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

Completed
Conditions
Pulmonary Arterial Hypertension
Interventions
Registration Number
NCT04567602
Lead Sponsor
Janssen-Cilag S.p.A.
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).

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
186
Inclusion Criteria
  • 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)
Exclusion Criteria
  • 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

Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Arm && Interventions
GroupInterventionDescription
Participants with PAHPAH medicationParticipants 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.
Primary Outcome Measures
NameTimeMethod
Change from Baseline in the Number of the Non-Invasive Low-Risk Criteria to 12 MonthsBaseline and 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.

Secondary Outcome Measures
NameTimeMethod
Change in Progression in the Number of Participants with Low/High Intermediate RiskBaseline up to 24 Months

Change in risk profile for the low/high intermediate risk participants according to following cut-off values for low intermediate risk/high intermediate risk, respectively: (6MWD \[greater than or equal to \[\>=\] or \< 300m\], Peak oxygen consumption \[VO2\] \[\>= or \< 50% predicted\], BNP \[less than or equal to \[\<=\] or \> 175 ng/l\], or NT-proBNP \[\<= or \> 850 ng/l\], right atrial area \[RA\] \[\<= or \> 22 centimeter \[cm\]\] and right atrial pressure \[RAP\] \[\<= or \> 11 millimeter of mercury \[mmHg\]\] or stroke volume index (SVI) (\> or \<= 38 milliliter per meter square \[ml/m2\])). The low intermediate risk is defined by a presence of at least three low intermediate parameters and a high intermediate risk is defined by a presence of at least three high intermediate parameters.

Change from Baseline in Participants with Narrative plotsBaseline and 12 Months

Participants will be invited to fill in two narrative plots; one at the enrollment visit and one at the month 12 visit.

Change from Baseline in Caregivers with Narrative PlotsBaseline and 12 Months

Caregivers will be invited to fill in two narrative plots; one at the enrollment visit and one at the month 12 visit.

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

Risk profile is assessed as improved, stable or worsened where improved indicates 1 or more non-invasive parameters from intermediate to low risk profile; stable indicates no change in the parameters; and worsened indicates 1 or more non-invasive parameters move from low or intermediate to intermediate or high range risk.

Overall Survival (OS)Up to 24 Months

OS is defined as the duration/time from the start of study treatment to date of death due to any cause.

Change from Baseline in 6MWDBaseline up to 24 Months

Change from baseline in 6MWD will be reported.

Change from Baseline in BNP or NT-proBNPBaseline up to 24 Months

Change from baseline in BNP or NT-proBNP will be reported.

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

The REVEAL risk calculator determines the risk status and the scores can be defined as: low risk as a score of \<= 6, intermediate risk as a score of 7 or 8, and high risk as a score of \>= 9 for the survival rates.

Change from Baseline in Echocardiographic ParametersBaseline up to 24 Months

Change in echocardiographic parameters will be reported as assessed by echocardiogram.

Change from Baseline in Hemodynamic ParametersBaseline up to 24 Months

Change in hemodynamic parameters will be reported.

Hospitalization Rate due to Worsening of PAHUp to 24 Months

The hospitalization rate due to PAH worsening will be calculated based on the number of participants with at least one hospitalization. The calculation of the rate will be based on the number of events over the whole follow-up time that is exposure time.

Change from Baseline in Health-Related Quality of Life (HRQoL) as Measured with Emphasis-10 QuestionnaireBaseline up to 24 Months

The emPHasis-10 is a short and easy questionnaire that consists of 10 items which address breathlessness, fatigue, control and confidence. Each item is scored on a semantic differential six-point scale (0-5), with contrasting adjectives at each end. A total emPHasis-10 score is derived using simple aggregation of the 10 items. emPHasis-10 scores range from 0 to 50, higher scores indicate worse quality of life.

Adherence to European Society of Cardiology and European Respiratory Society (ESC/ERS) guidelines and 6th World Symposium on Pulmonary Hypertension (WSPH)Up to 24 Months

Adherence to ESC/ERS guidelines and 6th WSPH will be reported.

Number of Participants with Adverse Events (AEs) and Adverse Drug Reactions (ADRs)Up to 24 Months

An AE is any untoward medical occurrence in a participant enrolled in a clinical study that does not necessarily have a causal relationship with the pharmaceutical/biological agent under study. An ADR is defined as a response to a medicinal (investigational or non-investigational) product that is noxious and unintended.

Trial Locations

Locations (30)

Azienda Ospedaliera G. Brotzu

🇮🇹

Cagliari, Italy

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

🇮🇹

Chieti, Italy

AOU Careggi

🇮🇹

Firenze, Italy

ASUI Santa Maria della Misericordia di Udine

🇮🇹

Udine, Italy

ASST Spedali Civili Brescia

🇮🇹

Brescia, Italy

Centro Cardiologico Monzino

🇮🇹

Milano, Italy

Ospedale Monaldi

🇮🇹

Napoli, Italy

AOU di Padova

🇮🇹

Padova, Italy

Università del Piemonte Orientale - Ospedale Maggiore della Carità di Novara

🇮🇹

Novara, Italy

Fondazione IRCCS Policlinico San Matteo

🇮🇹

Pavia, Italy

Fondazione Toscana Gabriele Monasterio CNR

🇮🇹

Pisa, Italy

Policinico A Gemelli

🇮🇹

Roma, Italy

ISMETT Istituto Mediterraneo Trapianti e Terapie ad Alta Specializzazione

🇮🇹

Palermo, Italy

AOU Policlinico Umberto I

🇮🇹

Roma, Italy

Azienda Ospedaliera Città della Salute e della Scienza di Torino

🇮🇹

Torino, Italy

Ospedale Borgo Roma

🇮🇹

Verona, Italy

Azienda Ospedaliero Universitaria Consorziale Policlinico di Bari

🇮🇹

Bari, Italy

Generale Regionale F. Miulli

🇮🇹

Acquaviva Delle Fonti, Italy

A.O. Universitaria Ospedali Riuniti di Ancona

🇮🇹

Ancona, Italy

Ospedale Di Venere

🇮🇹

Bari, Italy

Ospedale di Bolzano

🇮🇹

Bolzano, Italy

AO di Catanzaro Pugliese Ciaccio

🇮🇹

Catanzaro, Italy

Azienda Ospedaliero Universitaria Ospedali Riuniti di Foggia

🇮🇹

Foggia, Italy

Ospedale Policlinico San Martino IRCCS

🇮🇹

Genova, Italy

Presidio Ospedaliero di Ivrea

🇮🇹

Ivrea, Italy

UOC Oncologia Ospedale Provinciale di Macerata

🇮🇹

Macerata, Italy

Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico

🇮🇹

Milano, Italy

IRCCS Ospedale San Raffaele

🇮🇹

Milano, Italy

IRCCS Policlinico San Donato

🇮🇹

San Donato Milanese, Italy

Presidio SS Annunziata AOU Sassari

🇮🇹

Sassari, Italy

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