A Study Providing Treatment Access in Participants With Pulmonary Hypertension Completing a Parent Study and Having no Other Option

NCT ID: NCT05179876

Last Updated: 2026-02-13

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

PHASE3

Total Enrollment

280 participants

Study Classification

INTERVENTIONAL

Study Start Date

2022-05-04

Study Completion Date

2029-09-28

Brief Summary

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The purpose of the study is to enable participants with pulmonary hypertension (PH) currently treated with study intervention(s) in a clinical study (parent studies \[NCT03422328, NCT03904693,NCT04565990, NCT02932410, NCT03492177, and NCT04175600\]), to continue to benefit from the intervention after closure of the parent study in case they have no alternative means of access to the study intervention. This study will allow assessment of the long-term safety of each study intervention.

Detailed Description

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Conditions

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Hypertension, Pulmonary

Study Design

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Allocation Method

NON_RANDOMIZED

Intervention Model

PARALLEL

This is a prospective, open-label, extension study, with a platform-study design for long-term safety follow-up of participants using study intervention as in their respective pulmonary hypertension (PH) parent study (that is, pulmonary arterial hypertension \[PAH\] or chronic thromboembolic pulmonary hypertension \[CTEPH\]). Participants who have completed a parent study, that benefit from their study intervention maintenance and have no adequate alternative local treatment option (access to the study intervention or an equivalent approved therapy) will be enrolled in this study.
Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Macitentan

Participants who have completed a parent study, benefit from their study intervention maintenance and have no adequate alternative local treatment option will be enrolled in this study and will continue to receive study drug macitentan orally during the course of the study. For adult participants study visits will be scheduled every 6 months and for pediatric participants study visits will be scheduled every 3 months. The study includes on-site visits to collect efficacy and safety information until participant discontinuation/withdrawal, or the respective study intervention is made commercially available in the country/territory or an equivalent approved therapy becomes available, or the sponsor decides to terminate the study prematurely.

Group Type EXPERIMENTAL

Macitentan

Intervention Type DRUG

Adult participants will receive oral dose of macitentan 10 milligrams (mg) tablet once daily. Children greater than or equal to (\>=) 2 year to less than (\<) 18 years will be given an oral macitentan dose tailored to their body weight, ensuring an equivalent level of systemic exposure as in adults.

Selexipag

Participants who have completed a parent study, benefit from their study intervention maintenance and have no adequate alternative local treatment option will be enrolled in this study and will continue to receive study drug selexipag orally during the course of the study. Study visits are scheduled every 6 months to collect efficacy and safety information until participant discontinuation/withdrawal, or the respective study intervention is made commercially available in the country/territory or an equivalent approved therapy becomes available, or the sponsor decides to terminate the study prematurely.

Group Type EXPERIMENTAL

Selexipag

Intervention Type DRUG

Adult Participant will receive oral dose of selexipag tablet twice daily at the dose strength corresponding to their maintenance dose at the end of their parent study. Available strengths: 200, 400, 600, 800, 1000, 1200, 1400 and 1600 micrograms (µg). Children with body weight category of \>=50 kg will use the tablets at the required dose strength as described for adults. Children with a body weight \< 50 kg will receive tablets for pediatric use (dose strengths: 100 and 150 mcg), twice daily to enable continuation of individually maximum tolerated dose of selexipag according to their body weight category.

Macitentan/Tadalafil FDC

Participants who have completed a parent study, benefit from their study intervention maintenance and have no adequate alternative local treatment option will be enrolled in this study and will continue to receive drug Macitentan and Tadalafil fixed dose combination (FDC) orally during the course of the study. Study visits are scheduled every 6 months to collect efficacy and safety information until participant discontinuation/withdrawal, or the respective study intervention is made commercially available in the country/territory or an equivalent approved therapy becomes available, or the sponsor decides to terminate the study prematurely.

Group Type EXPERIMENTAL

Macitentan/Tadalafil FDC

Intervention Type DRUG

Participants will receive oral FDC of macitentan 10 mg and tadalafil 40 mg once daily during the course of the study as already received in the parent studies.

Interventions

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Macitentan

Adult participants will receive oral dose of macitentan 10 milligrams (mg) tablet once daily. Children greater than or equal to (\>=) 2 year to less than (\<) 18 years will be given an oral macitentan dose tailored to their body weight, ensuring an equivalent level of systemic exposure as in adults.

Intervention Type DRUG

Selexipag

Adult Participant will receive oral dose of selexipag tablet twice daily at the dose strength corresponding to their maintenance dose at the end of their parent study. Available strengths: 200, 400, 600, 800, 1000, 1200, 1400 and 1600 micrograms (µg). Children with body weight category of \>=50 kg will use the tablets at the required dose strength as described for adults. Children with a body weight \< 50 kg will receive tablets for pediatric use (dose strengths: 100 and 150 mcg), twice daily to enable continuation of individually maximum tolerated dose of selexipag according to their body weight category.

