Pulmonary Circulation最新文献

Exercise testing has long been essential for evaluating diagnosis, prognosis, and functional status in pulmonary hypertension (PH). Recent advances have clarified its role in defining reference values and prognostic markers. Nonetheless, substantial knowledge gaps persist regarding the implementation of invasive cardiopulmonary exercise testing (iCPET) and its potential to inform pathophysiology and therapeutic decision-making. This statement addresses the knowledge gaps that hinder the application of iCPET and exercise right heart catheterization (RHC) in the assessment of PH. We present research priorities and scenarios in which these tests may clarify drug mechanisms and support PH subphenotyping. An international, multidisciplinary task force of cardiology and pulmonology experts reviewed the literature and formulated consensus recommendations through iterative discussions.

Methamphetamine-associated pulmonary arterial hypertension (Meth-PAH) is an increasingly prevalent but understudied subtype of Group 1 pulmonary arterial hypertension (PAH). While most prevalent in the Western United States, its reach continues to expand with the evolving global methamphetamine epidemic. Despite its designation as a definite cause of PAH, there are no standardized diagnostic criteria or treatment guidelines specific to Meth-PAH. This review summarizes its epidemiology, proposed pathophysiology, clinical management, and treatment challenges. We outline a pragmatic approach to Meth-PAH, emphasizing structured screening for substance use, initiation of oral PAH-specific therapies regardless of abstinence, and therapy escalation based on adherence and serial risk assessment. Key knowledge gaps include Meth-PAH pathophysiology, performance of risk stratification tools, and the safety and efficacy of PAH therapies in patients with ongoing use. A multidisciplinary approach is needed to address both Meth-PAH and methamphetamine use disorder to improve outcomes in this high-risk, stigmatized population.

Noninvasive imaging markers may be helpful in identifying higher-risk patients with various lung diseases. Pulmonary artery to aorta ratio (PA/A ratio) on computed tomography (CT) is an indicator of pulmonary hypertension, but its relationship with other hemodynamic, imaging, and physiologic measurements, functional status, and outcomes requires further investigation. We set out to determine if the PA/A ratio is related to components of right ventricular (RV) afterload (pulmonary vascular resistance (PVR), PA compliance), lung diffusion capacity, radiographic emphysema, or honeycombing on chest CT, six-minute-walk distance, and transplant-free survival in COPD and/or interstitial lung disease (ILD). Data including PA/A ratio, hemodynamics, imaging, physiologic measurements, and survival in Redefining Pulmonary Hypertension through Pulmonary Vascular Disease Phenomics (PVDOMICS) cohort participants with COPD, ILD, or both were analyzed in regression and survival models to determine the association between PA/A ratio and various outcomes. We found that PA/A ratio analyzed as a continuous variable or dichotomized (> 0.9 vs. ≤ 0.9) was associated with higher PVR and lower PA compliance in fully adjusted models. Having either a PA/A ratio > 0.9 or a more elevated PVR was associated with worse transplant-free survival, while PA compliance was not associated with survival. PVR did not appear to mediate the relationship of PA/A ratio with survival. PA/A ratio did not correlate with six-minute walk distance or presence of emphysema or honeycombing, but was related to lower lung diffusion capacity. In conclusion, PA/A ratio on CT is related to aspects of RV afterload and to survival in COPD and ILD.

