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Overnight inpatient monitoring is common following balloon pulmonary angioplasty (BPA) for chronic thromboembolic pulmonary hypertension (CTEPH). We describe our institutional experience in same-day discharge (SDD) after BPA. Across 78 BPA sessions, there were only 2 (2.6%) admissions for hemoptysis with no reperfusion lung injury or deaths at 30 days., Competing Interests: The authors declare no conflicts of interest., (© 2024 The Author(s). Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)

Few studies have evaluated the effects of pulmonary arterial hypertension therapies on pericardial effusion. We evaluated hemodynamics, echocardiograms, and outcomes for 119 parenteral prostanoid-treated patients. We discovered an increased frequency of pericardial effusions posttreatment, and that a moderate-large pericardial effusion at initiation, but not at 1st follow-up, was significantly associated with mortality., Competing Interests: The authors declare no conflict of interest., (© 2023 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)

Macitentan is an oral endothelin receptor antagonist for the management of pulmonary arterial hypertension (PAH). The OPsumit® USers Registry (OPUS) and the OPsumit® Historical USers cohort (OrPHeUS) medical chart review provide real-world data for patients newly initiating macitentan. This study aims to describe the characteristics, safety profile, and clinical outcomes of PAH patients newly treated with macitentan in the combined OPUS/OrPHeUS data set. OPUS was a prospective, multicenter, long-term, observational drug registry from April 2014 to June 2020. OrPHeUS was a retrospective, US, multicenter chart review: observation period October 2013 to March 2017. All analyses were descriptive. At registry closure in June 2020, the combined population consisted of 5654 patients, of whom 81.9% were diagnosed with PAH. For these 4626 patients, median duration of macitentan exposure observed was 14.5 (Q1 = 5.2, Q3 = 29.0) months; idiopathic PAH (54.8%) was the most common form of PAH; macitentan was initiated as monotherapy (37.9%), or as part of double (48.0%) or triple therapy (14.1%); discontinuation due to nonhepatic/hepatic adverse events occurred in 17.1%/0.3% of patients; 9.9% of patients experienced ≥1 hepatic adverse events; Kaplan-Meier estimates showed that at 1 year 59.9% (95% confidence interval: 58.3, 61.5) of patients were free from hospitalization and survival was 90.4% (89.3, 91.3). This analysis of real-world data from the combined OPUS and OrPHeUS populations demonstrated that macitentan is well tolerated in a large, diverse population of PAH patients, with overall and hepatic safety profiles consistent with previous macitentan clinical trials., Competing Interests: V. V. M. reports serving as a steering committee member, as well as receiving consultant fees, and research grants/support from Janssen Pharmaceutical Companies of Johnson & Johnson; consultant fees and research grants/support from United Therapeutics, Bayer, and Acceleron Pharma, Inc.; consultant fees from Altavant, Caremark, CiVi Biopharma Inc., and Gossamer Bio; and research grants/support from Reata Pharmaceuticals and SoniVie. R. C. serves as a steering committee member, served on an advisory board, and received research grants/support, and speaker fees from Janssen Pharmaceutical Companies of Johnson & Johnson; has served on an advisory board for Bayer; has received consultancy and speaker fees from Bayer and Arena Pharmaceuticals; and has received research grants from United Therapeutics. N. H. K. has served a steering committee member and received research grants/support and speaker fees from Janssen Pharmaceutical Companies of Johnson & Johnson; has received research grants/support from Bellerophon, Eiger, Gossamer Bio, Lung Biotechnology, and SoniVie; consultant fees from Bayer, Merck, and United Therapeutics; and speaker fees from Bayer. R. P. F has served as a steering committee and advisory board member for Janssen Pharmaceutical Companies of Johnson & Johnson, Gossamer Bio, Liquidia, and Tenax; and as an advisory board member for Altavant Sciences and ShoTi; and received royalties from UptoDate. J.W. M. received financial support, research grants and personal fees from Janssen Pharmaceutical Companies of Johnson & Johnson; personal fees and financial support from Bayer Pharmaceuticals, United Therapeutics, Reata Pharmaceuticals and Arena Pharmaceuticals; and financial support from Eiger Pharmaceuticals, and Bellerophon. L. M. G. has served on advisory boards and received speaker fees from Janssen Pharmaceutical Companies of Johnson & Johnson, has received speaker fees from United Therapeutics, Gilead, and Bayer Pharmaceuticals; and served on advisory boards for United Therapeutics and Bayer Pharmaceuticals. C. M. reports personal fees from Janssen Pharmaceutical Companies of Johnson & Johnson, United Therapeutics, Gilead, and Bayer. A. R. reports speaker fees from Janssen Pharmaceutical Companies of Johnson & Johnson, Bayer Pharmaceuticals, United Therapeutics. J. R. L. has served on an advisory board for and received research support from Janssen Pharmaceutical Companies of Johnson & Johnson; has received research support from United Therapeutics and Bayer, and has served on an advisory board for Bayer. M. B. and S. L. are employees of Janssen Pharmaceutical Companies of Johnson & Johnson and in the past held stock/stock options for Actelion Pharmaceuticals Ltd. and currently hold stock/stock options in the parent company Johnson & Johnson. G. W. was an employee of Janssen Pharmaceutical Companies of Johnson & Johnson at the time that the analyses and this publication were developed. K. C. has served as a steering committee member, on an advisory board, and has received research grants/support, and consultancy fees from Janssen Pharmaceutical Companies of Johnson & Johnson; has received research grants from United Therapeutics and Ironwood Pharmaceuticals; has served on an advisory board for Bayer Healthcare; and has received consultancy fees from United Therapeutics., (© 2022 Actelion Pharmaceuticals Ltd. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)

