Riociguat in pulmonary arterial hypertension: Application of the 4-strata COMPERA 2.0 risk assessment tool in the PATENT studies.

Background: Risk stratification is an essential part of evaluating disease severity in patients with pulmonary arterial hypertension (PAH). This study applied the 4-strata COMPERA 2.0 risk model to the Phase 3 PATENT-1/2 studies of riociguat.

Impact of the COVID-19 pandemic on chronic disease management and patient reported outcomes in patients with pulmonary hypertension: The Pulmonary Hypertension Association Registry.

To better understand the impact of the COVID-19 pandemic on the care of patients with pulmonary hypertension, we conducted a retrospective cohort study evaluating health insurance status, healthcare access, disease severity, and patient reported outcomes in this population. Using the Pulmonary Hypertension Association Registry (PHAR), we defined and extracted a longitudinal cohort of pulmonary arterial hypertension (PAH) patients from the PHAR's inception in 2015 until March 2022. We used generalized estimating equations to model the impact of the COVID-19 pandemic on patient outcomes, adjusting for demographic confounders.

Gaps in the Care of Pulmonary Hypertension: A Cross-Sectional Patient Simulation Study Among Practicing Cardiologists and Pulmonologists.

Background Diagnosis of pulmonary hypertension (PH) is often delayed or missed, leading to disease progression and missed treatment opportunities. In this study, we measured variation in care provided by board-certified cardiologists and pulmonologists in simulated patients with potentially undiagnosed PH. Methods and Results In a cross-sectional study (https://www.

Safety of macitentan for the treatment of pulmonary hypertension: Real-world experience from the OPsumit® USers Registry (OPUS) and OPsumit® Historical USers cohort (OrPHeUS).

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.

Delphi consensus recommendation for optimization of pulmonary hypertension therapy focusing on switching from a phosphodiesterase 5 inhibitor to riociguat.

Dual combination therapy with a phosphodiesterase-5 inhibitor (PDE5i) and endothelin receptor antagonist is recommended for most patients with intermediate-risk pulmonary arterial hypertension (PAH). The RESPITE and REPLACE studies suggest that switching from a PDE5i to a soluble guanylate cyclase (sGC) activator may provide clinical improvement in this situation. The optimal approach to escalation or transition of therapy in this or other scenarios is not well defined.

Hispanic Ethnicity and Social Determinants of Health in Pulmonary Arterial Hypertension: The Pulmonary Hypertension Association Registry.

There is a noticeable underrepresentation of minorities in clinical trials and registries in pulmonary arterial hypertension (PAH). Prior studies evaluating the association between Hispanic ethnicity and clinical outcomes in patients with PAH have not assessed the socioeconomic profile of Hispanic individuals or the significance of social determinants of health in clinical outcomes. To determine the association between Hispanic ethnicity, social determinants of health, and clinical outcomes in PAH.

Health-related quality of life and hospitalizations in chronic thromboembolic pulmonary hypertension versus idiopathic pulmonary arterial hypertension: an analysis from the Pulmonary Hypertension Association Registry (PHAR).

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, morbid, potentially curable subtype of pulmonary hypertension that negatively impacts health-related quality of life (HRQoL). Little is known about differences in HRQoL and hospitalization between CTEPH patients and idiopathic pulmonary arterial hypertension (IPAH) patients. Using multivariable linear regression and mixed effects models, we examined differences in HRQoL assessed by emPHasis-10 (E10) and SF-12 between CTEPH and IPAH patients in the Pulmonary Hypertension Association Registry, a prospective multicenter cohort of patients newly evaluated at a Pulmonary Hypertension Care Center.

Comparative effectiveness of oral prostacyclin pathway drugs on hospitalization in patients with pulmonary hypertension in the United States: a retrospective database analysis.

Two oral medications targeting the prostacyclin pathway are available to treat pulmonary arterial hypertension in the United States: oral treprostinil and selexipag. We compared real-world hospitalization in patients receiving these medications. A retrospective administrative claims study was conducted using the Optum® Clinformatics® Data Mart database.

Obesity in Pulmonary Arterial Hypertension (PAH): The Pulmonary Hypertension Association Registry (PHAR).

Rationale: Obesity is associated with pulmonary arterial hypertension (PAH), but its impact on outcomes such as health-related quality of life (HRQoL), hospitalizations and survival is not well understood.

