Pulmonary Circulation最新文献

Eisenmenger syndrome represents a complex and severe form of pulmonary arterial hypertension (PAH) associated with congenital heart disease. Sotatercept, a first-in-class activin-signaling inhibitor, offers a novel therapeutic approach in the treatment of PAH, and has emerged as a promising therapeutic agent. However, patients with Eisenmenger syndrome were not included in its pivotal trials. Therefore, data regarding its effect in this patient cohort is lacking. We present two cases of patients with Eisenmenger syndrome treated with sotatercept. Sotatercept improved the subjective and objective exercise capacity in both patients without any significant adverse side effects.

Accurate resting oxygen consumption (rVO₂) quantification is critical for Fick-derived cardiac output calculations. Yet, clinical practice predominantly uses empirical estimations, which can be inaccurate. We evaluated the established rVO₂ prediction equations against direct metabolic cart measurements in systemic sclerosis patients, revealing significant discordances with potential diagnostic implications.

Patients with pulmonary arterial hypertension (PAH) experience long delays in diagnosis; however, little is known about the time between diagnosis and initiation of guideline-recommended optimal treatment. Immediate therapy is imperative for optimizing patient outcomes, as much of the pulmonary vasculature has already deteriorated by the time of diagnosis. This study used real-world U.S. data to investigate the time from the most recent right heart catheterization (RHC) to initiation of the first PAH-specific treatment. Adults with PAH were identified in the Komodo Health database using claims for RHC, pulmonary hypertension diagnostic codes, and PAH medications (1/1/2016-4/1/2024). Time from RHC to medication dispensing was examined and categorized by initial treatment regimen (monotherapy, dual therapy, and triple therapy). Baseline characteristics and treatment patterns were assessed. Among 7952 patients, the median time from most recent RHC to first PAH medication was 43.0 days. Most patients (77.7%) initially received monotherapy, with 20.0% and 2.3% receiving upfront dual and triple therapy, respectively. Cardiac comorbidities were present in most patients (85.8%). Among 2965 (48.0%) upfront monotherapy users who added another treatment during follow-up, the median time to dual therapy was 39 days. Of those who escalated to dual therapy, 567 (19.1%) eventually escalated to triple therapy, with a median time from dual therapy to triple therapy of 62 days. In real-world practice, delays between PAH diagnosis and initiation of optimal therapy remain significant, and barriers to access should be addressed.

Remote 6-min walk testing (6MWT) has been shown to be safe in pulmonary hypertension, but limited data exist on its longitudinal durability and relationship to supervised in-clinic testing. Cardiac Effort (CE), defined as total heartbeats divided by walk distance, provides physiologic context beyond distance alone. We evaluated the longitudinal performance of a home-based 6MWT and CE performed on a modified indoor walking space using a wearable chest sensor and compared results with in-clinic testing over 8 weeks. This single-center prospective study was conducted with institutional review board approval. Participants with PH performed supervised in-clinic and unsupervised home 6MWTs at baseline and Week 8. A chest-based wearable sensor recorded acceleration and heart rate data to estimate walk distance and calculate CE. Mixed-effects repeated-measures models were used to compare sensor-estimated and directly observed walk distance and CE in the clinic and at home over time. Agreement was assessed using Bland-Altman analyses, intraclass correlation coefficients (ICC), and Spearman correlations. Twenty-nine participants were enrolled, and no adverse events occurred. Sensor-estimated 6MWT distance and CE demonstrated minimal bias and excellent agreement with directly observed in-clinic measurements (6MWD bias 0.04%, ICC 0.995; CE bias -0.04%, ICC 0.995). Home-based 6MWD was approximately 40 m lower than supervised clinic testing. Mixed-effects models showed similar longitudinal changes between home and clinic testing. Changes in home 6MWD and CE correlated with corresponding in-clinic changes (6MWD: r = 0.40, p = 0.045; CE: r = 0.42, p = 0.03). Home-based 6MWT and CE obtained using a wearable chest sensor provide a safe, feasible method for remote functional assessment in PH that tracks with in-clinic testing. Frequent home assessment may facilitate earlier detection of clinical change, support therapeutic titration, and expand access to standardized physiologic monitoring.

Pulmonary arterial hypertension (PAH) is a rare and progressive disease resulting in increased workload of the right ventricle (RV). Despite advances in therapies, PAH remains highly morbid from a progressive vasculopathy and RV failure. For patients admitted to hospital with RV failure, transplant remains the only option for a select few. Sotatercept, a novel activin signaling inhibitor approved for the treatment of PAH, has demonstrated significant improvement in clinical outcomes across a wide spectrum of disease severity. However, descriptions of its use to treat acute RV failure in an intensive care unit (ICU) setting are limited. We report a case of cardiogenic shock and respiratory failure secondary to decompensated PAH requiring veno-arterial extracorporeal membrane oxygenation (VA ECMO), with rapid improvement following sotatercept initiation.

