Patients with pulmonary arterial hypertension: insights from the INSPECTIO study

<bold>Introduction.</bold> Pulmonary arterial hypertension (PAH) is a rare, progressive disease. The clinical importance of patient management based on noninvasive parameters is growing. <bold>Purpose.</bold> The primary objective of INSPECTIO was to assess the change from baseline (M0) to month 12 (M12) in the number of low-risk parameters among World Health Organization functional class (WHO/FC), 6-min walk distance (6MWD), brain natriuretic peptide (BNP) or N-terminal pro-BNP in patients (pts) treated with macitentan and/or selexipag as part of oral combination therapy. <bold>Methods.</bold> Observational, multicenter (29 centers in Italy) study on pts with PAH at low/intermediate mortality risk. <bold>Results.</bold> Among 177 enrolled patients, 148 (median age 63.0 years, 76.4% females, median time from diagnosis 22.6 months) completed the M12 follow up and were considered for this analysis. The number of non-invasive low-risk parameters increased in 43 (29.1%) pts and remained stable in 80 (54.1%) (mean change ±SD 0.16±0.79 [p=0.017]). At M0, 134 (90.5%) pts were in WHO/FC II or III. The WHO/FC status improved in 28 (18.9%) pts at M12. The mean ±SD 6MWD remained stable (381.5 m [±124.57] vs 385.8 m [±131.03]; p=0.95); the percentages of pts with 6MWD >440 m were similar: 32.5% at M0; 35.7% at M12. The quality of life remained stable (mean emPHasis-10 score at M0 20.0±11.51, mean change -1.2±11.10 [p= 0.36]). The therapy was optimized according to the guidelines: the number of pts on triple therapy increased from 42 to 51 (+21.4%). <bold>Conclusions.</bold> During the study, the number of noninvasive, low-risk parameters increased or remained stable in most pts. The therapy optimization based on risk assessment resulted in great value in the management of PAH.