Pulmonary Circulation最新文献_第2页

Comparing Patency of Pulmonary Stent Implantation and Balloon Angioplasty in Pulmonary Vein Stenosis: A Systematic Review and Meta-Analysis. 肺动脉支架置入术与球囊成形术治疗肺静脉狭窄通畅的比较：一项系统综述和荟萃分析。

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-01-14 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70036

Izza Batool, Muhammad Khuzzaim Khan, Muhammad Zohaib, Ibrahim Ahmed Khan, Syed Affan Ahmed Bukhari, Shreeja Shah, Khaled Harmouch, Hussam Al Hennawi, Bruce Klugherz

{"title":"Comparing Patency of Pulmonary Stent Implantation and Balloon Angioplasty in Pulmonary Vein Stenosis: A Systematic Review and Meta-Analysis.","authors":"Izza Batool, Muhammad Khuzzaim Khan, Muhammad Zohaib, Ibrahim Ahmed Khan, Syed Affan Ahmed Bukhari, Shreeja Shah, Khaled Harmouch, Hussam Al Hennawi, Bruce Klugherz","doi":"10.1002/pul2.70036","DOIUrl":"10.1002/pul2.70036","url":null,"abstract":"Pulmonary vein stenosis (PVS) is an insidious diagnosis associated with morbidity and mortality. Pharmacologic therapy may suffice initially, but advanced stages demand mechanical intervention. Pulmonary stent implantation (PSI) and pulmonary balloon angioplasty (PBA) are common strategies, both carrying restenosis risks. This meta-analysis compares PSI and PBA to determine the superior revascularization strategy. We systematically searched databases until November 2023, identifying 11 studies with 780 patients. Studies, including those involving patients undergoing balloon angioplasty (BA) or stent angioplasty (SA) for PVS, were selected. Case reports, editorials, and cross-sectional studies were omitted. Primary outcomes included restenosis requiring reintervention, 5-year freedom from restenosis, and procedure-related complications. Odds ratios (ORs) with 95% confidence intervals (CIs) were calculated using a random-effects model. Meta-regression analysis assessed factors like age and stent size. Study quality was evaluated using the Newcastle-Ottawa scale. This Systematic review and meta-analysis incorporated 11 observational studies. PSI exhibited a lower risk of restenosis requiring reintervention (OR 0.34, 95% CI 0.13, 0.87, p = 0.02) and significantly higher 5-year freedom from restenosis (OR 4.42, 95% CI 1.11, 17.62, p = 0.04) compared to PBA, with no significant difference in procedure-related complications. Meta-regression analysis showed age and stent size insignificantly affecting restenosis risk. Our review supports PSI as the preferred revascularization strategy for PVS due to superior patency benefits, emphasizing its role as the initial treatment choice. Further research is warranted for validation, considering individual patient factors in treatment selection.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 1","pages":"e70036"},"PeriodicalIF":2.2,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11732854/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143010518","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

Disparities in Clinical Outcomes Observed Within Electronic Health Record Data From a Statewide Cohort of Pulmonary Arterial Hypertension Patients. 从全国范围内肺动脉高压患者队列的电子健康记录数据中观察到临床结果的差异。

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-01-13 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70041

Rachel Dalton, Ankit A Desai, Tianze Jiao, Julio D Duarte

{"title":"Disparities in Clinical Outcomes Observed Within Electronic Health Record Data From a Statewide Cohort of Pulmonary Arterial Hypertension Patients.","authors":"Rachel Dalton, Ankit A Desai, Tianze Jiao, Julio D Duarte","doi":"10.1002/pul2.70041","DOIUrl":"10.1002/pul2.70041","url":null,"abstract":"Health disparities in patients with pulmonary arterial hypertension (PAH) have not been extensively reported in the United States. The aim of this project was to characterize the extent of demographic and socioeconomic disparities in clinical outcomes within a large, diverse PAH patient population. A retrospective, population-based study of electronic health record data from the OneFlorida Data Trust was completed. Adult patients seeking care within one of the 12 OneFlorida Network partner healthcare systems with a documented diagnosis of any form of pulmonary hypertension (PH), including PAH, via ICD-10 code were included. Social deprivation index and healthcare provider access scores were calculated from population-based centroids derived from patient home addresses. The primary outcome was all-cause mortality, with secondary outcomes including hospitalization, emergency department (ED) visits, and similar clinical outcomes in a combined cohort of patients with other forms PH. A total of 6379 patients were included in the PAH cohort, and 37,412 patients were included in the nonspecific PH cohort. PAH patients with the greatest social deprivation exhibited increased rates of ED visits and hospitalizations. Despite having similar rates of ED visits and hospitalizations compared to non-Hispanics, Hispanic PAH patients had markedly lower mortality rates. Similar associations were also observed in the combined cohort of 37,412 patients with other forms of PH. In conclusion, healthcare disparities exist in PAH outcomes across both demographic and socioeconomic boundaries. Patients identifying as Hispanic appear to have decreased rates of mortality compared to other races/ethnicities.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 1","pages":"e70041"},"PeriodicalIF":2.2,"publicationDate":"2025-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11729391/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142984650","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

