European Respiratory Journal最新文献_第2页

Transplantation, bridging, and support technologies in pulmonary hypertension. 肺动脉高压的移植、桥接和支持技术。

IF 16.6 1区医学

European Respiratory Journal Pub Date : 2024-10-31 Print Date: 2024-10-01 DOI: 10.1183/13993003.01193-2024

Laurent Savale, Alberto Benazzo, Paul Corris, Shaf Keshavjee, Deborah Jo Levine, Olaf Mercier, R Duane Davis, John T Granton

{"title":"Transplantation, bridging, and support technologies in pulmonary hypertension.","authors":"Laurent Savale, Alberto Benazzo, Paul Corris, Shaf Keshavjee, Deborah Jo Levine, Olaf Mercier, R Duane Davis, John T Granton","doi":"10.1183/13993003.01193-2024","DOIUrl":"10.1183/13993003.01193-2024","url":null,"abstract":"Despite the progress made in medical therapies for treating pulmonary hypertension (PH), a subset of patients remain susceptible to developing a maladaptive right ventricular phenotype. The effective management of end-stage PH presents substantial challenges, necessitating a multidisciplinary approach and early identification of patients prone to acute decompensation. Identifying potential transplant candidates and assessing the feasibility of such a procedure are pivotal tasks that should be undertaken early in the treatment algorithm. Inclusion on the transplant list is contingent upon a comprehensive risk assessment, also considering the specific type of PH and various factors affecting waiting times, all of which should inform the decision-making process. While bilateral lung transplantation is the preferred option, it demands expert intra- and post-operative management to mitigate the heightened risks of pulmonary oedema and primary graft dysfunction in PH patients. Despite the availability of risk assessment tools, the occurrence of acute PH decompensation episodes can be unpredictable, potentially leading to refractory right ventricular failure even with optimal medical intervention, necessitating the use of rescue therapies. Advancements in right ventricular assist techniques and adjustments to graft allocation protocols for the most critically ill patients have significantly enhanced the survival in intensive care, affording the opportunity to endure while awaiting an urgent transplant. Given the breadth of therapeutic options available, specialised centres capable of delivering comprehensive care have become indispensable for optimising patient outcomes. These centres are instrumental in providing holistic support and management tailored to the complex needs of PH patients, ultimately enhancing their chances of a successful transplant and improved long-term prognosis.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":null,"pages":null},"PeriodicalIF":16.6,"publicationDate":"2024-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11525343/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142105953","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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

Chronic thromboembolic pulmonary disease. 慢性血栓栓塞性肺病。

IF 16.6 1区医学

European Respiratory Journal Pub Date : 2024-10-31 Print Date: 2024-10-01 DOI: 10.1183/13993003.01294-2024

Nick H Kim, Andrea M D'Armini, Marion Delcroix, Xavier Jaïs, Mitja Jevnikar, Michael M Madani, Hiromi Matsubara, Massimiliano Palazzini, Christoph B Wiedenroth, Gérald Simonneau, David P Jenkins

{"title":"Chronic thromboembolic pulmonary disease.","authors":"Nick H Kim, Andrea M D'Armini, Marion Delcroix, Xavier Jaïs, Mitja Jevnikar, Michael M Madani, Hiromi Matsubara, Massimiliano Palazzini, Christoph B Wiedenroth, Gérald Simonneau, David P Jenkins","doi":"10.1183/13993003.01294-2024","DOIUrl":"10.1183/13993003.01294-2024","url":null,"abstract":"Chronic thromboembolic pulmonary hypertension is a complication of pulmonary embolism and a treatable cause of pulmonary hypertension. The pathology is a unique combination of mechanical obstruction due to failure of clot resolution, and a variable degree of microvascular disease, that both contribute to pulmonary vascular resistance. Accordingly, multiple treatments have been developed to target the disease components. However, accurate diagnosis is often delayed. Evaluation includes high-quality imaging modalities, necessary for disease confirmation and for appropriate treatment planning. All patients with chronic thromboembolic pulmonary disease, and especially those with pulmonary hypertension, should be referred to expert centres for multidisciplinary team decision on treatment. The first decision remains assessment of operability, and the best improvement in symptoms and survival is achieved by the mechanical therapies, pulmonary endarterectomy and balloon pulmonary angioplasty. With the advances in multimodal therapies, excellent outcomes can be achieved with 3-year survival of >90%.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":null,"pages":null},"PeriodicalIF":16.6,"publicationDate":"2024-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11525345/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142105939","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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

