The SAVOIR study: protocol for the development and validation of a PAH knowledge questionnaire

Area of Science:

• Cardiology
• Pulmonology
• Pharmacology

Background:

• Pulmonary hypertension associated with interstitial lung disease (ILD-PH) carries significant morbidity and mortality.
• Limited real-world data exists on longitudinal outcomes and risk stratification in ILD-PH.
• Therapeutic options for ILD-PH are currently restricted.

Purpose of the Study:

• To evaluate the effect of inhaled iloprost on risk stratification in ILD-PH patients within a real-world clinical setting.
• To assess changes in six-minute walk distance, natriuretic peptide levels, and hemodynamic parameters following iloprost initiation.
• To analyze survival outcomes and identify factors influencing prognosis in ILD-PH.

Main Methods:

• A retrospective cohort study of ILD-PH patients initiating inhaled iloprost (n=72) was conducted.
• A comparator group of ILD-PH patients not receiving iloprost (n=14) was included for survival analysis.
• Clinical, functional, hemodynamic data, and COMPERA 2.0 risk stratification were assessed at baseline and follow-up.

Main Results:

• Inhaled iloprost did not significantly improve 6-minute walk distance, WHO functional class, BNP, or hemodynamic parameters.
• Modest increases in right atrial pressure and decreases in mixed venous oxygen saturation were observed, with uncertain clinical significance.
• Mortality (32%) and hospitalization (50%) rates remained high, with limited changes in risk stratification scores.

Conclusions:

• Inhaled iloprost showed no measurable clinical, functional, or hemodynamic benefits in this ILD-PH cohort.
• Substantial mortality and hospitalization rates persist despite iloprost treatment.
• Current PAH-derived risk stratification tools are inadequate for ILD-PH, necessitating the development of ILD-PH-specific prognostic frameworks.