This study analyzed the US Open Payments and Medicare Part D databases from 2014 to 2022 to assess associations between industry-physician payments and PAH therapy prescribing. Among 5502 pulmonologist-, cardiologist- and pediatrician-prescribers of PAH therapies, drug-specific industry payments were associated with dose-response increases in Medicare spending.

The Pulmonary Hypertension (PH) Global Patient Survey provides the first truly international description of the symptomatic burden of PH and its treatment on patient's health-related quality of life (HRQoL). From 3329 adult responses across 88 countries, the "invisible" nature of PH is an over-arching theme, with repercussions on socialisation, relationships and employment. Emotional burden is common with 35.6% feeling misunderstood, 33.4% angry or frustrated and 32.0% isolated. A reduced capacity for paid employment, in combination with healthcare/medication costs and difficulty in accessing social and financial support, compound societal exclusion and isolation. Physical limitations are almost universally reported (78.6%) with poor sleep quality (42.3%) the greatest factor in physical well-being. Treatment side-effects are common (46.7%), are greatest with parenteral therapies, but improve with age. Women of child-bearing age bear the brunt of PH physical and psychological burden, compounded by inconsistent messages from specialist services surrounding pregnancy and contraception. Patient-reported outcome measures (PROM) accurately reflect impacts from PH but are underutilised in clinical practice. Many respondents would like to be better able to discuss their emotional symptoms with their PH specialist (but often find a lack of empathy). Psychological support is a highly requested, but frequently unmet, need reinforcing the importance of holistic care models and multiagency working in managing patients with PH.

The Pulmonary Hypertension Global Patient Survey (PH GPS) provides the first international examination of experiences among children with pulmonary hypertension (PH) and their caregivers. Through an online survey distributed via PH associations across 32 countries, we collected responses from 136 caregivers about diagnostic journeys, treatment experiences, healthcare access and research participation. Qualitative analysis revealed challenges around the 'invisible' nature of PH, with caregivers describing how schools and communities sometimes doubted their child's limitations in the absence of visible symptoms, adding to family burden. Quantitative findings demonstrated extensive caregiver burden with 40% providing constant care while facing significant employment consequences, including leaving work altogether, reduced hours, or career changes. Although 78.8% of children accessed specialist centres, significant barriers persisted: 41.2% encountered diagnostic delays exceeding 6 months, 34.8% travelled over 2 h for appointments and 27.7% received no healthcare cost reimbursement. Treatment experiences revealed 37.7% of children experienced side effects, yet only one-third discussed changes with their clinical teams, reflecting pragmatic decisions within limited therapeutic options. Research participation was low (19.2%), though families with trial experience showed greater willingness for future participation, compared to those without experience (70.8% vs. 49.5%). Barriers centred on awareness gaps (16.4%), safety concerns (14.5%), and age-related considerations (10.9%), distinct from adult PH populations where logistical challenges predominate. These findings demonstrate that paediatric care must extend beyond medical management to address family needs including social-work assistance for benefits navigation, flexible care delivery models incorporating telemedicine, pro-active communication about treatment tolerability and research protocols designed with families from inception.

In this first global study of patient perspectives in Group 2 and 3 PH, we demonstrate that those with Group 3 PH report significantly greater adverse impacts on physical and mental wellbeing than those in Group 2 or Group 1, despite a lower treatment side-effect profile.

To investigate the prognostic accuracy of three-dimensional echocardiographic (3DE) right ventricular (RV) data and compare it with that of risk stratification based on 2015 ESC Guidelines in pre-capillary pulmonary hypertension (PcPH) patients. We prospectively enrolled PcPH patients from March 2017 to May 2018. 3DE sequences were analyzed by semi-automatic software (TomTec 4D RV-Function 2.0). RV end-diastolic volume (EDV), end-systolic volume (ESV), ejection fraction, longitudinal strain of septum and free wall, tricuspid annular plane systolic excursion were obtained. All participants were classified into low and intermediate-high risk groups based on 2015 ESC Guidelines. Patients were followed-up till May 2019 for death due to RV failure as an end-point. We finally enrolled 112 PcPH patients (average 36 years, 39 males and 73 females) in our study. Mean follow-up time was 18 months, and 11 patients died. Receive operating characteristic curves identified RV-3D-EDV = 150 mL and RV-3D-ESV = 109 mL as optimal cut-offs. Multivariate Cox proportional regression analyses indicated RV-3D-EDV > 150 mL and RV-3D-ESV > 109 mL were independent predictors of mortality after adjusted by Risk stratification. McNemar-Bowker test revealed that compared with risk stratification, RV-3D-EDV > 150 mL (67.3% vs. 44.6%, p < 0.01) and RV-3D-ESV > 109 mL (62.4% vs. 44.6%, p < 0.01) had better predictive specificities for end-point. RV volumes detected by three-dimensional echocardiography suggested potential prognostic value for risk stratification in PH patients, warranting validation in larger cohorts.