In order to evaluate the therapeutic potential of fluoxetine in pulmonary arterial hypertension, 13 patients with pulmonary arterial hypertension underwent catheterization before and after 12 (N = 5) or 24 (N = 8) weeks fluoxetine therapy. No change was seen in the primary endpoint of pulmonary vascular resistance, other hemodynamic values, or any secondary endpoints., (© The Author(s) 2020.)

Patient-reported outcomes are important measures to include in pulmonary arterial hypertension clinical trials but are not widely utilized in clinical practice. Pulmonary Arterial Hypertension-Symptoms and Impact Questionnaire (PAH-SYMPACT) is the only pulmonary arterial hypertension-specific patient-reported outcomes instrument developed and validated in accordance with the US Food and Drug Administration guidance on patient-reported outcomes development. The PAH-SYMPACT tool measures pulmonary arterial hypertension-related symptoms and impact of pulmonary arterial hypertension on daily life. Symptoms are reported each day for seven consecutive days, and the impact of pulmonary arterial hypertension over one week is recalled and reported on day 7; however, daily symptom reporting may overburden patients and healthcare resources, limiting the practicality of PAH-SYMPACT outside of clinical trials. To determine the practicability of an abridged version of PAH-SYMPACT for which all reporting is completed on one day, symptom data from the SYMPHONY trial (NCT01841762; PAH-SYMPACT validation study) were retrospectively analyzed to assess whether symptoms reported on each day correlated with the weekly average and whether one-day symptom scores were sensitive to disease severity. Correlation coefficients comparing the weekly average and individual day symptom scores were mostly high or very high regardless of the day they were measured. Findings were similar when using either Spearman's rank correlation or weighted kappa method. One-day symptom scores differentiated well between World Health Organization functional classes II and III/IV pulmonary arterial hypertension and were sensitive to change in disease severity as measured by the Patient Global Assessment of Disease Severity. These data suggest that the one-day PAH-SYMPACT is feasible and appropriate for routine implementation in clinical practice., (© The Author(s) 2020.)

Sustained-release oral treprostinil, an oral prostacyclin, led to significant improvement in 6-minute walk distance (6MWD) versus placebo in treatment-naive patients with pulmonary arterial hypertension (PAH) but failed to lead to significant improvement in two 16-week trials in patients receiving background PAH therapies (FREEDOM studies). Long-term studies are lacking. Our objective was to evaluate 6MWD, functional class, hemodynamics, and other long-term outcomes during oral treprostinil administration in PAH. Patients receiving oral treprostinil through the FREEDOM studies at our institution were included and were followed for up to 7 years. The primary end point was change in pulmonary vascular resistance (PVR) at first follow-up catheterization. Other end points included 6MWD, functional class, and other hemodynamic results. Thirty-seven patients received oral treprostinil for a median of 948 days, with 81%, 61%, and 47% continuing therapy at 1, 2, and 3 years, respectively. Mean treprostinil dose at 3, 12, and 24 months was 4.3 ± 2.3, 8.6 ± 3.2, and 11.7 ± 5.8 mg/24 h, respectively. Compared with pretreatment values, there was no significant change in 6MWD at 3 or 12 months, no improvement in functional class at 12 months, and no significant change in hemodynamics at the first follow-up catheterization (N = 34). Oral treprostinil dose was inversely associated with change in PVR (r = -0.42, P < 0.05), and change in PVR was numerically better among patients in the highest dosing quartile. No significant improvement in 6MWD, functional class, or hemodynamics versus pretreatment values was seen with long-term oral treprostinil therapy, potentially because of inability to achieve a clinically effective dose.