Objectives: To assess the effect of obesity on health-related quality of life (HRQoL), hospitalizations and survival in patients with PAH.

Methods: We performed a cohort study of adults with PAH from the Pulmonary Hypertension Association Registry, a prospective multicenter registry.

Clinical Differences and Outcomes between Methamphetamine-associated and Idiopathic Pulmonary Arterial Hypertension in the Pulmonary Hypertension Association Registry.

Single-center studies demonstrated that methamphetamine use is associated with pulmonary arterial hypertension (Meth-APAH). We used the Pulmonary Hypertension Association Registry to evaluate the national distribution of Meth-APAH and to compare its impact on patient-reported and clinical outcomes relative to idiopathic PAH. To determine if patients with Meth-APAH differ from those with idiopathic PAH in demographics, regional distribution in the United States, hemodynamics, health-related quality of life, PAH-specific treatment, and health care use.

Age-related differences in hemodynamics and functional status in pulmonary arterial hypertension: Baseline results from the Pulmonary Hypertension Association Registry.

Background: The age of patients with pulmonary arterial hypertension (PAH) has increased, with registries now reporting mean ages of 50 to 65 years old. Limited data exist on age-related differences in hemodynamic and functional assessments in PAH.

Methods: Adults with PAH in the Pulmonary Hypertension Association Registry were divided into 3 groups (18-50, 51-65, and >65 years old).

Safety and tolerability of transition from inhaled treprostinil to oral selexipag in pulmonary arterial hypertension: Results from the TRANSIT-1 study.

Background: A long-term trial showed that the oral prostacyclin (PGl) receptor (IP) agonist, selexipag, delayed disease progression in patients with pulmonary arterial hypertension (PAH). Transition to selexipag in patients treated with more burdensome inhaled therapies that target the prostacyclin pathway may be considered by patients and physicians. The Phase 3b, prospective, open-label TRANSIT-1 (Tolerability and Safety of the Transition From Inhaled Treprostinil to Oral Selexipag in Patients With Pulmonary Arterial Hypertension) study evaluated the safety and tolerability of transition from inhaled treprostinil to oral selexipag.

Psychometric Validation of the Pulmonary Arterial Hypertension-Symptoms and Impact (PAH-SYMPACT) Questionnaire: Results of the SYMPHONY Trial.

Background: Disease-specific patient-reported outcome (PRO) instruments are important in assessing the impact of disease and treatment. The Pulmonary Arterial Hypertension-Symptoms and Impact Questionnaire is the first instrument for quantifying pulmonary arterial hypertension (PAH) symptoms and impacts developed according to the 2009 US Food and Drug Administration PRO guidance; previous qualitative research in patients with PAH supported its initial content validity.

Methods: Content finalization and psychometric validation were conducted by using data from A Study of Macitentan in Pulmonary Arterial Hypertension to Validate the PAH-SYMPACT (SYMPHONY), a single-arm, 16-week trial with macitentan 10 mg in US patients with PAH.

Recommendations for the clinical management of patients receiving macitentan for pulmonary arterial hypertension (PAH): A Delphi consensus document.

In patients treated with macitentan (Opsumit®, Actelion Pharmaceuticals Ltd., Basel, Switzerland) for pulmonary arterial hypertension (PAH), prevention and/or effective management of treatment-related adverse events may improve adherence. However, management of these adverse events can be challenging and the base of evidence and clinical experience for macitentan is limited.

Effects of yoga on patients in an adolescent mental health hospital and the relationship between those effects and the patients' sensory-processing patterns.

Problem: This study investigated the effects of yoga as a sensory regulation tool in reducing adolescent distress in an acute care psychiatric hospital.

Methods: This was a descriptive, correlational pre-intervention/post-intervention design conducted in a mental health hospital over 5 months from mid-January to mid-June 2012. The population consisted of a convenience sample of 75 adolescent mental health unit inpatients and partial-hospitalization patients 12-18 years of age who participated in two or more yoga sessions.

Fine structure in proton emission from 145Tm discovered with digital signal processing.

Fine structure in proton emission from the 3.1(3) mus activity of 145Tm was discovered by using a novel technique of digital processing of overlapping recoil implantation and decay signals. Proton transitions to the ground state of 144Er and to its first excited 2(+) state at 0.