Elective Thoracic Surgical Resections for Pulmonary Arteriovenous Malformations - A 16 Year Single-Center Experience. 选择性胸外科手术切除肺动静脉畸形- 16年单中心经验。

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-01-07 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70037

May Al-Sahaf, Jon Anderson, Jayanta Nandi, Ali Alsafi, Claire L Shovlin

{"title":"Elective Thoracic Surgical Resections for Pulmonary Arteriovenous Malformations - A 16 Year Single-Center Experience.","authors":"May Al-Sahaf, Jon Anderson, Jayanta Nandi, Ali Alsafi, Claire L Shovlin","doi":"10.1002/pul2.70037","DOIUrl":"10.1002/pul2.70037","url":null,"abstract":"Pulmonary arteriovenous malformations (PAVMs) cause cerebral abscess and ischemic stroke due to paradoxical emboli, risks that are increasingly recognized. We report the evolving placement of thoracic surgery in multi-disciplinary team management of PAVMs that were sporadic or associated with hereditary hemorrhagic telangiectasia. From 1983 to 2006, all patients receiving elective treatment had embolization. Between January 2006 and July 2022, 24 of 714 (3%) patients reviewed at our institution underwent elective surgical resection of one or more PAVMs. Initially, the bar for elective surgery had been set very high, and only patients with persistent symptoms of cerebral ischemia after maximal embolization and medical therapy were referred. As PAVM natural history, follow-up radiation exposures for residual PAVMs, and good surgical outcomes were appreciated, PAVM resection became part of the discussion for highly localized, very complex PAVMs which we consider are best treated surgically. Elective surgical intervention may be considered for other selected patients.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 1","pages":"e70037"},"PeriodicalIF":2.2,"publicationDate":"2025-01-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11705419/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142954097","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

New Insights Into Pediatric Pulmonary Hypertension From Bogota: Is Younger Always Better? 波哥大对儿童肺动脉高压的新见解：越年轻越好吗？

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-01-07 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70039

Michael A Smith, Jeffrey R Fineman

引用次数: 0

Precision Medicine for Pulmonary Vascular Disease: The Future Is Now (2023 Grover Conference Series). 肺血管疾病的精准医学：未来就是现在（2023 Grover会议系列）。

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-01-02 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70027

Lindsay M Forbes, Natalie Bauer, Aritra Bhadra, Harm J Bogaard, Gaurav Choudhary, Kara N Goss, Stefan Gräf, Gustavo A Heresi, Rachel K Hopper, Arun Jose, Yunhye Kim, Timothy Klouda, Tim Lahm, Allan Lawrie, Peter J Leary, Jane A Leopold, Suellen D Oliveira, Sasha Z Prisco, Ruslan Rafikov, Christopher J Rhodes, Duncan J Stewart, Rebecca R Vanderpool, Ke Yuan, Alexsandra Zimmer, Anna R Hemnes, Vinicio A de Jesus Perez, Martin R Wilkins

{"title":"Precision Medicine for Pulmonary Vascular Disease: The Future Is Now (2023 Grover Conference Series).","authors":"Lindsay M Forbes, Natalie Bauer, Aritra Bhadra, Harm J Bogaard, Gaurav Choudhary, Kara N Goss, Stefan Gräf, Gustavo A Heresi, Rachel K Hopper, Arun Jose, Yunhye Kim, Timothy Klouda, Tim Lahm, Allan Lawrie, Peter J Leary, Jane A Leopold, Suellen D Oliveira, Sasha Z Prisco, Ruslan Rafikov, Christopher J Rhodes, Duncan J Stewart, Rebecca R Vanderpool, Ke Yuan, Alexsandra Zimmer, Anna R Hemnes, Vinicio A de Jesus Perez, Martin R Wilkins","doi":"10.1002/pul2.70027","DOIUrl":"10.1002/pul2.70027","url":null,"abstract":"Pulmonary vascular disease is not a single condition; rather it can accompany a variety of pathologies that impact the pulmonary vasculature. Applying precision medicine strategies to better phenotype, diagnose, monitor, and treat pulmonary vascular disease is increasingly possible with the growing accessibility of powerful clinical and research tools. Nevertheless, challenges exist in implementing these tools to optimal effect. The 2023 Grover Conference Series reviewed the research landscape to summarize the current state of the art and provide a better understanding of the application of precision medicine to managing pulmonary vascular disease. In particular, the following aspects were discussed: (1) Clinical phenotypes, (2) genetics, (3) epigenetics, (4) biomarker discovery, (5) application of precision biology to clinical trials, (6) the right ventricle (RV), and (7) integrating precision medicine to clinical care. The present review summarizes the content of these discussions and the prospects for the future.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 1","pages":"e70027"},"PeriodicalIF":2.2,"publicationDate":"2025-01-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11693987/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142922774","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