Worsening dyspnoea as a predictor of progression of pulmonary fibrosis. 呼吸困难加重可预测肺纤维化的进展。

IF 16.6 1区医学

European Respiratory Journal Pub Date : 2024-10-31 Print Date: 2024-10-01 DOI: 10.1183/13993003.02211-2023

Marlies S Wijsenbeek, Jeffrey J Swigris, Paolo Spagnolo, Martin Kolb, Michael Kreuter, Hilario Nunes, Wibke Stansen, Klaus B Rohr, Yoshikazu Inoue

引用次数: 0

Risk stratification and treatment goals in pulmonary arterial hypertension. 肺动脉高压的风险分层和治疗目标。

IF 16.6 1区医学

European Respiratory Journal Pub Date : 2024-10-31 Print Date: 2024-10-01 DOI: 10.1183/13993003.01323-2024

Fabio Dardi, Athénaïs Boucly, Raymond Benza, Robert Frantz, Valentina Mercurio, Horst Olschewski, Göran Rådegran, Lewis J Rubin, Marius M Hoeper

{"title":"Risk stratification and treatment goals in pulmonary arterial hypertension.","authors":"Fabio Dardi, Athénaïs Boucly, Raymond Benza, Robert Frantz, Valentina Mercurio, Horst Olschewski, Göran Rådegran, Lewis J Rubin, Marius M Hoeper","doi":"10.1183/13993003.01323-2024","DOIUrl":"10.1183/13993003.01323-2024","url":null,"abstract":"Risk stratification has gained an increasing role in predicting outcomes and guiding the treatment of patients with pulmonary arterial hypertension (PAH). The most predictive prognostic factors are three noninvasive parameters (World Health Organization functional class, 6-min walk distance and natriuretic peptides) that are included in all currently validated risk stratification tools. However, suffering from limitations mainly related to reduced specificity of PAH severity, these variables may not always be adequate in isolation for guiding individualised treatment decisions. Moreover, with effective combination treatment regimens and emerging PAH therapies, markers associated with pulmonary vascular remodelling are expected to become of increasing relevance in guiding the treatment of patients with PAH. While reaching a low mortality risk, assessed with a validated risk tool, remains an important treatment goal, preliminary data suggest that invasive haemodynamics and cardiac imaging may add incremental value in guiding treatment decisions.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":null,"pages":null},"PeriodicalIF":16.6,"publicationDate":"2024-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11525341/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142105951","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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

Treatment algorithm for pulmonary arterial hypertension. 肺动脉高压的治疗算法。

IF 16.6 1区医学

European Respiratory Journal Pub Date : 2024-10-31 Print Date: 2024-10-01 DOI: 10.1183/13993003.01325-2024

Kelly M Chin, Sean P Gaine, Christian Gerges, Zhi-Cheng Jing, Stephen C Mathai, Yuichi Tamura, Vallerie V McLaughlin, Olivier Sitbon

引用次数: 0

Genetics and precision genomics approaches to pulmonary hypertension. 肺动脉高压的遗传学和精准基因组学方法。

IF 16.6 1区医学

European Respiratory Journal Pub Date : 2024-10-31 Print Date: 2024-10-01 DOI: 10.1183/13993003.01370-2024

Eric D Austin, Micheala A Aldred, Mona Alotaibi, Stefan Gräf, William C Nichols, Richard C Trembath, Wendy K Chung

