MedPath

Long-term Safety and Efficacy of Ralinepag in Pulmonary Arterial Hypertension

Phase 2
Completed
Conditions
Pulmonary Arterial Hypertension
Interventions
Registration Number
NCT02279745
Lead Sponsor
United Therapeutics
Brief Summary

This study was an open-label extension study to determine the long-term safety and tolerability of ralinepag in subjects with World Health Organization (WHO) Group 1 pulmonary arterial hypertension (PAH) who have completed Study APD811-003, or who were assigned to receive placebo and were discontinued due to clinical worsening.

Detailed Description

This study was an open-label extension study to determine the long-term safety and tolerability of ralinepag in subjects with WHO Group 1 PAH who completed Study APD811-003. Subjects who completed Study APD811-003 and met eligibility criteria for Study APD811-007 were enrolled. Additionally, placebo-treated subjects who discontinued study drug treatment due to clinical worsening in Study APD811-003 were permitted to enroll in Study APD811-007, upon approval of the medical monitor, provided that all end of study procedures including right heart catheterization (RHC) were performed per the study protocol. The Week 25 Visit in Study APD811-003 served as the Baseline Visit for Study APD811-007.

All subjects enrolled in Study APD811-007 received open-label treatment with ralinepag. The starting dose and titration schedule were individually determined and in accordance with the starting dose and titration schedule optimized from Study APD811-003. Adjustments in the dose and titration schedule were made according to subject tolerability.

After an individual subject completed Study APD811-003 and that subject's database was locked, subject unblinding occurred. Subjects on active treatment (ralinepag) remained on their current dose and had onsite clinical assessments performed every 3 months until the subject was discontinued from the study.

Subjects in the placebo treatment group underwent a dose titration period until a stable, maximum tolerated dose (MTD) was reached (up to 9 weeks), followed by a treatment period after the MTD was determined during which monthly onsite clinic assessments were performed for the first 3 months and then every 3 months until the subject was discontinued from the study or the study was terminated. Dose reductions could be made at any time for safety reasons. Incremental dose increases were also allowed during the Treatment Period at the discretion of the Investigator (as clinically indicated) and according to the stepwise titration scheme.

Subjects were assessed for clinical worsening during each clinic visit. If clinical worsening was confirmed, the Investigator could have opted to either continue treatment with ralinepag at the current dose, increase the dose of ralinepag, interrupt treatment, or discontinue the subject at his/her discretion.

In addition, attempts were made to contact all subjects at the time of Study APD811-007 termination to assess their vital (mortality) status. After the last subject enrolled in Study APD811-007 completed approximately 6 months of the study, a cumulative all-subject data analysis was performed for all subjects who entered the study. Subjects continued to have visits to the clinic every 3 months until the Sponsor discontinued the study. At the time of the Sponsor's decision to discontinue the study, all ongoing subjects completed an End of Study Visit. A 28-day Follow-up Visit was conducted to ensure appropriate subject safety. Subjects who remained on ralinepag were eligible to transition into the Phase 3 open-label extension study (ROR-PH-303) prior to APD811-007 study termination. For those subjects that did not enroll in Study ROR-PH-303, a 28-day Follow-up Visit was conducted to evaluate ongoing subject safety, including survival status.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
45
Inclusion Criteria
  • Evidence of a personally signed and dated informed consent document.
  • Was willing and able to comply with scheduled visits, treatment plan, laboratory tests, and other study procedures and was deemed an appropriate candidate for participation in a long-term extension study.
  • Female subjects were nonpregnant, nonlactating, surgically sterile or postmenopausal, or agreed to use an accepted method of birth control for at least 3 months prior to the first dose, during, and for at least 30 days after the last dose of study drug.
  • Male subjects were either surgically sterile or agreed to use a condom with spermicide when sexually active with a female partner who was not using an acceptable method of birth control during the study and for 30 days after the last dose of study drug.
  • Male and female subjects agreed not to participate in a conception process during the study and for 30 days after the last dose of study drug.
  • Fulfilled all eligibility criteria for Study APD811-003 and completed the study as planned.

Subjects who were assigned to placebo in Study APD811-003 and experienced clinical worsening in that study could enroll in Study APD811-007 after completing all end of study procedures per protocol, including RHC, for Study APD811-003 and had their data locked.

Read More
Exclusion Criteria
  • Subjects who enrolled in Study APD811-003 and were withdrawn from study drug treatment due to any adverse event (AE), serious adverse event (SAE), or subjects who did not complete Study APD811003, with the exception made for placebo-treated subjects who experienced a clinical worsening event.
  • Female •subjects who wished to become pregnant.
  • Systolic blood pressure <90 mmHg at Baseline.
  • Other severe acute or chronic medical or laboratory abnormalities that could have increased the risk associated with study participation or investigational product administration or interfered with the interpretation of study results and, in the judgment of the investigator, would have made the subject inappropriate for entry into this study.
Read More

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
Oral RalinepagRalinepagRalinepag immediate-release (IR) capsules of 10, 20, 30, 40, and 100 mcg or extended-release (XR) tablets of 50, 250, and 400 mcg for oral administration.
Primary Outcome Measures
NameTimeMethod
Change From Baseline in Pulmonary Vascular ResistanceAt 1 or 2 years after the subject enrolled into the study, pending their last RHC prior to Protocol Amendment 2.

