Real-world observational study on pulmonary arterial hypertension: Italian cohort treated with macitentan and/or selexipag as a part of a combination treatment (INSPECTIO)
Year: 2026
Type: article
Study design: Observational Study
Abstract: BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive disease associated with significant morbidity and mortality. Combination therapy targeting multiple pathways has been shown to improve clinical outcomes. METHODS: The INSPECTIO study was a prospective, multicenter, observational study conducted across 29 Italian centers specializing in PAH management. The study sought to explore the impact of combination therapy on disease progression and quality of life, by assessing non-invasive risk parameters, echocardiographic and hemodynamic conditions, and patient-reported outcomes (PROs). Patients with PAH at low or intermediate mortality risk, treated with macitentan and/or selexipag (as monotherapy or in combination), were enrolled. Data on non-invasive low-risk criteria (WHO Functional Class, 6-min walk distance [6MWD], and NT-proBNP), REVEAL risk scores, echocardiographic and hemodynamic parameters, and patient-reported outcomes (emPHasis-10) were collected at baseline and 12 months. A Narrative Medicine analysis complemented quantitative findings by exploring patients' experiences. RESULTS: Among 176 patients with follow-up data, the number of non-invasive low-risk criteria increased by an average of 0.15 at 12 months (p = 0.0167). REVEAL 2.0 and REVEAL Lite 2 scores showed significant reductions (mean changes: -1.0, p = 0.0142; -0.8, p = 0.0111, respectively). Modest changes were observed in echocardiographic and hemodynamic parameters. Narrative analysis highlighted strengthened patient-provider relationships and improved coping strategies. Safety outcomes aligned with known profiles. CONCLUSIONS: The INSPECTIO study supports the effectiveness of guideline-directed therapy and regular risk assessment to optimize treatment strategies in PAH. The increase in non-invasive low-risk criteria suggests a stabilization of disease over 12 months.
Source: Vascular Pharmacology
Authors: Michele D'Alto, Laura Scelsi, Livio Giuliani, Gian Piero Perna, Fabiana Baldi, Federico Guerra, Emma Di Poi, Marco Vicenzi, Roberto Badagliacca, Marco Corda, Edoardo Airò, Paolo Ferrero, Pietro Ameri, Francesca Bux, Piergiuseppe Agostoni, Carlo D'Agostino, Gavino Casu, Matteo Biancospino, Alessia Uglietti, Stefano De Santis
Institutions: Policlinico San Matteo Fondazione, Azienda Ospedaliero-Universitaria delle Marche, Università Cattolica del Sacro Cuore, Agostino Gemelli University Polyclinic, Marche Polytechnic University, University of Udine, Ospedale Santa Maria della Misericordia di Udine, University of Milan, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Policlinico Umberto I, Sapienza University of Rome, Azienda Ospedaliera G. Brotzu, National Research Council, Fondazione Toscana Gabriele Monasterio, University of Verona, Ospedale Policlinico San Martino, University of Genoa, Centro Cardiologico Monzino, Azienda Universitaria Ospedaliera Consorziale - Policlinico Bari
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $3,670
OpenAlex ID: W7127200801