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Long-Term, Open Label Extension Study of Pemziviptadil (PB1046) in PAH Subjects Following Completion of Study PB1046-PT-CL-0004

Phase 2
Terminated
Conditions
Pulmonary Arterial Hypertension
Interventions
Drug: Pemziviptadil (PB1046) Injection
Registration Number
NCT03795428
Lead Sponsor
PhaseBio Pharmaceuticals Inc.
Brief Summary

This is a multi-center, Phase 2 Long-Term, Open Label Extension (OLE) Study to assess the safety and tolerability of pemziviptadil (PB1046) at an optimally titrated dose. This is a Long-Term, Open label Extension (OLE) Study for subjects with (PAH), having participated in double-blind Study PB1046-PT-CL-0004. The study will include adult subjects previously diagnosed with symptomatic PAH, who are receiving background clinician-directed therapy for PAH.

During this period, subjects will continue to be followed for safety and tolerability, as well as for periodic efficacy, quality of life data and immunogenicity. The study will continue per the schedule of events until such time when pemziviptadil (PB1046) is able to be self-administered, becomes commercially available to the subjects in a particular country or region, or the sponsor terminates the study due to lack of efficacy, safety or other reasons.

Detailed Description

Subjects entering this study will enter from the double-blind Study PB1046-PT-CL-0004. The starting dose level of pemziviptadil (PB1046) for all subjects in this parent study was a sub-therapeutic or minimally effective dose (MED) of 0.2 mg/kg, administered by SC injection.

Subjects were randomized into the MED) Group or a dose-titration group. In the dose-titration group, individual subjects were titrated up to their maximum tolerated dose (MTD) in a blinded fashion, with the objective of titrating subjects up to a dose of at least 1.2 mg/kg or higher in the MTD Group, while subjects in the MED Group remained at the MED level of 0.2 mg/kg, and underwent "sham dose-titration" to maintain the blind.

Subjects entering the 0006 trial prior to implementation of this protocol amendment will remain blinded until such time that open label dosing will not unblind the 0004 study.

Recruitment & Eligibility

Status
TERMINATED
Sex
All
Target Recruitment
25
Inclusion Criteria
  • Subjects must have completed Week 17 / End of Study of PB1046-PT-CL-0004;
  • Willing and able to sign a written Informed Consent (IC) prior to all study-related procedures;
  • Agrees to use a medically acceptable method of contraception (both male and female patients) throughout the entire study period and continuing for 30 days after their last dose of study drug. if the possibility of conception exists. Medically acceptable methods of contraception include the following: abstinence (not having sex), vasectomy (with confirmed negative sperm counts), condoms and partner using vaginal spermicide and/or cervical cap with spermicide or sponge; oral, implantable, or injectable contraceptives (starting ˃2 months before dosing), diaphragm with vaginal spermicide, intrauterine device, surgical sterilization (˃6 months after surgery). Female subjects ˂45 years of age of non-childbearing potential are defined as being surgically sterile by bilateral tubal ligation, bilateral oophorectomy, or hysterectomy. Female subjects 45to-60 years of age, inclusive, who are post-menopausal for at least 1 year, and have a follicle-stimulating hormone (FSH) level confirmation indicating post-menopausal status, will be considered to be of non-childbearing potential. Female subjects > 60 years of age are considered post-menopausal and of non-childbearing potential;
  • Willing and able to understand and follow instructions, return to the study unit for specified study visits; and, be able to participate in the study through the Stable Dose Maintenance Period, at a minimum.
Exclusion Criteria
  • Concomitant medical disorder, condition, or history, that in the opinion of the Investigator, would impair the subject's ability to participate in or complete the requirements of the study;
  • Pregnant or lactating female subjects;
  • Significant liver dysfunction as measured by any one of the following during participation in PB1046-PT-CL-0004. (If exclusionary laboratory results become available after the subject has enrolled in PB1046-PT-CL-0006 they should be discontinued. a. alanine aminotransferase (ALT) > 3.0 times upper limit of normal (ULN) or; b. aspartate aminotransferase (AST) > 3.0 times ULN or; c. serum bilirubin ≥ 1.6 mg/dL.
  • Recent history of substance abuse that, in the opinion of the Investigator, would impair the subject's ability to participate in or complete the requirements of the study;
  • In the opinion of the principal investigator (PI), any major surgical procedure within 90 days, or a planned surgical procedure during the study period; which would impact participation in PB1046-PT-CL-0006.
  • Other new medical or psychiatric conditions which, in the opinion of the Investigator, would place the subject at increased risk, would preclude obtaining voluntary consent, or would confound the objectives of the study;
  • Known hypersensitivity to study drug or any of the excipients of the drug formulation.

