Longitudinal Outcomes of Patients With Group 3 Pulmonary Hypertension Treated With Iloprost

This observational real-world study aims to evaluate the longitudinal clinical outcomes of adult patients with Group 3 pulmonary hypertension (PH), associated with chronic lung diseases such as interstitial lung disease, who are treated with inhaled iloprost in routine clinical practice. Treatment options for Group 3 PH remain limited, and the use of pulmonary vasodilators is controversial due to potential worsening of gas exchange. Inhaled iloprost may provide a more selective approach by targeting well-ventilated lung regions. However, real-world data on its use in this population are scarce. The primary objective of this study is to assess changes in clinical risk status over time using the COMPERA 2.0 four-stratum risk model. Secondary objectives include describing patient characteristics, treatment patterns, and the evolution of functional parameters and biomarkers, as well as documenting relevant clinical events such as hospitalizations, treatment escalation, and discontinuation. The study will retrospectively analyze data from approximately 50 adult patients with confirmed Group 3 PH who received inhaled iloprost as part of their routine care in a specialized pulmonary hypertension center. No additional patient contact or interventions will occur.

Inclusion Criteria: * Age ≥18 years * Diagnosis of group 3 pulmonary hypertension confirmed by right heart catheterization, defined as mean pulmonary artery pressure \>20 mmHg, pulmonary artery wedge pressure ≤15 mmHg, and pulmonary vascular resistance \>2 Wood units * Presence of documented underlying chronic lung disease (e.g., interstitial lung disease confirmed by high-resolution computed tomography with restrictive pattern on pulmonary function testing, or chronic obstructive pulmonary disease) * Use of inhaled iloprost for a minimum of 3 months, documented in medical records * Availability of baseline clinical data (at initiation of inhaled iloprost) and at least one follow-up assessment Exclusion Criteria: * Pulmonary hypertension from other clinical groups (Groups 1, 2, 4, or 5) as the predominant diagnosis * Insufficient clinical information in medical records to allow characterization or longitudinal assessment