Pulmonary vascular resistance was collected by right heart catheterization (RHC).

Change From Baseline in Cardiac OutputAt 1 or 2 years after the subject enrolled into the study, pending their last RHC prior to Protocol Amendment 2.

Cardiac output was collected by right heart catheterization (RHC).

Change From Baseline in Mean Pulmonary Arterial PressureAt 1 or 2 years after the subject enrolled into the study, pending their last RHC prior to Protocol Amendment 2.

Mean pulmonary arterial pressure was collected by right heart catheterization (RHC).

Change From Baseline in Cardiac IndexAt 1 or 2 years after the subject enrolled into the study, pending their last RHC prior to Protocol Amendment 2.

Cardiac index was collected by right heart catheterization (RHC).

Secondary Outcome Measures
NameTimeMethod
Time From Randomization to the First Protocol-defined Clinical Worsening EventFrom Baseline to 28 days following discontinuation of study drug, up to 235 weeks.

Clinical worsening events were defined as death, or onset of a treatment-emergent adverse event (AE) with a fatal outcome occurring ≤14 days after treatment discontinuation; hospitalization for worsening PAH, heart-lung or lung transplant, or atrial septostomy; necessity of addition (or dose change) of any prostacyclin/prostacyclin analogue, phosphodiesterase type 5 inhibitor (PDE5-I) or soluble guanylate cyclase (sGC), or endothelin receptor antagonist (ERA); and the combined occurrence of a decrease in 6-Minute Walk Distance (6MWD) by at least 20% from Baseline, confirmed on two 6-Minute Walk Tests (6MWTs) on different days; worsening in WHO/New York Heart Association (NYHA) Functional Class (FC) from Baseline; and appearance of or worsening of signs/symptoms of right heart failure that did not respond to optimized oral diuretic therapy.

Change From Baseline in 6MWDFrom Baseline to discontinuation of study drug, up to 235 weeks

6MWD was measured at Baseline (prior to starting study drug) and every 3 months thereafter including the End of Study Visit.

Change From Baseline in WHO/NYHA FCFrom Baseline to 28 days following discontinuation of study drug, up to 235 weeks

WHO/NYHA FC was measured at Baseline (prior to starting study drug) and every 3 months thereafter including at the End of Study and 28-Day Follow-up Visits. FC recorded as I, II, III, or IV based on the following:

I: PH but without limitation of physical activity; physical activity without undue dyspnea or fatigue, chest pain or near syncope.

II: PH with slight limitation of physical activity; physical activity causes undue dyspnea or fatigue, chest pain or near syncope.

III: PH with marked limitation of physical activity; less than ordinary activity causes undue dyspnea or fatigue, chest pain or near syncope.

IV: PH with inability to carry out any physical activity without symptoms. Signs of right heart failure. Dyspnea and/or fatigue at rest. Discomfort is increased by any physical activity.

Trial Locations

Locations (46)

David Geffen School of Medicine at UCLA

🇺🇸

Los Angeles, California, United States

St Vincent's Hospital

🇦🇺

Fitzroy, Victoria, Australia

Multiprofile Hospital for Active Treatment "National Heart Hospital" EAD, Clinic of Cardiology

🇧🇬

Sofia, Bulgaria

University of Michigan Health System

🇺🇸

Ann Arbor, Michigan, United States

University of Pecs, Medical School, Heart Institute - Pécsi Tudományegyetem, Klinikai Központ, Szívgyógyászati Klinika

🇭🇺

Budapest, Hungary

John Paul II Hospital in Cracov Department of Cardiac and Vascular Diseases - Krakowski Szpital Specjalistyczny im. Jana Pawła II, Oddział Kliniczny Chorób Serca i Naczyń

🇵🇱

Krakow, Poland

"Prof. Dr. C.C. Iliescu" Institute of Cardiovascular Diseases, Department of Clinic Cardiology III - Institutul de Urgenţă pentru Boli Cardiovasculare "Prof. Dr. C.C. Iliescu", Secţia Clinica Cardiologie fIJ

🇷🇴

Bucharest, Romania

UC Health

🇺🇸

Cincinnati, Ohio, United States

Memorial Hermann Hospital - Texas Medical Center

🇺🇸

Houston, Texas, United States

Cedars-Sinai Medical Center

🇺🇸

Beverly Hills, California, United States

University of Alabama at Birmingham

🇺🇸

Birmingham, Alabama, United States

Harbor-UCLA Medical Center

🇺🇸

Torrance, California, United States

University of Colorado Cardiac and Vascular Center, Anschutz Inpatient Pavilion

🇺🇸

Aurora, Colorado, United States

University of California Davis Medical Center

🇺🇸

Sacramento, California, United States

Cleveland Clinic Florida

🇺🇸

Weston, Florida, United States

Chest Medicine Associates

🇺🇸

Portland, Maine, United States

University of Iowa Hospitals and Clinics

🇺🇸

Iowa City, Iowa, United States

University of Maryland Medical Center

🇺🇸

Baltimore, Maryland, United States

Boston University Medical Center General Clinical Research Unit (GCRU)

