A Study of Pulmonary Arterial Hypertension Participants Treated With Macitentan or Selexipag

NCT04567602 | Completed

• 2 other identifiers: CR10885567896049PAH4009
• Study Type: observational
• Target: 372
• Locations: 1 country
• Sites: 30

Brief Summary

The purpose of the study is to evaluate the change from baseline to 12 months after study enrollment in the number of the following non-invasive risk criteria: World Health Organization Functional Class (WHO/FC), 6-minute walk distance (6MWD), Brain Natriuretic Peptide (BNP) or N-terminal pro-brain Natriuretic Peptide (NT-proBNP).

Conditions

Pulmonary Arterial Hypertension

Outcome Measures

Primary Outcomes (1)

• Change from Baseline in the Number of the Non-Invasive Low-Risk Criteria to 12 Months

  Change from baseline in the number of the non-invasive low-risk criteria will be assessed according to the following parameters: world health organization (WHO)/functional class (FC)- I, II, 6-minute walk distance (6MWD) greater than (\>) 440 meters (m), and brain natriuretic peptide (BNP) less than (\<) 50 nanogram per liter (ng/l) or N-terminal-pro-hormone brain natriuretic Peptide (NT-proBNP) \<300 ng/l.

  Baseline and 12 Months

Secondary Outcomes (14)

• Number of Participants with Improved, Stable or Worsened Risk profile from Baseline to 12 and 24 Months

  Baseline up to 12 and 24 Months

• Change in Progression in the Number of Participants with Low/High Intermediate Risk

  Baseline up to 24 Months

• Change from Baseline in 6MWD

  Baseline up to 24 Months

• Change from Baseline in BNP or NT-proBNP

  Baseline up to 24 Months

• Change from Baseline in Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) 2.0 Risk Score up to 12 and 24 Months

  Baseline up to 12 and 24 Months

Study Arms (1)

Participants with PAH

Participants with confirmed diagnosis of pulmonary arterial hypertension (PAH) will be enrolled in the study and the data will be collected and observed to describe the application of European ESC/ERS guidelines and related 6th WSPH proceedings on risk assessment and related treatment strategy, in clinical practice.

Drug: PAH medication

Interventions

PAH medication DRUG

There will be no specific interventions.

Participants with PAH

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Study population consist of participants with confirmed diagnosis of PAH.

You may qualify if:

• Participants in the clinical classification of pulmonary hypertension Group 1 (PAH)
• Participants with idiopathic, heritable and associated forms of PAH (connective tissue disease, congenital heart disease corrected at least 1-year ago, human immunodeficiency virus infection, drug use or toxin, and porto-pulmonary)
• Confirmed diagnosis of PAH with hemodynamic study right heart catheterization (RHC) at enrolment or at latest follow-up (not later than twelve months from enrolment date)
• Participants with a low or intermediate mortality risk profile, as per clinical judgement based on a multiparametric approach including clinical, functional exercise, right ventricular function and hemodynamic parameters
• Participants currently in treatment with selexipag and/or macitentan (in monotherapy or in combination therapy)

You may not qualify if:

• Participants in Group 1 that are responders to the vasoreactivity test
• Participants with pulmonary veno-occlusive disease (PVOD), defined on the basis of the following chest findings at high-resolution computed tomography (HRCT): centrilobular ground-glass opacities, smooth thickening of interlobular septa, mediastinal lymph node enlargement
• Participants already in treatment with subcutaneous/intravenous prostanoids. Iloprost interventional (IV) (treatment of digital ulcers in systemic sclerosis \[SSc\] participants according to european league against rheumatism \[EULAR\] guidelines) is allowed
• Participants currently enrolled in an interventional study

Sponsors & Collaborators

• Janssen-Cilag S.p.A.: lead

Study Sites (30)

Generale Regionale F. Miulli

Acquaviva delle Fonti, 70021, Italy

Location

A.O. Universitaria Ospedali Riuniti di Ancona

Ancona, 60126, Italy

Location

Azienda Ospedaliero Universitaria Consorziale Policlinico di Bari

Bari, 70124, Italy

Location

Ospedale Di Venere

Bari, 70131, Italy

Location

Ospedale di Bolzano

Bolzano, 39100, Italy

Location

ASST Spedali Civili Brescia

Brescia, 25123, Italy

Location

Azienda Ospedaliera G. Brotzu

Cagliari, 09134, Italy

Location

AO di Catanzaro Pugliese Ciaccio

Catanzaro, 88100, Italy

Location

ASL2 Lanciano - Vasto - Chieti - Ospedale 'SS Annunziata' di Chieti

Chieti, 66100, Italy

Location

AOU Careggi

Florence, 50134, Italy

Location

Azienda Ospedaliero Universitaria Ospedali Riuniti di Foggia

Foggia, 71122, Italy

Location

Ospedale Policlinico San Martino IRCCS

Genova, 16132, Italy

Location

Presidio Ospedaliero di Ivrea

Ivrea, 10015, Italy

Location

UOC Oncologia Ospedale Provinciale di Macerata

Macerata, 62100, Italy

Location

Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico

Milan, 20122, Italy

Location

IRCCS Ospedale San Raffaele

Milan, 20132, Italy

Location

Centro Cardiologico Monzino

Milan, 20138, Italy

Location

Ospedale Monaldi

Napoli, 80131, Italy

Location

Università del Piemonte Orientale - Ospedale Maggiore della Carità di Novara

Novara, 28100, Italy

Location

AOU di Padova

Padua, 35128, Italy

Location

ISMETT Istituto Mediterraneo Trapianti e Terapie ad Alta Specializzazione

Palermo, 90127, Italy

Location

Fondazione IRCCS Policlinico San Matteo

Pavia, 27100, Italy

Location

Fondazione Toscana Gabriele Monasterio CNR

Pisa, 56124, Italy

Location

AOU Policlinico Umberto I

Roma, 00161, Italy

Location

Policinico A Gemelli

Roma, 00168, Italy

Location

IRCCS Policlinico San Donato

San Donato Milanese, 20097, Italy

Location

Presidio SS Annunziata AOU Sassari

Sassari, 07100, Italy

Location

Azienda Ospedaliera Città della Salute e della Scienza di Torino

Torino, 10126, Italy

Location

ASUI Santa Maria della Misericordia di Udine

Udine, 33100, Italy

Location

Ospedale Borgo Roma

Verona, 37134, Italy

Location

MeSH Terms

Conditions

Pulmonary Arterial Hypertension

Condition Hierarchy (Ancestors)

Hypertension, Pulmonary; Lung Diseases; Respiratory Tract Diseases

Study Officials

• Janssen-Cilag S.p.A., Italy Clinical Trial

  Janssen-Cilag S.p.A.

  STUDY DIRECTOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: INDUSTRY
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: September 23, 2020
• First Posted: September 28, 2020
• Study Start: October 6, 2020
• Primary Completion: January 25, 2024
• Study Completion: March 5, 2024
• Last Updated: June 25, 2025

Record last verified: 2025-06

Locations

IT (30)