Medicine ( Site 1022)

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Saint Louis, Missouri, United States

Medical University of South Carolina - PPDS ( Site 1003)

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Charleston, South Carolina, United States

John Hunter Hospital ( Site 1101)

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New Lambton Heights, New South Wales, Australia

Jewish General Hospital ( Site 2103)

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Montréal, Quebec, Canada

Hospital Universitario 12 de Octubre ( Site 1603)

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Madrid, Spain

University of California San Diego Medical Center ( Site 1002)

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San Diego, California, United States

University of Cincinnati Medical Center ( Site 1035)

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Cincinnati, Ohio, United States

The Cleveland Clinic Foundation. ( Site 1065)

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Cleveland, Ohio, United States

Duke University Medical Center ( Site 1026)

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Durham, North Carolina, United States

University of Kansas Medical Center ( Site 1020)

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Kansas City, Kansas, United States

Universitätsklinikum des Saarlandes ( Site 1513)

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Homburg, Saarland, Germany

Ospedale S. Giuseppe Multimedica ( Site 2403)

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Milan, Lombardia, Italy

Hôpital Erasme ( Site 1402)

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Anderlecht, Bruxelles-Capitale, Region De, Belgium

Centre Hospitalier Universitaire de Toulouse. ( Site 1315)

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Toulouse, Haute-Garonne, France

Brigham and Women's Hospital ( Site 1014)

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Boston, Massachusetts, United States

Hôpitaux Universitaires de Strasbourg ( Site 1307)

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Strasbourg, Bas-Rhin, France

Tufts Medical Center - PPDS ( Site 1012)

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Boston, Massachusetts, United States

UZ Leuven Campus Gasthuisberg ( Site 1401)

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Leuven, Vlaams-Brabant, Belgium

Hospital Universitario "Dr. Jose Eleuterio Gonzalez" ( Site 2504)

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Monterrey, Nuevo Leon, Mexico

VU Medisch Centrum ( Site 2601)

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Amsterdam, Noord-Holland, Netherlands

Peter Lougheed Centre ( Site 2102)

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Calgary, Alberta, Canada

CHU de Poitiers ( Site 1316)

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Poitiers, Vienne, France

Unidad de Investigación Clínica en Medicina, S.C ( Site 2505)

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Monterrey, Nuevo Leon, Mexico

The George Washington University Medical Faculty Associates ( Site 1025)

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Washington, District of Columbia, United States

Royal Papworth Hospital ( Site 1208)

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Cambridge, Cambridgeshire, United Kingdom

CHRU Lille ( Site 1306)

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Lille, Nord, France

CHU Bicêtre ( Site 1304)

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Le Kremlin-Bicêtre, Val-de-Marne, France

Imperial College Healthcare NHS Trust ( Site 1203)

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London, London, City Of, United Kingdom

Arizona Pulmonary Specialists ( Site 1010)

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Phoenix, Arizona, United States

University of California San Francisco ( Site 1019)

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San Francisco, California, United States

University of Nebraska Medical Center ( Site 1053)

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Omaha, Nebraska, United States

Medical College of Wisconsin - Froedtert Hospital ( Site 1051)

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Milwaukee, Wisconsin, United States

University of Colorado Hospital ( Site 1013)

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Aurora, Colorado, United States

AdventHealth Medical Group Advanced Lung Disease ( Site 1058)

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Orlando, Florida, United States

University of Michigan ( Site 1011)

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Ann Arbor, Michigan, United States

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