Common Variable Immunodeficiency Associated With Noninfectious Pulmonary Complications and Its Treatment: Beyond Immunoglobulin Therapy. 与非感染性肺部并发症相关的常见可变免疫缺陷及其治疗：超越免疫球蛋白治疗。

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-01-01 DOI: 10.1002/pul2.70034

Austin J Parsons, Domingo Franco-Palacios, Bryan Kelly, Gillian Grafton, Javardo McIntosh, David Coleman, Asif M Abdul Hameed, Alaa Abu Sayf

{"title":"Common Variable Immunodeficiency Associated With Noninfectious Pulmonary Complications and Its Treatment: Beyond Immunoglobulin Therapy.","authors":"Austin J Parsons, Domingo Franco-Palacios, Bryan Kelly, Gillian Grafton, Javardo McIntosh, David Coleman, Asif M Abdul Hameed, Alaa Abu Sayf","doi":"10.1002/pul2.70034","DOIUrl":"10.1002/pul2.70034","url":null,"abstract":"Common variable immunodeficiency (CVID) is a type of primary immunodeficiency that presents as a heterogenous disorder characterized by hypogammaglobinemia, poor response to vaccines, recurrent sinopulmonary infections, and can have noninfectious systemic manifestations. We performed a single-center, retrospective, observational study of five patients with noninfectious complications of CVID. All patients had CVID as defined by the European Society of Immunodeficiencies criteria and had received intravenous immunoglobulin therapy. There were multiple pulmonary manifestations of CVID including frequent pneumonias, bronchiectasis, granulomatous lung disease, and pulmonary hypertension. All our patients were treated with pulmonary vasodilators for severe precapillary pulmonary hypertension along with individualized immunosuppression regimen for interstitial lung disease. Despite treatment for interstitial lung disease and PH, their conditions worsened over 2-3 years with all patients progressing toward organ transplant evaluation. Idiopathic thrombocytopenia and non-cirrhotic portal hypertension were common, with three patients probably suffering from nodular regenerative hyperplasia. Noninfectious complications of CVID can affect different organs and progress despite advanced therapies. Single or multiorgan transplantation is a treatment option for patients with end-stage organ involvement refractory to medical therapy.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 1","pages":"e70034"},"PeriodicalIF":2.2,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11688575/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142915492","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 Pulmonary Artery Flow Hemodynamics Between PAH and PH-HFpEF: Insights From 4D-Flow CMR. PAH 和 PH-HFpEF 之间肺动脉血流动力学的差异：4D-血流 CMR 的启示。

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2025-01-01 DOI: 10.1002/pul2.70022

Bong-Joon Kim, Jeesoo Lee, Haben Berhane, Benjamin H Freed, Sanjiv J Shah, James D Thomas

