Pulmonary Circulation最新文献

Leveraging the potential of virtual platforms in the post-COVID-19 era, the Infection and Pulmonary Vascular Diseases Consortium (iPVDc), with the support of the Pulmonary Vascular Research Institute (PVRI), launched a globally accessible educational program to highlight top-notch research on inflammation and infectious diseases affecting the lung vasculature. This innovative virtual series has already successfully brought together distinguished investigators across five continents - Asia, Europe, South and North America, and Africa. Moreover, these open global forums have contributed to a comprehensive understanding of the complex interplay among immunology, inflammation, infection, and cardiopulmonary health, especially concerning pulmonary hypertension and related pulmonary disorders. These enlightening discussions have not only heightened awareness about the impact of various pathogenic microorganisms, including fungi, parasites, and viruses, on the onset and development of pulmonary vascular diseases but have also cast a spotlight on co-infections and neglected illnesses like schistosomiasis - a disease that continues to impose a heavy socioeconomic burden in numerous regions worldwide. Thus, the overall goal of this review article is to present the most recent breakthroughs from infectious PVDs as well as bring to light the scientific and educational insights from the 2023 iPVDc/PVRI virtual symposium series, shaping our understanding of these crucial health issues in this more than ever interconnected world.

Delta-like ligand 4 (DLL-4) inhibitor drugs are an emerging cancer treatment. In clinical trials for solid organ malignancies, intravenous administration of monoclonal antibodies that inhibit DLL-4 is associated with development of pulmonary hypertension, in the absence of left ventricular dysfunction. Analysis of 13 clinical trials showed that pulmonary hypertension is a complication of DLL-4 inhibition.

Current methods for quantifying perfusion from computed tomography pulmonary angiography (CTPA) often rely on semi-quantitative scoring systems and requires an experienced evaluator. Few studies report on absolute quantitative variables derived from the images, and the methods are varied with mixed results. Dual-energy CTPA (DE-CTPA) enables automatic quantification of lung and lobar perfusion with minimal user interaction by utilizing machine learning based software. We aimed to evaluate differences in DE-CTPA derived quantitative perfusion variables between patients with acute pulmonary embolism (PE) and chronic thromboembolic pulmonary hypertension (CTEPH). This retrospective, single-center, observational study included 162 adult patients diagnosed with PE (n = 81) or CTEPH (n = 81) and scanned using dual-energy CT between 2020 and 2023. Mann-Whitney U tests and permutational analysis of variance (PERMANOVA) were used for comparative analyses. We found whole lung perfusion blood volume to be lower (p < 0.001) in PE patients (median 3399 mL [2554, 4284]) than in CTEPH patients (median 4094 mL [3397, 4818]). The same was observed at single lung and lobar level. PERMANOVA encompassing all perfusion variables showed a difference between the two groups (F-statistic = 13.3, p = 0.002). Utilizing logistic regression, right and left lower lobe perfusion blood volume showed some ability to differentiate between PE and CTEPH with area under the receiver operation characteristics curve values of 0.71 (95% CI: 0.56; 0.84) and 0.72 (95% CI: 0.56; 0.86). Pulmonary perfusion is lower in patients with PE than patients with CTEPH, highlighted by differences in DECT-derived perfusion blood volume. Quantitative perfusion variables might be useful to differentiate between the two diseases.

Current risk assessment of pulmonary embolism (PE) stratifies patients based on hemodynamic stability, clinical parameters of severity, right ventricular dysfunction and cardiac injury but fails to integrate a wide variety of comorbid conditions. The Charlson Comorbidity Index (CCI) predicts mortality based on patients' diseases and provides a system to quantify disease burden. The National Inpatient Sample (NIS) database (2016-2018) was used to identify patients with PE and calculate CCI score groups of 0, 1-2, 3-5, and ≥6 and stratify them by outcome. Of 561,625 patients with PE, 176,460 (31.4%) had CCI score of 0, 223,870 (39.8%) had CCI of 1-2, 102,305 (18.2%) had CCI of 3-5, and 58,990 (10.5%) had CCI ≥ 6. Higher CCI scores were associated with increased mortality: CCI 1-2 (adjusted odds ratio [aOR] 2.09), CCI 3-5 (aOR 3.12), CCI ≥ 6 (aOR 5.44) compared to CCI 0, along with stepwise increases in shock and mechanical ventilation with each increase in CCI score group. CCI scores ≥3 had increased length of stay (1.4-1.72 days) and increased total hospital costs ($3651-$4265) compared to CCI0. Patients with CCI ≥ 3 were less likely to receive systemic thrombolysis, catheter directed thrombolysis and mechanical thrombectomy. Acute PE in patients with elevated comorbidity scores is associated with higher morbidity and mortality, increased hospital resource utilization, and decreased usage of advanced therapies in a large cohort reflective of patients across the United States. Integration of comorbidities in risk assessment profiles identifies patients with higher short-term mortality which may guide management strategy.

The discrimination between pre and postcapillary exercise-induced pulmonary hypertension relies on accurate measurement of pulmonary capillary wedge pressure, which can be unreliable. We found that exercise pulmonary artery compliance and right atrial pressure (AUC 0.88, 0.89, respectively) can differentiate subtypes of exercise-induced pulmonary hypertension in the absence of wedge pressure.

