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 (PAH). 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 PH patients from PVDOMICS (Pulmonary Vascular Disease Phenomics). HRQOL was assessed using emPHasis-10 (e-10), Medical Outcomes Survey Short Form-36 (SF-36; physical component (PCS) and mental component (MCS)), and Minnesota Living with Heart Failure Questionnaire (MLHF). Pearson’s 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 demographics, disease prevalence, functional class, and hemodynamics. Cox proportional hazard models were used to assess associations between HRQOL and survival across WSPH groups. Results Among 691 PH patients, HRQOL correlated with functional class and six-minute walk distance but not hemodynamics. HRQOL was severely depressed across WSPH groups for all measures except the SF-36 MCS. When compared to Group 1, Group 2 subjects had significantly worse HRQOL (e-10: 29 vs 24, p=0.001, PCS: 32.9 ± 8 vs 38.4 ± 10, p<0.0001, MLHF: 50 vs 38, p=0.003). Group 3 subjects similarly had worse e-10 (31 vs 24, p<0.0001) and PCS scores (33.3 ± 9 vs 38.4 ± 10, p<0.0001) compared to Group 1, which persisted in multivariable models (p<0.05). HRQOL was associated in adjusted models with survival across Groups 1, 2, and 3. Interpretation HRQOL is depressed in PH and particularly in Groups 2 and 3 despite less severe hemodynamics. HRQOL is associated with functional capacity, but hemodynamic disease severity poorly estimates the impact of PH on patient’s lives. Further studies are needed to better identify predictors and treatments to improve HRQOL across the spectrum of pulmonary hypertension.