{"title":"Genetics and precision genomics approaches to pulmonary hypertension.","authors":"Eric D Austin, Micheala A Aldred, Mona Alotaibi, Stefan Gräf, William C Nichols, Richard C Trembath, Wendy K Chung","doi":"10.1183/13993003.01370-2024","DOIUrl":"10.1183/13993003.01370-2024","url":null,"abstract":"Considerable progress has been made in the genomics of pulmonary arterial hypertension (PAH) since the 6th World Symposium on Pulmonary Hypertension, with the identification of rare variants in several novel genes, as well as common variants that confer a modest increase in PAH risk. Gene and variant curation by an expert panel now provides a robust framework for knowing which genes to test and how to interpret variants in clinical practice. We recommend that genetic testing be offered to specific subgroups of symptomatic patients with PAH, and to children with certain types of group 3 pulmonary hypertension (PH). Testing of asymptomatic family members and the use of genetics in reproductive decision-making require the involvement of genetics experts. Large cohorts of PAH patients with biospecimens now exist and extension to non-group 1 PH has begun. However, these cohorts are largely of European origin; greater diversity will be essential to characterise the full extent of genomic variation contributing to PH risk and treatment responses. Other types of omics data are also being incorporated. Furthermore, to advance gene- and pathway-specific care and targeted therapies, gene-specific registries will be essential to support patients and their families and to lay the foundation for genetically informed clinical trials. This will require international outreach and collaboration between patients/families, clinicians and researchers. Ultimately, harmonisation of patient-derived biospecimens, clinical and omic information, and analytic approaches will advance the field.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":null,"pages":null},"PeriodicalIF":16.6,"publicationDate":"2024-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11525347/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142105945","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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

Management of pulmonary hypertension in special conditions. 特殊情况下的肺动脉高压管理。

IF 16.6 1区医学

European Respiratory Journal Pub Date : 2024-10-31 Print Date: 2024-10-01 DOI: 10.1183/13993003.01180-2024

Ioana R Preston, Luke S Howard, David Langleben, Mona Lichtblau, Tomas Pulido, Rogerio Souza, Karen M Olsson

{"title":"Management of pulmonary hypertension in special conditions.","authors":"Ioana R Preston, Luke S Howard, David Langleben, Mona Lichtblau, Tomas Pulido, Rogerio Souza, Karen M Olsson","doi":"10.1183/13993003.01180-2024","DOIUrl":"10.1183/13993003.01180-2024","url":null,"abstract":"Care of pulmonary hypertension (PH) patients in special situations requires insightful knowledge of the pathophysiology of the cardiopulmonary system and close interaction with different specialists, depending on the situation. The role of this task force was to gather knowledge about five conditions that PH patients may be faced with. These conditions are 1) perioperative care; 2) management of pregnancy; 3) medication adherence; 4) palliative care; and 5) the influence of climate on PH. Many of these aspects have not been covered by previous World Symposia on Pulmonary Hypertension. All of the above conditions are highly affected by psychological, geographical and socioeconomic factors, and share the need for adequate healthcare provision. The task force identified significant gaps in information and research in these areas. The current recommendations are based on detailed literature search and expert opinion. The task force calls for further studies and research to better understand and address the special circumstances that PH patients may encounter.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":null,"pages":null},"PeriodicalIF":16.6,"publicationDate":"2024-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11525332/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142105946","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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

Embracing the challenges of neonatal and paediatric pulmonary hypertension. 迎接新生儿和小儿肺动脉高压的挑战。

IF 16.6 1区医学

European Respiratory Journal Pub Date : 2024-10-31 Print Date: 2024-10-01 DOI: 10.1183/13993003.01345-2024

Dunbar Ivy, Erika B Rosenzweig, Steven H Abman, Maurice Beghetti, Damien Bonnet, Johannes Menno Douwes, Alessandra Manes, Rolf M F Berger