Catheter-directed intervention (CDI) in patients presenting with pulmonary embolism (PE) is suggested to improve in-hospital outcomes. We aimed to compare the effect of early versus late initiation of CDI on in-hospital outcomes. A systematic review and meta-analysis were conducted according to PRISMA guidelines. A comprehensive search of PubMed, Scopus, Web of Science, and Embase databases was performed from inception to May 2025. Data extraction was conducted independently by multiple reviewers. Statistical analysis was performed using R software version 4.3.2, and odds ratio (OR) and mean difference (MD) with 95% confidence intervals (CIs) were combined using a random-effects model. Six studies, including 53,472 patients, were included. Early intervention before 24 h significantly reduced mortality (OR 0.61, 95% CI [0.44-0.84]; I² = 0%). Moreover, Early intervention before 24 h significantly reduced the all-cause readmission in patients with acute PE (OR = 0.81 (95% CI: [0.73, 0.89]; I² = 0%). There was no significant difference in risk of major bleeding in early vs late intervention (OR = 0.82; 95% CI: [0.48, 1.39]; I² = 69.3%). Also, early intervention before 24 h significantly reduced the length of hospital stay (LOS) (MD = -3.11 days (95% CI: [-4.33, -1.89]; I² = 0%). This meta-analysis showed that early CDI, particularly within 24 h of presentation, is associated with a significant reduction in both mortality and all-cause readmission in patients with acute PE, without increasing the risk of bleeding. These findings support the concept of "time-is-myocardium" and reinforce the need for structured protocols that enable early therapeutic intervention. Further randomized controlled trials are needed to confirm these findings and to establish the optimal timing for this potentially life-saving therapy.

In this single-blind, crossover pilot study, supplemental oxygen at 2 L/min during 6-min walk test reduced Cardiac Effort in patients with pulmonary arterial hypertension, while 6-min walk distance remained unchanged. This decrease suggests improved physiology for an unchanged workload. Continuous electrocardiogram heart rate monitoring adds considerable value to the 6-min walk.

Intermediate-high-risk (IHR) pulmonary embolism (PE) represents a heterogeneous group in whom guideline-based criteria may insufficiently capture biologic and hemodynamic variability relevant to early deterioration. Data-driven phenotyping may improve risk stratification and support individualized decisions regarding reperfusion therapy. In this retrospective cohort study (2012-2025), 553 guideline-defined IHR PE patients were analyzed using unsupervised machine learning. Thirty-six demographic, clinical, laboratory, echocardiographic, and CT variables were standardized and encoded as appropriate for clustering. Multiple algorithms were compared, and the optimal model was selected using silhouette width and stability metrics. Clinical characteristics, imaging findings, treatment patterns, and outcomes were compared across phenotypes. The primary outcome was in-hospital mortality; secondary outcome was all-cause long-term mortality. Multivariable logistic regression and Cox models assessed associations with outcomes, and pre-post-treatment changes were evaluated. Two phenotypes were identified using the k-prototypes algorithm (silhouette width = 0.697). Cluster 1 (RV-failure phenotype; n = 360) exhibited younger age, lower systolic blood pressure, more severe RV dysfunction, higher thrombotic burden, and lower baseline TAPSE/PASP ratios. Cluster 2 (comorbidity-dominant phenotype; n = 193) comprised older patients with more cardiovascular/metabolic comorbidities but relatively preserved hemodynamics. In-hospital mortality was 6.0% overall and lower in Cluster 2 (3.6% vs. 7.2%); Cluster 2 remained independently associated with reduced early mortality (OR: 0.43; 95% CI: 0.19-0.98). The CDT-cluster interaction term was not statistically significant. Both phenotypes demonstrated significant improvements in RV function after reperfusion, with greater gains-including TAPSE/PASP-in Cluster 1. Over a median follow-up of 73.2 months, long-term mortality did not differ significantly between phenotypes (log-rank p = 0.11). Unsupervised ML revealed two clinically meaningful IHR PE phenotypes with divergent early risk but comparable long-term outcomes. These findings suggest that phenotype-based assessment may refine risk stratification and help guide individualized decisions regarding CDT and other reperfusion strategies in acute PE.

