Pulmonary Circulation最新文献_第2页

Pulmonary Embolism as a Cause of Death in Lung Transplant Recipients: Data From a Nationwide Registry.

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-03-11 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70056

Hye Sung Kim, Rohit Gupta, Parth Rali

引用次数: 0

Developing Benchmarks in the Diagnosis and Treatment of Pulmonary Arterial Hypertension in a Tertiary, Academic Medical Center. 在一家三级学术医疗中心制定肺动脉高压诊断和治疗基准。

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-03-03 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70063

Cyrus A Kholdani, Justin H Lee, Kai E Swenson, Juliana Liu, Andrew Hsi, Kristina T Kudelko, Andrew J Sweatt, Edda F Spiekerkoetter, Vinicio De Jesus Perez, Joseph Rigdon, Haley Hedlin, Adam M Andruska, Raquel L Lyn, Roham T Zamanian, Yon K Sung

{"title":"Developing Benchmarks in the Diagnosis and Treatment of Pulmonary Arterial Hypertension in a Tertiary, Academic Medical Center.","authors":"Cyrus A Kholdani, Justin H Lee, Kai E Swenson, Juliana Liu, Andrew Hsi, Kristina T Kudelko, Andrew J Sweatt, Edda F Spiekerkoetter, Vinicio De Jesus Perez, Joseph Rigdon, Haley Hedlin, Adam M Andruska, Raquel L Lyn, Roham T Zamanian, Yon K Sung","doi":"10.1002/pul2.70063","DOIUrl":"10.1002/pul2.70063","url":null,"abstract":"Benchmarks of clinical management are essential for improving the quality of care. However, the lack of established quality metrics for pulmonary arterial hypertension (PAH) contributes to practice heterogeneity. We assessed our center's diagnostic practices, therapeutic practices, and risk-adjusted survival patterns over time for the purpose of establishing quality benchmarks. We analyzed the demographics, clinical characteristics, and diagnostic evaluation of 702 PAH patients enrolled between 1999 and 2019. We examined outcomes in this cohort, including an analysis of risk stratification, therapeutic practice patterns, hospitalizations, organ transplant, and survival. Initial diagnostic workup of incident PAH cases demonstrated excellent completion of echocardiographic (99%) and pulmonary function testing (91%), with improved completion of VQ scanning over the study time period (90% between 2015 and 2019). Right heart catheterization (RHC) was performed in all patients; RHC performed at our center was more likely to include complete hemodynamic data than those performed at referring institutions (55.4% and 30.4% respectively). The average number of PAH-specific medications prescribed increased over time; however, there was no significant increase in the use of parenteral therapy over time, even when stratified by the REVEAL risk score. Survival rates in the cohort were 94% at 1 year, 75% at 5 years, and 60% at 10 years, comparable to those of other PAH cohorts. Analysis of our well-characterized cohort of PAH patients reveals the extent to which guideline-directed diagnostic and therapeutic care is delivered at our specialty center, and the associated outcomes; these data may serve as a benchmark for continued improvements in quality of PAH care.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 1","pages":"e70063"},"PeriodicalIF":2.2,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11875057/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143543210","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Differences in Direct Fick and Thermodilution Measurements of Cardiac Output: Impact on Pulmonary Hypertension Classification.

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-02-26 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70053

Garry W Hamilton, Luke R Fletcher, William Harley, Robert Azzopardi, R Kimberly Chan, Jordan Fulcher, Lachlan F Miles, Omar Farouque, Mark C G Horrigan

{"title":"Differences in Direct Fick and Thermodilution Measurements of Cardiac Output: Impact on Pulmonary Hypertension Classification.","authors":"Garry W Hamilton, Luke R Fletcher, William Harley, Robert Azzopardi, R Kimberly Chan, Jordan Fulcher, Lachlan F Miles, Omar Farouque, Mark C G Horrigan","doi":"10.1002/pul2.70053","DOIUrl":"10.1002/pul2.70053","url":null,"abstract":"Direct Fick (DF) and bolus thermodilution (TD) are endorsed by pulmonary hypertension (PH) guidelines to measure cardiac output. In contemporary practice, agreement between methods is unknown, as are the diagnostic consequences of disagreement. We sought to evaluate the frequency and degree of disagreements between cardiac output measurement techniques and assess their impact on the hemodynamic assessment of PH. This was a single-center study that included 182 patients who had cardiac output concurrently measured by DF and TD. Oxygen consumption was measured by indirect calorimetry. Agreement between DF and bolus TD cardiac output was assessed using Bland-Altman analysis. The median DF and TD cardiac outputs were 5.42 L/min (interquartile range [IQR] 3.90-7.41) and 4.10 L/min (IQR 3.47-5.10), respectively. Significant disagreement was observed with DF yielding higher cardiac output results than TD. Mean error was proportional to cardiac output (-3.75% at 3 L/min to +44.5% at 7 L/min), and limits of agreement were wide. Disagreement was increased by 19.2% in the presence of least moderate tricuspid regurgitation and by 16.0% in patients with atrial fibrillation. Among 152 patients with PH, hemodynamic classification discordance occurred in 18 (11.8%) patients. Disagreement between DF and TD was observed, which resulted in a discrepant hemodynamic classification in approximately 12% of patients. These techniques should, therefore, not be used interchangeably for serial surveillance, and without a clinical gold standard, a rationale exists for utilizing both methods concurrently in certain clinical situations.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 1","pages":"e70053"},"PeriodicalIF":2.2,"publicationDate":"2025-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11865337/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143524178","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

