Pulmonary Circulation最新文献

Landmark trials of sotatercept in pulmonary arterial hypertension (PAH) excluded patients with significant cardiopulmonary comorbidities. To evaluate the real-world effectiveness and safety of sotatercept in patients with Group I PAH and cardiopulmonary comorbidities. We conducted a single-center prospective observational study of adults with PAH on stable background therapy who initiated sotatercept between August 2024 and April 2025. Clinical, echocardiographic, and adverse event data were collected at baseline, 3 months, and 6 months. Forty-five patients (mean age 59.1 years old; 88.8% female) were included. Common comorbidities include CAD (44.7%), HTN (40.4%), ILD (23.4%), and COPD (12.7%). Most patients were on triple background pulmonary vasodilator therapy (63.8%). At 6-month follow-up, oxygen requirement at rest improved from 4.64 to 3.20 L/min (p = 0.001), 6MWD increased by 20 meters (310.5 ± 142.4 m to 330.4 ± 132.3 m, p = 0.01). REVEAL Lite 2 scores improved, with over half the cohort achieving or maintaining low risk status. Adverse effects were mild, including epistaxis and increased hematocrit. Sotatercept appears safe and clinically beneficial in PAH patients with cardiopulmonary comorbidities.

Pulmonary arterial hypertension (PAH) is a progressive disease with significant morbidity and mortality. Due to nonspecific symptoms, diagnosis can be challenging and subject to substantial delays. Using data from Mayo Clinic's electronic health records, we looked at causes of delayed diagnosis and whether earlier diagnosis means better outcomes. This retrospective cohort study included adults with PAH confirmed by right heart catheterization (RHC) between 2015 and 2019. Univariate and multivariate analyses evaluated the association of 229 clinical and laboratory features with time to PAH diagnosis and survival outcomes. Early diagnosis was defined as < 6 months and a delayed diagnosis as ≥ 22.2 months from an eligible event. Survival probability was determined using Kaplan-Meier analysis. The study enrolled 160 patients. The most common initial PAH symptoms were dyspnea (42.5%) and fatigue (16.9%). It took a median of 7, 13, 26, and 123 days from eligible event to the first X-ray, electrocardiogram, echocardiogram, and RHC, respectively. Factors most closely associated with delayed diagnosis were chronic obstructive pulmonary disease, normal creatinine, high systolic blood pressure, acute respiratory infection, normal iron, normal diastolic blood pressure, and being male aged 18.5 to < 25 years. Compared with delayed diagnosis patients (22.6%), more early diagnosis patients saw a pulmonologist (35.3%) before another provider. After 5 years, 73% of patients in the early diagnosis and 31% in the delayed diagnosis group were still alive. This study identified factors associated with a delayed diagnosis of PAH and found that earlier diagnosis and treatment initiation were associated with significantly improved survival.

This study quantified prior authorization (PA)-insurance-required approval-burden for pediatric pulmonary hypertension (PH) at an accredited center. Among 53 patients, 72% of 283 prescriptions between 2021 and 2023 required PA, with non-FDA-approved medications showing highest volume. Despite 97% approval and all patients ultimately obtaining medication, delays (mean 4.2 days), repeat requests (39%), and reliance on patient assistance programs created significant administrative burden. Results highlight the need for policy reform to ensure timely, equitable access to essential therapies.

Assessing operability in late-presenting ventricular septal defects (VSD) with pulmonary arterial hypertension (PAH) is complex. Although a comprehensive assessment is often recommended, pulmonary vascular resistance index (PVRI) is the cornerstone of most decisions. PVRI-based operability assessment, nonetheless, is not infallible. Cases with PVRI much higher than operability cut-offs have done well, while some with lower PVRI have experienced late recurrence of PAH. We propose the Diastolic Pulmonary Shunt Index (DiPSI), defined as the diastolic pulmonary gradient (DPG = PADP - PAWP) divided by Qp/Qs, as an adjunct to aid clinical judgment. DiPSI, by offering a physiologically grounded, flow-adjusted index, may help clarify borderline cases, providing a more nuanced assessment of operability in late presenters with large VSD than PVRI alone.

Transition from parenteral prostanoids to oral selexipag may be considered in select patients with pulmonary arterial hypertension (PAH) to reduce the therapeutic burden imposed on patients and caregivers, but its safety and efficacy remain uncertain. A retrospective cohort study was conducted involving adult patients with PAH who transitioned from parenteral prostanoids to selexipag at Canadian tertiary referral centers between January 2016 and November 2020. The primary outcome was transition failure at 12 months, defined as a composite of death or PAH-related worsening. Baseline predictors of transition failure were identified using univariate Cox regression, with follow-up data collected up to 36 months post-transition or until site-specific data submission, occurring between May 2021 and February 2023. At 12 months post-transition, 11 of 36 patients (31%) experienced transition failure, including 4 deaths, all classified as high-risk pre-transition. Of the 25 patients (69%) who successfully transitioned, only one (4%) experienced transition failure over the subsequent observation period (median follow-up 28 months). Pre-transition variables protective against transition failure included a right atrial pressure (RAP) < 8 mmHg (HR 0.290, 95% CI 0.084-0.999, p = 0.049), absence of hospitalizations in the year preceding transition (HR 0.239, 95% CI 0.064-0.885, p = 0.032), lower brain natriuretic peptide (< 50 pg/mL)/N-terminal pro-B-type natriuretic peptide (< 300 pg/mL) levels (HR 0.174, 95% CI 0.045-0.676, p = 0.011), and REVEAL 2.0 score < 7 (HR 0.162, 95% CI 0.049-0.541, p = 0.003). These findings suggest that transition from parenteral prostanoids to selexipag carries the risk of clinical deterioration and should only be considered in carefully selected, low-risk patients.

Pulmonary hypertension (PH) significantly impacts outcomes in heart failure with preserved ejection fraction (HFpEF), especially with right ventricular dysfunction (RVD), yet effective treatments are limited. This study retrospectively evaluated sacubitril-valsartan's effects on pulmonary pressures, RV function, and clinical outcomes in 20 severe HFpEF-PH patients. Significant improvements were observed over a median 397-day follow-up. Sacubitril-valsartan notably reduced RV systolic pressure (RVSP) by 27.50 mmHg (p < 0.0001) and peak tricuspid regurgitation (TR) velocity by 0.825 m/s (p < 0.0001). TR severity improved significantly (p = 0.0426). RV dimensions decreased significantly (basal: p = 0.0083; mid-cavity: p = 0.0025). RV-pulmonary arterial coupling (TAPSE/RVSP) improved from 0.27 to 0.47 (p < 0.0001), and NYHA functional class improved from mean III to II (p = 0.0164). Systolic blood pressure also decreased by 24.0 mmHg (p < 0.0001). Diastolic function parameters (E velocity, E/e', LAVI) did not show significant changes. These findings suggest that sacubitril-valsartan could improve pulmonary pressures, RV dimensions, RV-PA coupling, TR severity, and functional status in HFpEF-PH patients, supporting its potential therapeutic role.