{"title":"Embracing the challenges of neonatal and paediatric pulmonary hypertension.","authors":"Dunbar Ivy, Erika B Rosenzweig, Steven H Abman, Maurice Beghetti, Damien Bonnet, Johannes Menno Douwes, Alessandra Manes, Rolf M F Berger","doi":"10.1183/13993003.01345-2024","DOIUrl":"10.1183/13993003.01345-2024","url":null,"abstract":"Paediatric pulmonary arterial hypertension (PAH) shares common features with adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for caring for infants and children with PAH, as presented by the paediatric task force of the 7th World Symposium on Pulmonary Hypertension. We provide updates on diagnosing, classifying, risk-stratifying and treating paediatric pulmonary hypertension (PH) and identify critical knowledge gaps. An updated risk stratification tool and treatment algorithm is provided, now also including strategies for patients with associated cardiopulmonary conditions. Treatment of paediatric PH continues to be hindered by the lack of randomised controlled clinical trials. The challenging management of children failing targeted PAH therapy is discussed, including balloon atrial septostomy, lung transplantation and pulmonary-to-systemic shunt (Potts). A novel strategy using a multimodal approach for the management of PAH associated with congenital heart diseases with borderline pulmonary vascular resistance is included. Advances in diagnosing neonatal PH, especially signs and interpretation of PH by echocardiography, are highlighted. A team approach to the rapidly changing physiology of neonatal PH is emphasised. Challenges in drug approval are discussed, particularly the challenges of designing accurate paediatric clinical trials with age-appropriate end-points and adequate enrolment.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":null,"pages":null},"PeriodicalIF":16.6,"publicationDate":"2024-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11525338/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142105942","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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

Effect of sotatercept on circulating proteomics in pulmonary arterial hypertension. 索泰特受体对肺动脉高压循环蛋白质组学的影响

IF 16.6 1区医学

European Respiratory Journal Pub Date : 2024-10-31 Print Date: 2024-10-01 DOI: 10.1183/13993003.01483-2024

Laurent Savale, Ly Tu, Corinne Normand, Athénaïs Boucly, Olivier Sitbon, David Montani, Karen M Olsson, Da-Hee Park, Jan Fuge, Jan C Kamp, Marc Humbert, Marius M Hoeper, Christophe Guignabert

引用次数: 0

Definition, classification and diagnosis of pulmonary hypertension. 肺动脉高压的定义、分类和诊断。

IF 16.6 1区医学

European Respiratory Journal Pub Date : 2024-10-31 Print Date: 2024-10-01 DOI: 10.1183/13993003.01324-2024

Gabor Kovacs, Sonja Bartolome, Christopher P Denton, Michael A Gatzoulis, Sue Gu, Dinesh Khanna, David Badesch, David Montani

{"title":"Definition, classification and diagnosis of pulmonary hypertension.","authors":"Gabor Kovacs, Sonja Bartolome, Christopher P Denton, Michael A Gatzoulis, Sue Gu, Dinesh Khanna, David Badesch, David Montani","doi":"10.1183/13993003.01324-2024","DOIUrl":"10.1183/13993003.01324-2024","url":null,"abstract":"Pulmonary hypertension (PH) is a haemodynamic condition characterised by elevation of mean pulmonary arterial pressure (mPAP) >20 mmHg, assessed by right heart catheterisation. Pulmonary arterial wedge pressure (PAWP) and pulmonary vascular resistance (PVR) distinguish pre-capillary PH (PAWP ≤15 mmHg, PVR >2 Wood Units (WU)), isolated post-capillary PH (PAWP >15 mmHg, PVR ≤2 WU) and combined post- and pre-capillary PH (PAWP >15 mmHg, PVR >2 WU). Exercise PH is a haemodynamic condition describing a normal mPAP at rest with an abnormal increase of mPAP during exercise, defined as a mPAP/cardiac output slope >3 mmHg/L/min between rest and exercise. The core structure of the clinical classification of PH has been retained, including the five major groups. However, some changes are presented herewith, such as the re-introduction of \"long-term responders to calcium channel blockers\" as a subgroup of idiopathic pulmonary arterial hypertension, the addition of subgroups in group 2 PH and the differentiation of group 3 PH subgroups based on pulmonary diseases instead of functional abnormalities. Mitomycin-C and carfilzomib have been added to the list of drugs with \"definite association\" with PAH. For diagnosis of PH, we propose a stepwise approach with the main aim of discerning those patients who need to be referred to a PH centre and who should undergo invasive haemodynamic assessment. In case of high probability of severe pulmonary vascular disease, especially if there are signs of right heart failure, a fast-track referral to a PH centre is recommended at any point during the clinical workup.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":null,"pages":null},"PeriodicalIF":16.6,"publicationDate":"2024-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11533989/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142105941","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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