What question did this study set out to answer?

This research aims to evaluate patient reported outcomes in Group 3 pulmonary hypertension among COPD and ILD patients to inform future PRO development.

May 20, 2026

B106-18 Assessing Candidate Patient Reported Outcomes in PH-COPD and PH-ILD to Better Inform Development of PROs Specific to Group 3 PH

Key Points

This research aims to evaluate patient reported outcomes in Group 3 pulmonary hypertension among COPD and ILD patients to inform future PRO development.
Single-center prospective cohort study conducted from 2010-2016
Analyzed various patient reported outcomes (PROs) in subjects evaluated for lung transplantation
Used Mann Whitney U test and multivariate linear regression to assess associations between PRO scores and pulmonary vascular resistance (PVR)
68% of COPD subjects and 57% of ILD subjects had pulmonary hypertension (PH)
No differences in PRO scores among COPD patients with or without PH, while significant physical functional decline noted in ILD patients with severe PH (p < 0.01)
Multivariate analysis showed an association between change in PVR and LT-VLA scores (ΔPVR by 1 WU: 0.65 change in LT-VLA, p = 0.03)

Abstract

Abstract Rationale Clinical trials in Group 3 pulmonary hypertension (PH) have included patient reported outcomes (PROs) as primary or secondary endpoints. Yet, no PROs are specific to Group 3 PH. We sought to assess candidate PROs in chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) to better understand the domains impacted by developing Group 3 PH. Methods In a single-center prospective cohort (2010-2016), we assessed various PROs in subjects with COPD and ILD being evaluated for lung transplantation. PROs assessed included: the SF12-Physical Component Score (SF12PCS), the SF12-Mental Component Score (SF12MCS), the Airway Questionnaire 20-Revised (AQ20R), the Euroqol 5D (EQ5D), the Visual Analog Score (VAS), the Geriatric Depression Scale (GDS), and the Lung Transplant-Valued Life Activities (LT-VLA) instrument. Presence of PH was defined by mPAP 20 mmHg and PVR 2 WU. Presence of severe PH was defined by PVR 5 WU. We tested differences in the various PROs amongst subjects with and without PH using the Mann Whitney U test, followed by multivariate linear regression assessing the association between PRO scores and PVR. Results Of 288 subjects, 75 had COPD and 213 had ILD. PH was present in 68% of subjects with COPD and 57% of subjects with ILD. Severe PH was present in 12% of subjects with COPD and 43% of subjects with ILD. Amongst patients with COPD, there was no difference in PROs when comparing PH-COPD versus those without PH, nor when stratified by diagnosis of severe PH-COPD (Table). Amongst patients with ILD, the presence of PH was associated with worse physical functioning by LT-VLA, but no differences were found in other PROs. When stratified by severe PH, those with severe PH-ILD also had worse physical functioning by LT-VLA (1.71 1.27-2.27 vs 1.18 0.73-1.70, p 0.01). The difference was not only statistically significant but also exceeded the minimally clinically important difference of 0.3 for the LT-VLA instrument. Multivariate linear regression also demonstrated an association between PVR and LT-VLA, even after adjusting for age, vital capacity, and carbon dioxide (ΔPVR by 1 WU: 0.65 change in LT-VLA 95%CI: 0.08-1.21, p = 0.03). Conclusions Despite Group 3 PH impacting adverse clinical outcomes, identifying candidate PROs remains elusive in this population. Patients with PH-COPD report similar scores across various PROs as patients with COPD without PH. Future development of PROs specific to the impact of developing PH on patients with ILD should focus on physical functioning given the differences we found in LT-VLA. This abstract is funded by: NHLBI K23 HL111115 (JPS), RO1 HL134851 (JPS)

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