Wu, Benjamin

ABSTRACT Palliative care can be offered concurrently as a treatment option to patients with pulmonary arterial hypertension (PAH) to relieve symptoms and improve quality of life. However, it is infrequently used and is associated with misconceptions. Limited studies have reported patients' perspectives of palliative care as a treatment option for PAH. We conducted a web‐based survey, informed by a brief targeted literature review and input from patients and clinicians, which was completed by adults (≥ 18 years) in the United States (US) with self‐reported PAH. Patients reported demographic and disease variables and their perspectives and experiences with palliative care. Patients self‐reported their Pulmonary Hypertension Functional Class Self‐Report class, EmPHasis‐10, and 6‐min walk test. A total of 200 respondents (86.5% female; mean age 58.5 years) from 42 US states completed the survey between 19 September and 3 October 2023. Most respondents described palliative care as “end‐of‐life care” ( n = 43, 25.7%) and associated it with the need for extra support ( n = 111, 55.5%). Many respondents ( n = 171, 85.5%) had not discussed palliative care services, nor had a palliative care consultation with their healthcare provider (HCP); most had not been offered a consultation ( n = 109/171, 63.7%). Despite this low frequency of discussions, respondents reported feeling comfortable discussing palliative care with their HCPs ( n = 177, 88.5%). The survey results demonstrated that these patients with PAH had limited experiences with palliative care, where palliative care was frequently subject to misinterpretation. These findings emphasize the need for proactive engagement between patients and HCPs to ensure palliative care is optimally used and understood.

ABSTRACT Pulmonary hypertension due to interstitial lung disease (PH‐ILD) is associated with high morbidity and mortality. Real‐world patients initiating inhaled treprostinil are not well‐characterized. This retrospective cohort study aimed to evaluate healthcare resource utilization in patients with PH‐ILD who initiated treatment with inhaled treprostinil vs patients who remained untreated. Adult patients diagnosed with PH‐ILD were indexed on initiation of inhaled treprostinil and patients who remained untreated were indexed on first observed PH diagnosis (31 March 2021–30 September 2024). Patients were excluded if they were ever treated with any pulmonary arterial hypertension therapy. Inhaled treprostinil patients were matched to up to four untreated patients. All‐cause per‐patient per‐month (PPPM) hospitalizations and ICU‐related hospitalizations were the primary outcomes of interest. 294 patients treated with inhaled treprostinil and 736 untreated patients were identified. Mean all‐cause PPPM hospitalizations remained unchanged in the pre‐index vs post‐index periods in the inhaled treprostinil cohort (0.11 vs 0.12; p = 0.42) but significantly increased in untreated patients (0.12 vs 0.23; p < 0.01). ICU utilization also remained unchanged in the pre‐index vs post‐index periods for the inhaled treprostinil cohort (0.06 vs 0.07; p = 0.11) compared to the untreated cohort (0.06 vs 0.13; p < 0.01). Untreated patients had significantly higher post‐index PPPM hospitalizations ( p < 0.01) and ICU utilization ( p < 0.01) compared to inhaled treprostinil patients. Patients who initiated inhaled treprostinil had a 30% decreased risk of hospitalization compared to untreated patients (relative risk: 0.70; 95% CI: 0.59–0.83; p < 0.01). Among real‐world patients with PH‐ILD, treatment with inhaled treprostinil is associated with fewer all‐cause hospitalizations and ICU‐related hospitalizations compared to untreated patients with PH‐ILD.

Abstract Background Post-hoc analyses of the FREEDOM-EV study demonstrated that addition of oral treprostinil (ORE) to background monotherapy improves risk in patients with pulmonary arterial hypertension (PAH). Evidence is needed to understand the incremental effect of ORE added to dual background therapy with an endothelin receptor antagonist (ERA) and phosphodiesterase-5 inhibitor (PDE5)/soluble guanylate cyclase stimulator (sGC). Purpose To describe longitudinal changes in REVEAL Lite 2 and COMPERA 2.0 risk status in patients initiating ORE on dual background therapy. Methods A multisite, double-blind retrospective chart review of PAH patients was conducted by US PAH-treating physicians and included patients meeting prior diagnostic PAH criteria (mPAP ≥25 mmHg/PAWP ≤15 mmHg/PVR &gt;3 WU), age ≥18 at diagnosis, began a PAH treatment 01/2014–03/2017, and had NT-proBNP/BNP, WHO functional class (FC), and 6-minute walk distance (6MWD) at baseline visit. Baseline was most recent assessment prior to ORE initiation. This analysis included a subset of patients on dual background therapy at time of ORE initiation without prior prostacyclin use. Follow-up was evaluated in 0–6 and 6–12 months after ORE initiation. Results 30 (61.2%) of 49 ORE patients were on dual background therapy at time of ORE start; 23 had baseline assessments while on dual background therapy and were included in this analysis. At baseline, mean (SD) 6MWD was 140.9 (49.8) m and 21 (91.3%) patients were FC III. Prior to ORE, median (IQR) time on ERA was 6.6 (5.5–7.6) months and PDE5/sGC was 3.7 (3.3–5.7) months. After ORE start, median (IQR) time to follow-up was 4.3 (3.4–5.6) months in 0–6 months and 10.9 (9.6–11.6) months in 6–12 months. Using REVEAL Lite 2, baseline mean (SD) REVEAL Lite 2 risk score was 7.9 (0.8); 1 (4.4%), 2 (8.7%), and 20 (87%) patients were in the low, intermediate, and high risk strata, respectively. In 0–6 months (n=22), 13 (59.1%) patients improved and 9 (40.9%) stabilized REVEAL Lite 2 risk strata from baseline. In 6–12 months (n=22), mean (SD) REVEAL Lite 2 risk score was 6.5 (1.1); 15 patients (68.2%) improved, 6 (27.3%) stabilized, and 1 (4.6%) worsened REVEAL Lite 2 risk strata from baseline. Using COMPERA 2.0, at baseline, 1 (4.4%) patient was in the intermediate-low and 22 (95.7%) patients were in the intermediate-high risk strata. In 0–6 months (n=15), 10 (66.7%) patients improved and 5 (33.3%) patients stabilized COMPERA 2.0 risk strata from baseline. In 6–12 months (n=17), 15 (88.2%) patients improved and 2 (11.8%) stabilized COMPERA 2.0 risk strata from baseline. Conclusion Of patients initiating oral treprostinil in this study, most were on dual background therapy and at high risk by REVEAL Lite 2 or intermediate-high risk by COMPERA 2.0. This analysis suggests adding ORE to dual background therapy can improve risk status in patients, with clinical benefit attained within 6 months and continued incremental improvement through first year of therapy. Funding Acknowledgement Type of funding sources: Private company. Main funding source(s): United Therapeutics Corporation