How a Most Unlikely Drug Changed the Outcome of Pulmonary Arterial Hypertension.

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-02-26 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70059

Ivan M Robbins, Anna R Hemnes

{"title":"How a Most Unlikely Drug Changed the Outcome of Pulmonary Arterial Hypertension.","authors":"Ivan M Robbins, Anna R Hemnes","doi":"10.1002/pul2.70059","DOIUrl":"10.1002/pul2.70059","url":null,"abstract":"Epoprostenol is a prostaglandin that was first identified by investigators in 1976 and found to be a potent vasodilator and inhibiter of platelet aggregation, initially in animal studies and subsequently in humans. Based on these properties epoprostenol, which has a short half-life of several minutes in vivo requiring continuous intravenous infusion, was administered to patients with pulmonary arterial hypertension (PAH) and found to decrease pulmonary vascular resistance (PVR) during acute vasodilator testing. Investigators then studied the effects of long-term infusion of epoprostenol and observed sustained hemodynamic and clinical improvement, albeit with persistent elevation of pulmonary artery pressure and PVR. The results of a randomized multicenter study confirmed the benefit of epoprostenol, including improved survival, in patients with PAH leading to FDA approval in 1995. Before this, there was no effective therapy for the vast majority of patients with PAH. A number of other less complicated and safer medications have subsequently been approved for the treatment of patients with PAH. However, nearly 30 years after approval, despite the complexity and risks of continuous intravenous therapy, epoprostenol remains the most effective treatment for PAH.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 1","pages":"e70059"},"PeriodicalIF":2.2,"publicationDate":"2025-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11862565/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143516532","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Echocardiographic Grading of Right Ventricular Afterload in Left Heart Disease: Relation to Right Ventricular Function, Pulsatile and Resistant Load, and Outcome.

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-02-20 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70055

Bettia Edith Celestin

引用次数: 0

An Unsupervised Approach to Derive Right Ventricular Pressure-Volume Loop Phenotypes in Pulmonary Hypertension. 推导肺动脉高压右心室压力-容积环路表型的无监督方法

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-02-20 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70057

Nikita Sivakumar, Cindy Zhang, Connie Chang-Chien, Pan Gu, Yikun Li, Yi Yang, Darin Rosen, Tijana Tuhy, Ilton M Cubero Salazar, Matthew Kauffman, Rachel L Damico, Casey Overby Taylor, Joseph L Greenstein, Steven Hsu, Paul M Hassoun, Catherine E Simpson

{"title":"An Unsupervised Approach to Derive Right Ventricular Pressure-Volume Loop Phenotypes in Pulmonary Hypertension.","authors":"Nikita Sivakumar, Cindy Zhang, Connie Chang-Chien, Pan Gu, Yikun Li, Yi Yang, Darin Rosen, Tijana Tuhy, Ilton M Cubero Salazar, Matthew Kauffman, Rachel L Damico, Casey Overby Taylor, Joseph L Greenstein, Steven Hsu, Paul M Hassoun, Catherine E Simpson","doi":"10.1002/pul2.70057","DOIUrl":"10.1002/pul2.70057","url":null,"abstract":"Although right ventricle (RV) dysfunction drives clinical worsening in pulmonary hypertension (PH), information about RV function has not been well integrated in PH risk assessment. The gold standard for assessing RV function and ventriculo-arterial coupling is the construction of multi-beat pressure-volume (PV) loops. PV loops are technically challenging to acquire and not feasible for routine clinical use. Therefore, we aimed to map standard clinically available measurements to emergent PV loop phenotypes. One hundred and one patients with suspected PH underwent right heart catheterization (RHC) with exercise, multi-beat PV loop measurement, and same-day cardiac magnetic resonance imaging (CMR). We applied unsupervised k-means clustering on 10 PV loop metrics to obtain three patient groups with unique RV functional phenotypes and times to clinical worsening. We integrated RHC and CMR measurements to train a random forest classifier that predicts the PV loop patient group with high discrimination (AUC = 0.93). The most informative variable for PV loop phenotype prediction was exercise mean pulmonary arterial pressure (mPAP). Distinct and clinically meaningful PV loop phenotypes exist that can be predicted using clinically accessible hemodynamic and RV-centric measurements. Exercise mPAP may inform RV pressure-volume relationships.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 1","pages":"e70057"},"PeriodicalIF":2.2,"publicationDate":"2025-02-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11842459/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143483970","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

RNA-Seq and ChIP-Seq Identification of Unique and Overlapping Target Genes and Pathways Regulated by TBX4 in Human Pulmonary Fibroblasts and Pericytes.