Intervention Type DRUG

Macitentan/Tadalafil FDC

Participants will receive oral FDC of macitentan 10 mg and tadalafil 40 mg once daily during the course of the study as already received in the parent studies.

Intervention Type DRUG

Other Intervention Names

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JNJ-67896062 JNJ-67896049 JNJ-68150420

Eligibility Criteria

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Inclusion Criteria

* Participant must sign an informed consent form (ICF) (or their legally designated representative must sign) indicating that participant understands the purpose of, and procedures required for, the study and is willing to participate in the study
* Participant treated with oral macitentan or selexipag or fixed dose combination (FDC) of macitentan 10 milligrams (mg) and tadalafil 40 mg at the end of a sponsor parent study and: a) the indication of the parent study is included in the intervention-specific appendices (ISA) (pulmonary arterial hypertension \[PAH\]; b) participant has completed the parent study; c) no alternative means of access to study intervention (or equivalent approved therapy) have been identified; d) participant may continue to benefit from treatment with the study intervention; e) Participant is at least 18 years old for macitentan/tadalafil FDC, and at least 2 years old for macitentan or selexipag
* A female participant of childbearing potential must: a) have a negative urine or serum pregnancy test prior to first intake of study intervention; b) agree to perform monthly urine pregnancy test up to the end of the safety follow-up period; c) If heterosexually active, agree to follow contraceptive methods until 30 days after the last intake of the study intervention. For pediatric female participants: It is the responsibility of the investigator to ensure appropriate counselling, including consultation with a specialist (if needed), to the participant and/or parent(s)/ legally designated representative (LDR)(s) on the acceptable method of contraception

Exclusion Criteria

General:

* Participants prematurely discontinued from the study intervention in their parent study
* Female participant being pregnant, or breastfeeding, or planning to become pregnant while enrolled in this study
* Planned or current treatment with another investigational treatment

Macitentan-specific:

* Known allergies, hypersensitivity, or intolerance to macitentan or its excipients
* Hemoglobin less than (\<) 80 grams per liter (g/L)
* Serum aspartate (AST) and/or alanine aminotransferases (ALT) greater than (\>) 3\* upper limit of normal (ULN)
* Known and documented severe hepatic impairment that is, Child-Pugh Class C. For participants with hepatic impairment, Child-Pugh Class (Child-Pugh score) should be fully assessed and documented in the source documents at screening

Selexipag-specific:

* Known allergies, hypersensitivity, or intolerance to selexipag or its excipients
* Suspected or known pulmonary veno-occlusive disease (PVOD)
* Uncontrolled thyroid disease
* Severe coronary heart disease or unstable angina, myocardial infarction within the last 6 months, decompensated cardiac failure (if not under close medical supervision), severe arrhythmia, cerebrovascular events (for example, transient ischemic attack, stroke) within the last 3 months, or congenital or acquired valvular defects with clinically relevant myocardial function disorders not related to pulmonary hypertension (PH)
* Known and documented severe hepatic impairment that is, Child-Pugh Class C. For participants with hepatic impairment, Child-Pugh Class (Child-Pugh score) should be fully assessed and documented in the source documents at screening
* Children only: (a) Current suspicion of intussusception or ileus or gastrointestinal obstruction, per the investigator's judgment; (b) hemoglobin or hematocrit \<75 percent (%) of the lower limit of normal range

Macitentan/tadalafil FDC-specific:

* Known allergies, hypersensitivity, or intolerance to macitentan or tadalafil or their excipients
* Hemoglobin \<80 g/L
* Serum aspartate (AST) and/or alanine aminotransferases (ALT) \>3\* ULN range
* Known and documented severe hepatic impairment that is, Child-Pugh Class C. For participants with hepatic impairment, Child-Pugh Class should be fully assessed and documented in the source documents at screening
* Severe renal impairment (estimated glomerular filtration rate \[eGF\]/creatinine clearance \<30 milliliter per minute \[mL/min\])
Minimum Eligible Age

2 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Actelion

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Actelion Pharmaceuticals Ltd Clinical Trial

Role: STUDY_DIRECTOR

Actelion

Locations

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The Republican Scientific-Practical Center ''Cardiology''

