Classi, Peter

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 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 >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

While parenteral prostacyclin (pPCY) therapy, delivered either subcutaneously or intravenously, is recommended for pulmonary arterial hypertension patients with severe or rapidly developing disease, some patients refuse this treatment. This study aimed to understand, directly from patients with pulmonary arterial hypertension, why pPCY was refused and, in some cases, later accepted. Interviews were conducted with 25 pulmonary arterial hypertension patients who previously refused pPCY therapy (Group A: Refused/Never initiated (n = 9) and Group B: Refused/Initiated (n = 16)). Patients in both groups believed that pPCY could improve their symptoms, slow disease progression, and provide them a greater ability to perform activities. Reasons for refusal included concern over side effects and the perceived limitations of pPCY on daily activities. Group A perceived their decision as a balance between quality of life and prolonging life and most acknowledged they would reconsider pPCY if other treatment options were exhausted. Group B cited they initiated therapy due to a worsening of symptoms, disease progression, to improve quality of life, to be there for their family, or a desire to live. Following initiation, Group B indicated their experience met expectations with reduced symptoms, slowed disease progression, and perception of improved survival; concerns related to pPCY were described as manageable. Given the efficacy of pPCY therapy, clinicians should apply knowledge of these findings in clinical practice. Patients noted improvements to parenteral pump technologies to include smaller size, water resistance, and implantability may increase their acceptance of this modality. Development efforts should focus on technologies that increase the acceptance of pPCY when indicated.

Pulmonary hypertension resulting from chronic lung disease such as chronic obstructive pulmonary disease and interstitial lung disease is categorized by the World Health Organization as Group 3 pulmonary hypertension. To identify the symptoms and impacts of World Health Organization Group 3 pulmonary hypertension and to capture data related to the patient experience of this disease, qualitative research interviews were undertaken with 3 clinical experts and 14 individuals with pulmonary hypertension secondary to chronic obstructive pulmonary disease or interstitial lung disease. Shortness of breath, fatigue, cough, and swelling were the most frequently reported symptoms of pulmonary hypertension due to chronic obstructive pulmonary disease or interstitial lung disease, and shortness of breath was further identified as the single most bothersome symptom for most patients (71.4%). Interview participants also described experiencing a number of impacts related to pulmonary hypertension and pulmonary hypertension symptoms, including limitations in the ability to perform activities of daily living and impacts on physical functioning, family life, and social life as well as emotional impacts, which included frustration, depression, anxiety, isolation, and sadness. Results of these qualitative interviews offer an understanding of the patient experience of pulmonary hypertension due to chronic obstructive pulmonary disease or interstitial lung disease, including insight into the symptoms and impacts that are most important to patients in this population. As such, these results may help guide priorities in clinical treatment and assist researchers in their selection of patient‐reported outcome measures for clinical trials in patients with pulmonary hypertension due to chronic obstructive pulmonary disease or interstitial lung disease.