Study & Design

Study Type
INTERVENTIONAL
Study Design
SEQUENTIAL
Arm && Interventions
GroupInterventionDescription
Pemziviptadil (PB1046) Injection-OL Active Drug-Up-Titration to Stable DosePemziviptadil (PB1046) InjectionPemziviptadil (PB1046) Injection: Regardless of dose assignment, all subjects will be up-titrated in 0.2 mg/kg weekly increments, beginning with 0.4 mg/kg at Week 1, to the target dose of 1.2 mg/kg or higher depending on safety and tolerability.
Primary Outcome Measures
NameTimeMethod
Change in Oral Body Temperature from baselineDuration of extension study - Starting the day of first dose and completing 28 days after last dose.
Change in Heart Rate from baselineDuration of extension study - Starting the day of first dose and completing 28 days after last dose.
12-Lead ECG - Incidence of clinically significant abnormal ECG findings as measured by 12 Lead ECGDuration of extension study - Starting the day of first dose and completing 28 days after last dose.
Incidence of Clinical Laboratory AbnormalitiesDuration of extension study - Starting the day of first dose and completing 28 days after last dose.
Change in Diastolic Blood Pressure from baselineDuration of extension study - Starting the day of first dose and completing 28 days after last dose.
Incidence and Severity of Adverse EventsDuration of extension study - Starting the day of first dose and completing 28 days after last dose.
Change in Respiratory Rate from baselineDuration of extension study - Starting the day of first dose and completing 28 days after last dose.
Change in Systolic Blood Pressure from baselineDuration of extension study - Starting the day of first dose and completing 28 days after last dose.
Incidence of ImmunogenicityDuration of extension study - Starting up to 30 days prior to first dose of study drug in original study (PB1046-PT-CL-0004/0005) and completing 28 days after last dose.

Incidence of positive immunogenicity results after receipt of study drug

Secondary Outcome Measures
NameTimeMethod
Change from baseline in emPHasis-10 (Health Related Quality of Life) scoreDuration of extension study - Starting the day of first dose and completing 28 days after last dose.

Scores, which assess breathlessness, fatigue, control and confidence, range from 0 to 50, higher scores indicate worse quality of life.

Incidence of Clinical WorseningDuration of extension study - Starting the day of first dose and completing 28 days after last dose.

As defined by any one of the following: 1. All cause mortality; 2. Hospitalization due to worsening PAH; 3. Initiation of parenteral prostacyclin; 4. Any three of the following: 15% decrease in 6MWD, Functional class III or IV symptoms, Addition of PAH therapy, Worsening right heart failure

Change from baseline in NYHA/WHO Functional Class (FC)Duration of extension study - Starting the day of first dose and completing 28 days after last dose.
SurvivalDuration of extension study - Starting the day of first dose and completing 28 days after last dose.
Change in REVEAL Registry Risk Calculator ScoreDuration of extension study - Starting the day of first dose and completing 28 days after last dose.

Risk scores range from 0 (Lowest risk) to 22 (Highest risk) in PAH subjects

Change from baseline in NT-proBNPDuration of extension study - Starting the day of first dose and completing 28 days after last dose.
Change from baseline in 6MWD (6 minute walk distance test)Duration of extension study - Starting the day of first dose and completing 28 days after last dose.

Measured in meters walked in 6 minutes.

Change from baseline in Borg Dyspnea Index (BDI)Duration of extension study - Starting the day of first dose and completing 28 days after last dose.

BDI scale as measured from 0 to 10 (0 being no breathlessness and 10 being maximal breathlessness)

Trial Locations

Locations (16)

The Lindner Center for Research and Education at The Christ Hospital

🇺🇸

Cincinnati, Ohio, United States

NYU Langone Health

🇺🇸

New York, New York, United States

University of Rochester Medical Center

🇺🇸

Rochester, New York, United States

Emory University, The Emory Clinic

🇺🇸

Atlanta, Georgia, United States

University of Iowa Hospitals and Clinics

🇺🇸

Iowa City, Iowa, United States

University of Texas Southwestern Medical Center

🇺🇸

Dallas, Texas, United States

Allegheny General Hospital

🇺🇸

Pittsburgh, Pennsylvania, United States

UPMC Presbyterian

🇺🇸

Pittsburgh, Pennsylvania, United States

University of Miami - Pulmonary Research Center

🇺🇸

Miami, Florida, United States

University of Cincinnati

🇺🇸

Cincinnati, Ohio, United States

Tufts Medical Center

🇺🇸

Boston, Massachusetts, United States

Brigham and Women's Hospital

🇺🇸

Boston, Massachusetts, United States

University of California San Diego

🇺🇸

La Jolla, California, United States

University of California - Davis

🇺🇸

Sacramento, California, United States

INTEGRIS Baptist Medical Center

🇺🇸

Oklahoma City, Oklahoma, United States

University of Kansas Medical Center

🇺🇸

Kansas City, Kansas, United States

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