Minsk, , Belarus

Site Status RECRUITING

Minsk Regional Clinical Hospital Of The Red Banner Of Labor

Minsk, , Belarus

Site Status RECRUITING

UZ Leuven

Leuven, , Belgium

Site Status COMPLETED

University Multiprofile Hospital for Active Treatment- UMHAT Sveta Anna AD

Sofia, , Bulgaria

Site Status RECRUITING

Beijing Anzhen Hospital

Beijing, , China

Site Status RECRUITING

The Second Xiangya Hospital of Central South Hospital

Changsha, , China

Site Status RECRUITING

Jiangsu Province Hospital

Nanjing, , China

Site Status RECRUITING

Qingdao Women and Children's Hospital

Qingdao, , China

Site Status RECRUITING

Childrens Hospital of Shanghai

Shanghai, , China

Site Status RECRUITING

The First Affiliated Hospital of Xian Jiaotong University

Xi'an, , China

Site Status RECRUITING

Gottsegen György Országos Kardiológiai Intézet

Budapest, , Hungary

Site Status RECRUITING

Klinika Kardiologii Z Oddzialem Intensywnego Nadzoru Kardiologicznego UM W Bialymstoku

Bialystok, , Poland

Site Status RECRUITING

Szpital Uniwersytecki nr 2 im dr Jana Biziela w Bydgoszczy, Klinika Kardiologii

Bydgoszcz, , Poland

Site Status COMPLETED

SPSK nr 7 SUM w Katowicach Gornoslaskie Centrum Medyczne im Prof Leszka Gieca

Katowice, , Poland

Site Status RECRUITING

Oddzial Kardiologii Wojewodzki Szpital Specjalistyczny im W Bieganskiego

Lodz, , Poland

Site Status RECRUITING

Wojewodzki Szpital Specjalistyczny im Stefana Kardynala Wyszynskiego SPZOZ

Lublin, , Poland

Site Status RECRUITING

SPSK2 PUM Klinika Kardiologii

Szczecin, , Poland

Site Status RECRUITING

Wojewodzki Szpital Specjalist Osrodek Badawczo Rozwojowy

Wroclaw, , Poland

Site Status RECRUITING

Wojewodzki Szpital Specjalistyczny we Wroclawiu

Wroclaw, , Poland

Site Status RECRUITING

Scientific and Research Institution of Cardiovascular Diseases Complex Problems

Kemerovo, , Russia

Site Status COMPLETED

E.Meshalkin National Medical Research Center of the Ministry of Health of the Russian Federation

Novosibirsk, , Russia

Site Status RECRUITING

Federal State Budgetary Institution

Saint Petersburg, , Russia

Site Status COMPLETED

Institute of Cardiology of Tomsk National Research Medical Center of Rus Academy of Sciences

Tomsk, , Russia

Site Status RECRUITING

Regional Clinical Hospital No1

Tyumen, , Russia

Site Status RECRUITING

Abdullah, IA

Durban, , South Africa

Site Status COMPLETED

Dr Kalla

Lenasia, , South Africa

Site Status RECRUITING

Chungnam National University Hospital

Daejeon, , South Korea

Site Status RECRUITING

Gachon University Gil Medical Center

Incheon, , South Korea

Site Status RECRUITING

Seoul National University Hospital

Seoul, , South Korea

Site Status RECRUITING

Asan Medical Center

Seoul, , South Korea

Site Status RECRUITING

Samsung Medical Center

Seoul, , South Korea

Site Status RECRUITING

The Catholic University of Korea Seoul St Marys Hospital

Seoul, , South Korea

Site Status RECRUITING

Kaohsiung Veterans General Hospital

Kaohsiung City, , Taiwan

Site Status RECRUITING

National Cheng Kung University Hospital

Tainan, , Taiwan

Site Status RECRUITING

National Taiwan University Hospital

Taipei, , Taiwan

Site Status RECRUITING

Taipei Veterans General Hospital

Taipei, , Taiwan

Site Status RECRUITING

Chang-Gung Memorial Hospital, LinKou Branch

Taoyuan District, , Taiwan

Site Status RECRUITING

Maharaj Nakorn Chiang Mai hospital Faculty of Medicine

Chiang Mai, , Thailand

Site Status RECRUITING

Municipal Inst. Of Dnipropetrovsk Region. Council

Dnipro, , Ukraine

Site Status RECRUITING

State Institute Of Phthisiology And Pulmonology N.A. F.G. Yanovskiy Of Ams Ukraine

Kyiv, , Ukraine

Site Status COMPLETED

Hanoi Heart Hospital

Hanoi, , Vietnam

Site Status RECRUITING

Hanoi Medical University Hospital

Hanoi, , Vietnam

Site Status RECRUITING

Children's Hospital 1

Ho Chi Minh City, , Vietnam

Site Status RECRUITING

Countries

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Belarus Belgium Bulgaria China Hungary Poland Russia South Africa South Korea Taiwan Thailand Ukraine Vietnam

Central Contacts

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Study Contact

Role: CONTACT

844-434-4210

Other Identifiers

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NOPRODPAPUH3001

Identifier Type: OTHER

Identifier Source: secondary_id

2021-002297-11

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

2023-506791-27-00

Identifier Type: REGISTRY

Identifier Source: secondary_id

CR109121

Identifier Type: -

Identifier Source: org_study_id

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