Analysis of quality of life indicators using various questionnaires in patients with idiopathic pulmonary hypertension

Idiopathic pulmonary hypertension (IPAH) is a rare severe disease with a poor prognosis. Assessing quality of life (QoL) using questionnaires can significantly complement clinical, laboratory and instrumental data. Aim. To assess QoL using various questionnaires in patients with a newly and previously diagnosed IPAH. Materials and methods. 73 patients with IPAH were included in the study, all patients filled out Qol questionnaires: universal (SF-36, EQ-5D-3L) and special for patients with pulmonary hypertension (CAMPHOR, PAH-SYMPACT, EmPHasis-10). Two groups were formed, comparable by age and gender: group 1-42 patients with newly diagnosed IPAH; group 2-31 patients with a previously diagnosed IPAH. Results. Significant differences in SF-36 were found in the scale of Mental Health (52 [40; 64] % in the first group and 60 [52; 80] % in the second, p=0,02) and Mental component summary (40,8 [34,3; 49,3] % and 49,1 [41,0; 55,9] %, respectively, p=0,03). According to the EQ-5D-3L results, differences were found in the Visual Analogue Scale (VAS) and EQ-5D Index: higher values were found in the second group (the median VAS score was 50 [40; 60] % in the first group and 60 [50; 70] % in the second, p=0,006, EQ-5D Index – 0,60 [0,30; 0,71] and 0,66 [0,57; 0,76], respectively, p=0,05). No significant differences were found in the results of CAMPHOR, PAH-SYMPACT, and EmPHasis-10 in two groups. Conclusions. Pathogenetic therapy promotes improvement of functional status of patients with IPAH. Low emotional status indicators in patients with a prevalent disease indicates the need to develop a system of psychological assistance for IPAH patients.