Pulmonary hypertension (PH) associated with left heart disease (LHD-PH) and pulmonary hypertension associated with chronic lung disease (CLD-PH) are the most common PH subtypes but lack effective treatments. In a prospective cohort study, 90 patients (39 LHD-PH, 51 CLD-PH) with severe disease [pulmonary vascular resistance (PVR) > 5 Wood units and recent right heart failure (RHF)] were treated with subcutaneous Treprostinil (scTRE). Clinical, echocardiographic, and hemodynamic parameters were assessed at baseline and at 12 months; survival was tracked for 5 years. In LHD-PH and CLD-PH patients at 12 months, 6-min walking distance (6MWD) improved by 88 m [64;112] and 52 m [22;89], respectively, World Health Organisation functional class (WHO FC) improved in 77% and 78% of patients and mean pulmonary artery pressure (mPAP) decreased by 5.78 mmHg and 9.05 mmHg, respectively. Tricuspid annular plane systolic excursion/systolic pulmonary artery pressure (TAPSE/sPAP) ratio improved in patients with CLD-PH. Median scTRE dose at 12 months was 30 ng/kg/min. Overall survival at 5 years was 38.6% in LHD-PH group and 49.7% in CLD-PH group. Patients with baseline pulmonary arterial wedge pressure (PAWP) < 12 mmHg had better outcomes. Open-label scTRE was associated with a spectrum of clinically relevant benefits in a select group of LHD-PH and CLD-PH patients recently hospitalized for RV failure and was well tolerated.

Vasoreactivity, which refers to the reduction of mean pulmonary arterial pressure in response to inhaled vasodilators, is a well-established metric for prognostication and treatment selection in patients with pulmonary arterial hypertension. However, the role of vasoreactivity in chronic thromboembolic pulmonary hypertension is less studied. We investigated whether vasoreactivity at time of diagnosis carries prognostic value in all patients with chronic thromboembolic pulmonary hypertension, and how it relates to the effect of long-term pulmonary vasodilator treatments. Patients diagnosed with CTEPH were prospectively subjected to 40 ppm inhaled nitric oxide testing at the diagnostic right heart catheterization. Classic (acute decrease of mean pulmonary arterial pressure > 10 mmHg to a level below 40 mmHg, n = 25), absolute (acute decrease of by 10 mmHg, n = 47) and percent (acute decrease by 10%, n = 129, all with maintained cardiac output) definitions of vasoreactivity were examined in retrospect. The relationship between each definition and transplantation-free survival was assessed with Cox regression models adjusted for baseline mPAP, age, sex, and WHO functional class. Patients (n = 325) were observed over a median of 5.5 years (interquartile range 2.6-9.2). Vasoreactivity, by the percent definition, was associated with significantly improved adjusted 5-year transplantation-free survival (hazard ratio = 0.61, 95% confidence interval 0.38-0.97, p = 0.036). Among not operated patients (n = 174, 53.5%), vasodilator PH medications were associated with improved survival in vasoresponders (hazard ratio = 0.46, 95% confidence interval 0.22-0.96, p = 0.04), but had no impact on survival in non-vasoresponders (hazard ratio = 0.67, 95% confidence interval 0.36-1.24, p = 0.20). Vasoreactivity at baseline catheterization carries prognostic value in CTEPH.

Considering the beta-3 adrenoceptor agonist, mirabegron, may display vasodilator and cardioprotective properties, we investigated the therapeutic potential of mirabegron in monocrotaline-induced pulmonary hypertension in rats. High-dose mirabegron improved right ventricular function, and endothelium-dependent relaxation in isolated rat pulmonary arteries, suggesting that mirabegron may be beneficial in pulmonary arterial hypertension.

Early graft dysfunction following lung transplantation is commonly attributed to primary graft dysfunction (PGD). However, other rare etiologies may present with similar clinical and radiologic features. We report a unique case of a 58-year-old male with idiopathic pulmonary fibrosis who underwent bilateral lung transplantation. The donor was a young adult with traumatic brain injury. Within hours post-transplant, the recipient developed severe hypoxemia and diffuse infiltrates on chest imaging, consistent with Grade 3 PGD. Despite supportive therapy, there was limited improvement. Histopathological examination of transbronchial biopsies revealed cerebral cortical neurons and glial tissue embedded in the pulmonary vasculature, confirming cerebral tissue embolism. This case highlights cerebral tissue pulmonary embolism as an under-recognized cause of early allograft dysfunction. Clinicians should consider donor-derived embolic events, particularly in trauma-related donors, in the differential diagnosis of PGD-like presentations.