Pediatric pulmonary arterial hypertension (PAH) can present with a wide spectrum of disease severity. Pulmonary hypertension (PH) crises can lead to acute decompensation requiring extracorporeal membrane oxygenation (ECMO) support, including extracorporeal cardiopulmonary resuscitation (eCPR). We evaluated outcomes for pediatric PH patients requiring ECMO. A single-institution retrospective review of pediatric PAH patients with World Symposium on PH (WSPH) groups 1 and 3 requiring ECMO cannulation from 2010 through 2022 (n = 20) was performed. Primary outcome was survival to hospital discharge. Secondary outcomes were survival to decannulation and 1-year survival. Of 20 ECMO patients, 16 (80%) survived to decannulation and 8 (40%) survived to discharge and 1 year follow up. Of three patients who had two ECMO runs; none survived. There were five patients who had eCPR for the first run; one survived to discharge. The univariate logistic regression model showed that venovenous ECMO was associated with better survival to hospital discharge than venoarterial ECMO, (OR: 0.12, 95% CI: 0.01-0.86, p = 0.046). PH medications (administered before, during, or after ECMO) were not associated with survival to discharge. For children with decompensated PAH requiring ECMO, mortality rate is high, and management is challenging. While VA ECMO is the main configuration for decompensated PH, VV ECMO could be considered if there is adequate ventricular function, presence of a systemic to pulmonary shunt, or an intercurrent treatable illness to improve survival to discharge. A multidisciplinary approach with requisite expertise should be utilized on a case-by-case basis until more reliable data is available to predict outcomes.

Pulmonary arterial hypertension (PAH) is a severe disease caused by progressive distal pulmonary artery obstruction. One cause of PAH are loss-of-function mutations in the potassium channel subfamily K member 3 (KCNK3). KCNK3 encodes a two-pore domain potassium channel, which is crucial for pulmonary circulation homeostasis. However, our understanding of the pathophysiological mechanisms underlying KCNK3 dysfunction in PAH is still incomplete. Taking advantage of unique Kcnk3-deficient rats, we analyzed the transcriptomic changes in the lungs from homozygous Kcnk3-deficient rats and wild-type (WT) littermates and compared them to PAH patient transcriptomic data. Transcriptome analysis of lung tissue obtained from WT and Kcnk3-deficient rats identified 1915 down- or upregulated genes. In addition, despite limited similarities at the gene level, we found a strong common signature at the pathway level in PAH patients and Kcnk3-deficient rat lungs, especially for immune response. Using the dysregulated genes involved in the immune response, we identified Spleen Associated Tyrosine Kinase (SYK), a significantly downregulated gene in human PAH patients and Kcnk3-deficient rats, as a hub gene. Our data suggests that the altered immune system response observed in PAH patients may be partly explained by KCNK3 dysfunction through the alteration of SYK expression.

Right ventricle-pulmonary artery (RV-PA) coupling describes the energetic relationship between RV contractility and its afterload. The gold standard for assessment of this relationship requires invasive pressure-volume (PV) loop measurements. Non-invasive surrogates of RV-PA coupling have been developed, such as the echocardiographic tricuspid annular plane systolic excursion to pulmonary artery systolic pressure ratio (TAPSE/PASP), but their performance has not been systematically assessed. We sought to assess performance of TAPSE/PASP ratio compared to PV loop-defined RV-PA coupling. A systematic search was conducted. Studies were included if PV loop derived RV-PA coupling metrics were compared to echocardiographic or magnetic resonance imaging surrogates. We conducted a meta-analysis of TAPSE/PASP correlation to PV loop-defined RV-PA coupling. 1452 studies were identified in the initial search, of which ten met inclusion criteria. Five studies allowed for pooled analysis of TAPSE/PASP to Ees/Ea correlation (r = 0.52, 95% confidence interval 0.36-0.65). There was moderate heterogeneity across the pooled studies. Despite the common use of Non-invasive surrogates of RV-PA coupling, there is only moderate correlation with gold standard measurements. These metrics do not inform on the individual components of RV-PA coupling, limiting their use in the management of patients with RV dysfunction.

The objective of this analysis was to compare clinician-based and formally calculated risk assessments by REVEAL Lite 2 and COMPERA 2.0 and to characterize parenteral prostacyclin utilization within 90 days of baseline in high-risk patients. A multisite, double-blind, retrospective chart review of patients with pulmonary arterial hypertension (PAH) was conducted with an index period of January 2014-March 2017. Patients were categorized into the "any PAH medication" or "prostacyclin-enriched" cohort based on latest PAH medication initiated within the index period. Clinicians classified the patient's 1-year mortality risk as "low," "intermediate," or "high" based on their clinical assessment. REVEAL Lite 2 and COMPERA 2.0 scores were independently calculated. Risk assessment congruency was evaluated. Parenteral prostacyclin use was evaluated within 90 days of baseline. Thirty-two clinicians participated and abstracted data for 299 patients with PAH. At baseline, mean patient age was 52 years, 6-min walk distance was 226 m, and most patients were WHO functional class II or III. Half of the patients (53%) were classified by clinician assessment as intermediate risk, while most were classified as high risk by REVEAL Lite 2 (59%) and intermediate-high risk by COMPERA 2.0 (52%). Parenteral prostascyclins were underutilized in high-risk patients, and not initiated in a timely fashion. Clinician-assessed risk category was incongruent with tool-based risk assessments in 40%-54% of patients with PAH, suggesting an underestimation of the patient's risk category by clinician gestalt. Additionally, there was a lack of timely prostacyclin initiation for patients with PAH stratified as high-risk by either tool.