{"title":"Differences in Pulmonary Artery Flow Hemodynamics Between PAH and PH-HFpEF: Insights From 4D-Flow CMR.","authors":"Bong-Joon Kim, Jeesoo Lee, Haben Berhane, Benjamin H Freed, Sanjiv J Shah, James D Thomas","doi":"10.1002/pul2.70022","DOIUrl":"10.1002/pul2.70022","url":null,"abstract":"Pulmonary artery (PA) flow analysis is crucial for understanding the progression of pulmonary hypertension (PH). We hypothesized that PA flow characteristics vary according to PH etiology. In this study, we used 4D flow cardiovascular magnetic resonance imaging (CMR) to compare PA flow velocity and wall shear stress (WSS) between patients with pulmonary arterial hypertension (PAH) and those with heart failure with preserved ejection fraction and pulmonary hypertension (PH-HFpEF). We enrolled 13 PAH and 15 PH-HFpEF patients. All participants underwent echocardiography, 4D flow CMR, and right heart catheterization. We compared right ventricular outflow tract (RVOT) flow and main pulmonary artery (MPA) hemodynamics, including peak velocity and mean and maximum WSS, between groups. PH-HFpEF patients were older and more likely to have hypertension. PAH patients had higher mean PA pressure (47.8 ± 8.8 vs. 32.9 ± 6.9 mmHg, p < 0.001) and pulmonary vascular resistance (PVR) (8.6 ± 4.6 vs. 2.6 ± 2.2 wood unit, p < 0.001). RVOT systolic notching was more common in PAH patients (8 of 13 vs. 0 of 15), and they had shorter RVOT acceleration time (85.5 ± 20.9 vs. 135.0 ± 21.7 ms, p < 0.001). PAH patients had lower MPA Vmax (0.8 ± 0.2 vs. 1.1 ± 0.4 m/s, p = 0.032), mean WSS (0.29 ± 0.09 vs. 0.36 ± 0.06 Pa, p = 0.035), and maximal WSS (0.99 ± 0.18 vs. 1.21 ± 0.19 Pa, p = 0.011). Anterior MPA analysis confirmed lower WSS in PAH patients. PVR was negatively correlated with MPA mean WSS (r = -0.630, p = 0.002). PAH patients had lower MPA Vmax and lower mean MPA WSS in 4D flow CMR compared to PH-HFpEF patients. These distinct PA flow characteristics suggest that the flow hemodynamics of the PA remodeling process differ depending on the underlying etiology of PH.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 1","pages":"e70022"},"PeriodicalIF":2.2,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11693845/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142922761","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

Performance of Risk-Stratification Scores for Patients With Pulmonary Arterial Hypertension in a Multi-Ethnic Asian Population. 多种族亚洲人群肺动脉高压患者的风险分层评分表现

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2024-12-29 eCollection Date: 2025-01-01 DOI: 10.1002/pul2.70032

Haowen Jiang, Ju Le Tan, Wen Ruan, Jin Shing Hon, Aidila Ismail, Chee Lan Lim, Sumathy Perumal, Michelle Koh, Duu Wen Sewa, Ghee Chee Phua, Ying Zi Oh, Sue-Ann Ng, Cassandra Hong, Andrea Low, Soo Teik Lim, Jonathan Yap

{"title":"Performance of Risk-Stratification Scores for Patients With Pulmonary Arterial Hypertension in a Multi-Ethnic Asian Population.","authors":"Haowen Jiang, Ju Le Tan, Wen Ruan, Jin Shing Hon, Aidila Ismail, Chee Lan Lim, Sumathy Perumal, Michelle Koh, Duu Wen Sewa, Ghee Chee Phua, Ying Zi Oh, Sue-Ann Ng, Cassandra Hong, Andrea Low, Soo Teik Lim, Jonathan Yap","doi":"10.1002/pul2.70032","DOIUrl":"10.1002/pul2.70032","url":null,"abstract":"Guidelines recommend risk stratification of pulmonary arterial hypertension (PAH) patients to guide management. There are currently several risk stratification scores available, which have largely been validated in various pulmonary hypertension registries in the West but not in Asia. We aim to study the performance of these different risk scores in PAH patients from a multi-ethnic Asian population. A retrospective review of all PAH patients from Jan 2014 to Jun 2021 from a tertiary cardiac center was performed. Mortality outcomes were obtained from national registries. Using the 2022 ESC/ERS, REVEAL Lite 2.0 and COMPERA 2.0 risk scores, patients were classified into different risk strata at baseline and at follow-up and changes in any risk strata recorded. The prognosis of patients based on these factors was compared. A total of 153 patients (mean age: 57 ± 17 years; 117 women; 94 Chinese, 33 Malay, 19 Indian) were included. All three scores showed significant difference in mortality outcomes between the different risk strata both at baseline and at follow-up (p < 0.05), with the highest risk group showing the highest mortality. Patients who worsened to or remained at intermediate/high-risk generally had a worse prognosis than those who remained stable at or improved to low-risk strata. The 2022 ESC/ERS and COMPERA 2.0 risk scores had C-statistics of 0.73 (0.58-0.88) and 0.80 (0.72-0.88), respectively, for predicting 1-year mortality. Serial risk stratification is a useful tool in prognosticating Asian PAH patients and may play an important role in guiding therapeutic management.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"15 1","pages":"e70032"},"PeriodicalIF":2.2,"publicationDate":"2024-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11682694/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142907551","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

Safety, tolerability, and efficacy of an in-class combination therapy switch from bosentan plus sildenafil to ambrisentan plus tadalafil in children with pulmonary arterial hypertension. 肺动脉高压患儿从波生坦+西地那非到阿姆布里森坦+他达拉非的联合治疗的安全性、耐受性和有效性