🇺🇸

Boston, Massachusetts, United States

The Ohio State University Wexner Medical Center - Martha Morehouse Medical Pavilion

🇺🇸

Columbus, Ohio, United States

University Hospitals Case Medical Center

🇺🇸

Cleveland, Ohio, United States

UPMC, Presbyterian

🇺🇸

Pittsburgh, Pennsylvania, United States

UT Southwestern Medical Center

🇺🇸

Dallas, Texas, United States

St. Vincent's Hospital

🇦🇺

Darlinghurst, New South Wales, Australia

The Prince Charles Hospital

🇦🇺

Chermside, Queensland, Australia

Royal Hobart Hospital

🇦🇺

Hobart, Tasmania, Australia

Fiona Stanley Hospital

🇦🇺

Murdoch, Australia

Department of Internal Medicine I - Cardiology, University Hospital Olomouc

🇨🇿

Olomouc, Czechia

Multiprofile Hospital for Active Treatment " St. Anna", Sofia AD, Cardiology Clinic

🇧🇬

Sofia, Bulgaria

Second Internal Clinic - Clinic of Cardiology and Angiology, 1st Faculty of Medicine, Charles University in Prague, General University Hospital in Prague

🇨🇿

Prague, Czechia

Gottsegen Gyorgy Orszagos Kardiológiai lntézet - National Institute of Cardiology, Department of Adult Cardiology

🇭🇺

Budapest, Hungary

Semmelweis University, Department of Pulmonology - Semmelweis Egyetem Pulmonológiai Klinika

🇭🇺

Budapest, Hungary

University of Szeged Faculty of Medicine, 2nd Department of Medicine and Cardiology Center, Albert Szent-Györyi Clinical Center - SZTE ÁOK Szent-Györgyi A lbert Klinikai Központ I I. sz. Belgyógyászati Klini ka és Kard ilógiai Központ

🇭🇺

Budapest, Hungary

University of Derecen Clinical Research Center Cardiology and Cardiac Surgery Department - Debreceni Egyetem Klinikai Kozpont Kardiologiai es Szivsebeszeti Klinika

🇭🇺

Debrecen, Hungary

Biegański Provincial Specialist Hospital Department of Cardiology - Wojewódzki Szpital Specjalistyczny im. dr Wł. Biegańskiego w Lodzi, Oddział Kardiologiczny

🇵🇱

Lodz, Poland

Medical University of Bialystok Clinical Hospital Cardiology Clinic - Uniwersytecki Szpital Kliniczny, Klinika Kardiologii z Oddziałem Intensywnego Nadzoru Kardiologicznego

🇵🇱

Kraków, Poland

"Marius Nasta" Institute of Pneumoftiziology, Department of Pneumoftiziology IV - Institutul de Pneumoftiziologie "Marius Nasta", Sectia Clinica Pneumoftiziologie IV

🇷🇴

Bucharest, Romania

Institut za plucne bolesti Vojvodine Sremska Kamenica, Klinika za urgcntnu pulmologiju, Odeljcnje intenzivne nege - Institute of Pulmonary Diseases of Vojvodina Sremska Kamenica (IPDVSK), The Clinic for Urgent Pulmonology, ICU - Intensive Care Unit

🇷🇸

Sremska Kamenica, Serbia

Clinical Centre of Serbia (CCS), Cardiology Clinic - Klinicki Centar SrЬije, Klinika za kardiologiju

🇷🇸

Belgrade, Serbia

Кlinicko-bolnicki Centar Zemun, Кlinika za internu medicinu, Sluzba za kardiologiju

🇷🇸

Belgrade, Serbia

"Dr. Victor Babes" Clinic Hospital for Infesctious Diseases and Pneumoftiziology, Department of Clinic Pneumology II

🇷🇴

Timisoara, Romania

Cardiology department, East Slovak Institute for Cardiovascular Diseases - Kardiologické oddelenie Klinika kardiológie , Východoslovenský ústav srdcových a cievnych chorôb, a.s

🇸🇰

Košice, Slovakia

Department of Heart Failure and Transplantation, National Institute of Cardiovascular Diseases - Oddelenie zlyhávania a transplantácie srdca, Národný ústav srdcových a cievnych chorôb, a.s.

🇸🇰

Bratislava, Slovakia

Hospital 12th of October, Department of Cardiology

🇪🇸

Madrid, Spain

Clinic Hospital of Barcelona, Department of Pneumology

🇪🇸

Barcelona, Spain

General University Hospital Vall d'Hebron, Department of Pneumology

🇪🇸

Barcelona, Spain

© Copyright 2025. All Rights Reserved by MedPath