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Case Presentation: A 76-year-old male Vietnam veteran with a medical history of OSA on CPAP, mild COPD, Parsonage-Turner syndrome (a rare neurologic syndrome manifesting with shoulder and arm pain), hypertension, gastroesophageal reflux, hiatal hernia, and prior endocarditis presented with 1 year duration progressive exertional dyspnea with minimal activity by referral from an outside pulmonologist. The patient reported possible exposure to Agent Orange during his service but was otherwise without significant occupational or environmental exposures. His exercise tolerance was well-maintained up until the last 12 months. Aside from marginal cigarette use, he denied any recreational drug use or any anorectic use. The patient provided records from a recent right heart catheterization (RHC) months earlier for review., Competing Interests: Financial/Nonfinancial Disclosures The authors have reported to CHEST the following: F. F. R. has served as a consultant or speaker for United Therapeutics Corporation, Actelion Pharmaceuticals, Gilead Sciences, Bayer Healthcare Pharmaceuticals, and Lung Therapeutics Inc. None declared (V. W., I. R. D., L. S.)., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Pulmonary hypertension (PH) is a common complication of interstitial lung disease (ILD) and is associated with worse outcomes and increased mortality. Evaluation of PH is recommended in lung transplant candidates, but there are currently no standardized screening approaches. Trials have identified therapies that are effective in this setting, providing another rationale to routinely screen patients with ILD for PH., Research Question: What screening strategies for identifying PH in patients with ILD are supported by expert consensus?, Study Design and Methods: The study convened a panel of 16 pulmonologists with expertise in PH and ILD, and used a modified Delphi consensus process with three surveys to identify PH screening strategies. Survey 1 consisted primarily of open-ended questions. Surveys 2 and 3 were developed from responses to survey 1 and contained statements about PH screening that panelists rated from -5 (strongly disagree) to 5 (strongly agree)., Results: Panelists reached consensus on several triggers for suspicion of PH including the following: symptoms, clinical signs, findings on chest CT scan or other imaging, abnormalities in pulse oximetry, elevations in brain natriuretic peptide (BNP) or N-terminal pro-brain natriuretic peptide (NT-proBNP), and unexplained worsening in pulmonary function tests or 6-min walk distance. Echocardiography and BNP/NT-proBNP were identified as screening tools for PH. Right heart catheterization was deemed essential for confirming PH., Interpretation: Many patients with ILD may benefit from early evaluation of PH now that an approved therapy is available. Protocols to evaluate patients with ILD often overlap with evaluations for pulmonary hypertension-interstitial lung disease and can be used to assess the risk of PH. Because standardized approaches are lacking, this consensus statement is intended to aid physicians in the identification of patients with ILD and possible PH, and provide guidance for timely right heart catheterization., (Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Riociguat is effective in delaying the time to clinical worsening (TCW) in patients with groups 1 and 4 pulmonary hypertension., Research Question: Is riociguat more effective than placebo in prolonging TCW in sarcoidosis-associated pulmonary hypertension (SAPH)?, Study Design and Methods: This was a double-blind placebo-controlled trial. Patients with SAPH confirmed by right heart catheterization were randomized 1:1 to riociguat or placebo. Patients underwent 6-min walk distance (6MWD) and spirometry testing every 8 weeks. The primary end point was TCW, which was defined by the time to the first of the following: (1) all-cause mortality, (2) need for hospitalization because of worsening cardiopulmonary status attributable to progression of disease, (3) > 50 m decrease in the 6MWD test, or (4) worsening of World Health Organization functional class., Results: A total of 16 patients were randomized to riociguat (n = 8) or placebo (n = 8). No difference was found in pulmonary artery mean, pulmonary vascular resistance, initial 6MWD, or FVC between the two groups. Five of eight patients who received placebo met TCW criteria, whereas none of the patients who received riociguat experienced a qualifying event. By log-rank analysis, patients who received riociguat were in the study for a significantly longer period (χ 2  = 6.259; P = .0124). The 6MWD decreased in the placebo group (median, -55.9 m; range, -176.8 to 60 m), but rose in the riociguat group (median, +42.7 m; range, -7.5 to +91.4 m; P = .0149), with a placebo-corrected difference of 94 m (P < .01). Four of eight patients who received riociguat, but only 1 of 8 patients who received placebo, showed a > 30-m improvement in 6MWD (P > .05). No significant adverse events associated with riociguat occurred., Interpretation: Over the 1 year of the study, riociguat was effective in preventing clinical worsening and improving exercise capacity in patients with SAPH., Trial Registry: ClinicalTrials.gov; No.: NCT02625558; URL: www.clinicaltrials.gov., (Copyright © 2021 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)