RecruitingPhase 3NCT05179876

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

A Prospective, Open-label, Platform Study for Long-term Follow-up of Participants Using Study Intervention in Pulmonary Hypertension Parent Studies


Sponsor

Actelion

Enrollment

280 participants

Start Date

May 4, 2022

Study Type

INTERVENTIONAL

Conditions

Summary

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.


Eligibility

Min Age: 2 Years

Inclusion Criteria3

  • 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 Criteria22

  • 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 \[mL/min\])

Interventions

DRUGMacitentan

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.

DRUGSelexipag

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.

DRUGMacitentan/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.


Locations(43)

The Republican Scientific-Practical Center ''Cardiology''

Minsk, Belarus

Minsk Regional Clinical Hospital Of The Red Banner Of Labor

Minsk, Belarus

UZ Leuven

Leuven, Belgium

University Multiprofile Hospital for Active Treatment- UMHAT Sveta Anna AD

Sofia, Bulgaria

Beijing Anzhen Hospital

Beijing, China

The Second Xiangya Hospital of Central South Hospital

Changsha, China

Jiangsu Province Hospital

Nanjing, China

Qingdao Women and Children's Hospital

Qingdao, China

Childrens Hospital of Shanghai

Shanghai, China

The First Affiliated Hospital of Xian Jiaotong University

Xi'an, China

Gottsegen György Országos Kardiológiai Intézet

Budapest, Hungary

Klinika Kardiologii Z Oddzialem Intensywnego Nadzoru Kardiologicznego UM W Bialymstoku

Bialystok, Poland

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

Bydgoszcz, Poland

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

Katowice, Poland

Oddzial Kardiologii Wojewodzki Szpital Specjalistyczny im W Bieganskiego

Lodz, Poland

Wojewodzki Szpital Specjalistyczny im Stefana Kardynala Wyszynskiego SPZOZ

Lublin, Poland

SPSK2 PUM Klinika Kardiologii

Szczecin, Poland

Wojewodzki Szpital Specjalist Osrodek Badawczo Rozwojowy

Wroclaw, Poland

Wojewodzki Szpital Specjalistyczny we Wroclawiu

Wroclaw, Poland

Scientific and Research Institution of Cardiovascular Diseases Complex Problems

Kemerovo, Russia

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

Novosibirsk, Russia

Federal State Budgetary Institution

Saint Petersburg, Russia

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

Tomsk, Russia

Regional Clinical Hospital No1

Tyumen, Russia

Abdullah, IA

Durban, South Africa

Dr Kalla

Lenasia, South Africa

Chungnam National University Hospital

Daejeon, South Korea

Gachon University Gil Medical Center

Incheon, South Korea

Seoul National University Hospital

Seoul, South Korea

Asan Medical Center

Seoul, South Korea

Samsung Medical Center

Seoul, South Korea

The Catholic University of Korea Seoul St Marys Hospital

Seoul, South Korea

Kaohsiung Veterans General Hospital

Kaohsiung City, Taiwan

National Cheng Kung University Hospital

Tainan, Taiwan

National Taiwan University Hospital

Taipei, Taiwan

Taipei Veterans General Hospital

Taipei, Taiwan

Chang-Gung Memorial Hospital, LinKou Branch

Taoyuan District, Taiwan

Maharaj Nakorn Chiang Mai hospital Faculty of Medicine

Chiang Mai, Thailand

Municipal Inst. Of Dnipropetrovsk Region. Council

Dnipro, Ukraine

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

Kyiv, Ukraine

Hanoi Heart Hospital

Hanoi, Vietnam

Hanoi Medical University Hospital

Hanoi, Vietnam

Children's Hospital 1

Ho Chi Minh City, Vietnam

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NCT05179876


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