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-02-19 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70058

Ying Cai, Ling Yan, Joy D Cogan, Lora K Hedges, Bethany Nunley, Nick Negretti, Jennifer M S Sucre, James West, Eric D Austin, Rizwan Hamid

{"title":"RNA-Seq and ChIP-Seq Identification of Unique and Overlapping Target Genes and Pathways Regulated by TBX4 in Human Pulmonary Fibroblasts and Pericytes.","authors":"Ying Cai, Ling Yan, Joy D Cogan, Lora K Hedges, Bethany Nunley, Nick Negretti, Jennifer M S Sucre, James West, Eric D Austin, Rizwan Hamid","doi":"10.1002/pul2.70058","DOIUrl":"10.1002/pul2.70058","url":null,"abstract":"Transcription factor TBX4 rare variants associate with pulmonary arterial hypertension (PAH), particularly in children, and are the second most common cause of heritable PAH. However, TBX4's down-stream targets and the molecular and cellular pathways these targets regulate remain largely unknown in PAH. We combined RNA-seq and ChIP-seq results to identify TBX4 direct targets in lung fibroblasts and pericytes, respectively. There were 555 genes with altered expression with TBX4 knockdown in both fibroblasts and pericytes by RNA-seq, and which also were found to be bound by TBX4 by ChIP-seq. Gene ontology analysis found that these were dominated by genes related to extracellular matrix, actin organization, and migration guidance, although there were also significant groups related to serine/threonine kinase signaling, GTPase mediated signaling, and glycoprotein metabolism. Migration and proliferation studies using TBX4 knockdown fibroblasts confirmed functional effects. These studies provide the first insights into how genes and pathways regulated by TBX4 are impacted and inform future studies about the key biological processes that lead to PAH in patients who carry pathologic TBX4 rare variants.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 1","pages":"e70058"},"PeriodicalIF":2.2,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11839389/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143468910","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Introducing Historical Vignettes in Pulmonary Circulation. 介绍肺循环的历史小故事。

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-02-19 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70061

Harold I Palevsky, Ghazwan Butrous, C Gregory Elliott

引用次数: 0

Developing a Neonatal Pulmonary Hypertension Core Outcome Set (NeoPH COS)-A Study Protocol.

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-02-19 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70052

Cara Ellen Morgan, Kerry Woolfall, Natasharazia Aikman, Crhistopher Gale, Nimish Subhedar

{"title":"Developing a Neonatal Pulmonary Hypertension Core Outcome Set (NeoPH COS)-A Study Protocol.","authors":"Cara Ellen Morgan, Kerry Woolfall, Natasharazia Aikman, Crhistopher Gale, Nimish Subhedar","doi":"10.1002/pul2.70052","DOIUrl":"10.1002/pul2.70052","url":null,"abstract":"Pulmonary hypertension (PH) in newborn babies is a relatively rare, heterogeneous condition that has high associated mortality in the neonatal period and beyond. There are limited evidence-based strategies to treat or prevent this condition. Over the last two decades, there has been an increase in the number of studies assessing new therapies and treatment strategies in babies with PH. However, comparison of different treatments between studies is limited by inconsistency in outcome reporting. To address this issue, we aim to develop a core outcome set (COS) for neonates and infants less than 3 months of age, corrected for prematurity, diagnosed with PH, through international consensus with key stakeholders including parents and/or guardians, healthcare professionals and researchers. The development of the COS will be divided into two stages: (1) identification of potential outcomes through a mixed methods systematic literature review and qualitative interviews with parents and/or guardians of babies with pulmonary hypertension; (2) determining core outcomes through an online Delphi survey and consensus meeting. An advisory group with global membership including parents and/or guardians, healthcare professionals, and researchers recruited internationally was formed to guide the COS. The methodology utilized to develop a neonatal PH COS aims to ensure applicability and adoption in international settings and relevance across disciplines. The COS will help to improve trial design and homogeneity of outcomes reported in neonatal trials of PH. This will translate into higher-quality evidence for therapeutic strategies for PH in neonates.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 1","pages":"e70052"},"PeriodicalIF":2.2,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11839391/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143468857","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

The Role of Ion Channels in Pulmonary Hypertension: A Review.

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-02-16 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70050

Han-Fei Li, Xin-Yao Li, Yu-Qing Sun, Ze-Ying Zhi, Liao-Fan Song, Meng Li, Yi-Ming Feng, Zhi-Hao Zhang, Yan-Feng Liu, Yu-Jing Chen, Fan-Rong Zhao, Tian-Tian Zhu

引用次数: 0