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2024-12-26 eCollection Date: 2024-10-01 DOI: 10.1002/pul2.70011

Cara Morgan, Nikmah Idris, Kathy Elterefi, Luca Di Ienno, Andrew Constantine, Sadia Quyam, Roberta Bini, Shahin Moledina

{"title":"Safety, tolerability, and efficacy of an in-class combination therapy switch from bosentan plus sildenafil to ambrisentan plus tadalafil in children with pulmonary arterial hypertension.","authors":"Cara Morgan, Nikmah Idris, Kathy Elterefi, Luca Di Ienno, Andrew Constantine, Sadia Quyam, Roberta Bini, Shahin Moledina","doi":"10.1002/pul2.70011","DOIUrl":"10.1002/pul2.70011","url":null,"abstract":"The aim of this single-centre retrospective observational study was to evaluate the safety, tolerability, and efficacy of an in-class combination therapy switch from bosentan plus sildenafil to ambrisentan plus tadalafil in children with pulmonary arterial hypertension. Children aged over 5 years who were established on sildenafil plus bosentan were offered to undergo a therapy switch from May 2014 to May 2021 and, if remaining in the service, followed up to May 2024. Children with Eisenmenger syndrome, open intra or extra-cardiac shunt, or with pulmonary hypertension-associated lung disease were excluded. As part of a structured clinical program children were assessed via walk test, echocardiography, cardiac magnetic resonance imaging (CMRI), cardiopulmonary exercise testing, and serum biomarkers. Fifty-two children were included, 33 in the switch group and 19 in the control group. Clinical characteristics at diagnosis and baseline assessments did not differ between groups. All children tolerated the medication switch. Over a median 13.0 [12.0,13.7] week follow-up in the switch group there was a significant improvement in World Health Organization functional class (WHO FC, p < 0.001); reduction in estimated right ventricular systolic pressure by echocardiography of 7 mmHg (p = 0.03) and a 2% increase (p = 0.03) in right ventricular ejection fraction on CMRI. There was a sustained improvement in WHO FC (p < 0.01) in the switch group at medium-term follow-up of 40.9 [35.2,49.3] weeks. Long-term outcome of transplant- or Potts shunt-free survival was comparable between the two groups.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 4","pages":"e70011"},"PeriodicalIF":2.2,"publicationDate":"2024-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11671346/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142903169","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

Exploring the Genetic Association Between Connective Tissue Diseases and the Risk of Pulmonary Arterial Hypertension: A Mendelian Randomization Analysis. 探索结缔组织疾病与肺动脉高压风险之间的遗传关联：孟德尔随机分析。

IF 2.2 4区医学

Pulmonary Circulation Pub Date : 2024-12-24 eCollection Date: 2024-10-01 DOI: 10.1002/pul2.70033

Xiaoqin Wang, Leilei Yang, Bingjie Gu, Dinglei Su

{"title":"Exploring the Genetic Association Between Connective Tissue Diseases and the Risk of Pulmonary Arterial Hypertension: A Mendelian Randomization Analysis.","authors":"Xiaoqin Wang, Leilei Yang, Bingjie Gu, Dinglei Su","doi":"10.1002/pul2.70033","DOIUrl":"10.1002/pul2.70033","url":null,"abstract":"This study aimed to explore the potential causal link between genetic predisposition to various connective tissue diseases (CTDs), namely systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), polymyositis (PM), dermatomyositis (DM), systemic sclerosis (SSc), mixed connective tissue disease (MCTD), and rheumatoid arthritis (RA), and the incidence of pulmonary arterial hypertension (PAH) utilizing Mendelian randomization (MR). Employing a two-sample MR approach, genetic variants associated with CTDs served as instrumental variables to investigate the exposure-outcome relationship, with GWAS data sourced from the FinnGen Biobank. Comprehensive statistical analyses, including the inverse variance weighted (IVW) method, were conducted, alongside heterogeneity, pleiotropy, and sensitivity tests to ensure the robustness and validity of findings. The results revealed that in the Finnish population, no significant causal associations were identified between PAH and SLE, SS, PM, DM, MCTD, or RA. Notably, a significant association was observed between SSc and an increased risk of PAH (IVW: OR = 1.278, 95% CI = 1.061-1.540, p = 0.010). However, this finding was not replicated in other European populations. These results indicate the unique genetic and pathological pathways underlying SSc-associated PAH, emphasizing the need for tailored screening and management protocols in this patient group.","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":"14 4","pages":"e70033"},"PeriodicalIF":2.2,"publicationDate":"2024-12-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11667098/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142884845","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