Clinical use of the respiratory quotient obtained from indirect calorimetry.

Background: The respiratory quotient (RQ) obtained from indirect calorimetry (IC), defined by the ratio carbon dioxide production (VCO2)/oxygen consumption (VO2), is affected by extremes of substrate use by the body. Underfeeding, which promotes use of endogenous fat stores, should cause decreases in the RQ, whereas overfeeding, which results in lipogenesis, should cause increases in the RQ. Marked increases in VCO2 (with subsequent increases in RQ) in response to overfeeding may cause respiratory compromise in patients with limited pulmonary reserve.

Effects of therapeutic doses of albuterol on beta2-adrenergic receptor density and metabolic changes.

Beta2-agonist drugs at inhaled supratherapeutic doses or when given orally or parenterally alter peripheral lymphocyte beta2-adrenoceptor density (betaAR) and have demonstrable metabolic effects. However, it is not known whether these changes occur at therapeutic inhaled doses. We therefore studied the effects of therapeutic doses of inhaled albuterol in five asthmatic subjects (mean age 23.

Effects of acute prolonged exposure to high-altitude hypoxia on exercise-induced breathlessness.

The direct effects of hypoxia on exercise-induced breathlessness are unclear. Increased breathlessness on exercise is known to occur at high altitude, but it is not known whether this is related to the hypoxia per se, or to other ventilatory parameters. To examine the role of high-altitude hypoxia in exercise-induced breathlessness, studies were performed in 10 healthy, normal subjects at sea level and after acute exposure to an altitude of 4450 m.

Are patients fed appropriately according to their caloric requirements?

Background: Specific morbidity related to underfeeding and overfeeding necessitates the design of nutrition support regimens that provide calories equal to those required on the basis of energy expenditure. This prospective multicenter trial was designed to determine what percent of patients in long-term acute care facilities receive feeding appropriate to their needs and whether accuracy of feeding has an impact on patient clinical status.

Methods: Patients on mechanical ventilation who were hospitalized at 32 Vencor Hospitals over a 9-week period and who were receiving only enteral nutrition by continuous infusion at a presumed goal rate were evaluated once by indirect calorimetry (IC) while on feeding.

Isocapnic hypoxemia and neuropsychological functioning.

We evaluated the cognitive effects of hypoxemia independent of hypocapnia in 20 right-handed male subjects using a battery of brief neuropsychological tests. Results of a profile analysis indicated that performance during hypoxia was reliably different for Digit Symbol and Finger Tapping tests. Trend analysis demonstrated a significant linear pattern for Finger Tapping results, such that lower levels of oxygen were associated with slower rates of tapping.

Safety and efficacy of using high-dose topical and nebulized anesthesia to obtain endobronchial cultures.

We evaluated the safety and efficacy of high-dose topical and nebulized airway anesthesia in normal volunteers and in patients undergoing diagnostic fiberoptic bronchoscopy. Lidocaine solution (4 percent) was used for gargling, for spraying the palate and oropharynx with an atomizer, and for nebulization with an air-powered nebulizer (mean total dose, 1,682 mg) and 2 percent lidocaine (Xylocaine) jelly for anesthetizing nasal passages. In six normal subjects and in eight patients, lidocaine blood levels were measured at baseline, after gargling, after spraying, after nebulization, and then at 5, 10, 15, 30, and 60 min; 19 normal subjects and ten patients underwent the same anesthesia protocol but had no blood drawn.

Biodegradation testing of radioactive waste forms.

Biodegradation tests were conducted on solidified waste forms containing ion exchange resins contaminated with high levels of radioactive nuclides. These tests were part of a program to test waste forms in accordance with the U.S.

The effects of hypoxemia on cardiac output. A dose-response curve.

To establish a dose-response curve for the effects of isocapnic hypoxemia on cardiac output (CO), we studied 20 healthy men, aged 20 to 34 years, using a tight-fitting face mask and an isocapnic partial rebreathing system (a modified anesthesia machine). We blended oxygen and hypoxic gas to achieve arterial oxygen saturations (SaO2) of 80, 85, and 90 percent; subjects also breathed 100 percent oxygen and room air (RA). Target SaO2 and end-tidal carbon dioxide were continuously monitored using an ear oximeter and CO2 gas analyzer.