Health-Related Quality of Life Across the Spectrum of Pulmonary Hypertension.

Background: Health-related quality of life (HRQOL) is frequently impaired in pulmonary arterial hypertension. However, little is known about HRQOL in other forms of pulmonary hypertension (PH).
Research Question: Does HRQOL vary across groups of the World Symposium on Pulmonary Hypertension (WSPH) classification system?
Study Design and Methods: This cross-sectional study included patients with PH from the Pulmonary Vascular Disease Phenomics (PVDOMICS) cohort study. HRQOL was assessed by using emPHasis-10 (e-10), the 36-item Medical Outcomes Study Short Form survey (physical component score [PCS] and mental component score), and the Minnesota Living with Heart Failure Questionnaire. Pearson correlations between HRQOL and demographic, physiologic, and imaging characteristics within each WSPH group were tested. Multivariable linear regressions compared HRQOL across WSPH groups, adjusting for demographic characteristics, disease prevalence, functional class, and hemodynamics. Cox proportional hazards models were used to assess associations between HRQOL and survival across WSPH groups.
Results: Among 691 patients with PH, HRQOL correlated with functional class and 6-min walk distance but not hemodynamics. HRQOL was severely depressed across WSPH groups for all measures except the 36-item Medical Outcomes Study Short Form survey mental component score. Compared with Group 1 participants, Group 2 participants had significantly worse HRQOL (e-10 score, 29 vs 24 [P = .001]; PCS, 32.9 ± 8 vs 38.4 ± 10 [P < .0001]; and Minnesota Living with Heart Failure Questionnaire score, 50 vs 38 [P = .003]). Group 3 participants similarly had a worse e-10 score (31 vs 24; P < .0001) and PCS (33.3 ± 9 vs 38.4 ± 10; P < .0001) compared with Group 1 participants, which persisted in multivariable models (P < .05). HRQOL was associated in adjusted models with survival across Groups 1, 2, and 3.
Interpretation: HRQOL was depressed in PH and particularly in Groups 2 and 3 despite less severe hemodynamics. HRQOL is associated with functional capacity, but the severity of hemodynamic disease poorly estimates the impact of PH on patients' lives. Further studies are needed to better identify predictors and treatments to improve HRQOL across the spectrum of PH.
Competing Interests: Financial/Nonfinancial Disclosures The authors have reported to CHEST the following: H. M. D. has received grant funding from Bayer Pharmaceuticals, has served on advisory boards for Janssen Pharmaceuticals and has received consulting fees from Janssen Pharmaceuticals. N. S. H. serves on a clinical trial steering committee for Aerovate, advisory board for Gossamer, DSMB for Merch, and consultant for Liquidia and United Therapeutics. R.M. has received an honorarium from the American Academy of Sleep Medicine; funds for service on the American Board of Internal Medicine and as associate editor of the American Journal of Respiratory and Critical Care Medicine; has received National Institutes of Health funding; and has received royalties from UpToDate. A. R. H. has served as a consultant for Bayer, GossamerBio, Janssen, Merck, Tenax, and United Therapeutics. She owns stock in Tenax. P. M. H. has served on a steering committee for Merck and as consultant ARIA-CVS. S. C. M. has served as a consultant for Acceleron, Janssen, Merck, and United Therapeutics. None declared (A. B., A. B. L., E. M. H., M. S. J., J. A. L., M. K. R., S. C. E., E. B. R., R. P. F., F. P. R., G. J. B.).
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