Publications
343 results found
Afoke J, Mohal J, Kanaganayagam GS, et al., 2023, Cardiopulmonary exercise testing augments watchful waiting in asymptomatic severe primary mitral regurgitation, PERFUSION-UK, ISSN: 0267-6591
Souza R, Badesch DB, Ghofrani HA, et al., 2023, Effects of sotatercept on haemodynamics and right heart function: analysis of the STELLAR trial, EUROPEAN RESPIRATORY JOURNAL, Vol: 62, ISSN: 0903-1936
Hoeper MM, Badesch DB, Ghofrani HA, et al., 2023, Phase 3 Trial of Sotatercept for Treatment of Pulmonary Arterial Hypertension, NEW ENGLAND JOURNAL OF MEDICINE, Vol: 388, Pages: 1478-1490, ISSN: 0028-4793
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- Citations: 33
Aktaa S, Gale CP, Brida M, et al., 2023, European Society of Cardiology quality indicators for the care and outcomes of adults with pulmonary arterial hypertension. Developed in collaboration with the Heart Failure Association of the European Society of Cardiology, EUROPEAN JOURNAL OF HEART FAILURE, Vol: 25, Pages: 469-477, ISSN: 1388-9842
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- Citations: 1
Coghlan JG, Gaine S, Channick R, et al., 2023, Early selexipag initiation and long-term outcomes: insights from randomised controlled trials in pulmonary arterial hypertension, ERJ OPEN RESEARCH, Vol: 9
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- Citations: 1
Humbert M, McLaughlin V, Gibbs JSR, et al., 2023, Sotatercept for the treatment of pulmonary arterial hypertension: PULSAR open-label extension, EUROPEAN RESPIRATORY JOURNAL, Vol: 61, ISSN: 0903-1936
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- Citations: 2
Leong K, Howard L, Giudice FL, et al., 2023, Utility of cardiac magnetic resonance feature tracking strain assessment in chronic thromboembolic pulmonary hypertension for prediction of REVEAL 2.0 high risk status, PULMONARY CIRCULATION, Vol: 13, ISSN: 2045-8932
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- Citations: 1
Kariotis S, Jammeh E, Swietlik EM, et al., 2022, Biological heterogeneity in idiopathic pulmonary arterial hypertension identified through unsupervised transcriptomic profiling of whole blood, Nature Communications, Vol: 13, Pages: 1-1, ISSN: 2041-1723
Hoeper MM, Dwivedi K, Pausch C, et al., 2022, Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis, LANCET RESPIRATORY MEDICINE, Vol: 10, Pages: 937-948, ISSN: 2213-2600
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- Citations: 30
Hoeper M, Ghofrani H, Gibbs JS, et al., 2022, Effect of sotatercept on multicomponent improvement in the PULSAR study, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Hoeper MM, Pausch C, Olsson KM, et al., 2022, COMPERA 2.0: a refined four-stratum risk assessment model for pulmonary arterial hypertension, EUROPEAN RESPIRATORY JOURNAL, Vol: 60, ISSN: 0903-1936
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- Citations: 73
Jones RJ, De Bie EMDD, Groves E, et al., 2022, Autoimmunity is a significant feature of idiopathic pulmonary arterial hypertension., American Journal of Respiratory and Critical Care Medicine, Vol: 206, Pages: 81-93, ISSN: 1073-449X
RATIONALE: Autoimmunity is thought to play a role in idiopathic pulmonary arterial hypertension (IPAH). It is not clear if this is causative or a bystander of disease and if it carries any prognostic or treatment significance. OBJECTIVE: To study autoimmunity in IPAH using a large cross-sectional cohort. METHODS: Assessment of the circulating immune cell phenotype was undertaken using flow cytometry and the profile of serum immunoglobulins was generated using a standardised multiplex array of 19 clinically validated autoantibodies in 473 cases and 946 controls. Additional GST-fusion array and ELISA data were used to identify a serum autoantibody to BMPR2. Clustering analyses and clinical correlations were employed to determine associations between immunogenicity and clinical outcomes. MEASUREMENTS AND MAIN RESULTS: Flow cytometric immune profiling demonstrates IPAH is associated with an altered humoral immune response in addition to raised IgG3. Multiplexed autoantibodies were significantly raised in IPAH, and clustering demonstrated three distinct clusters: 'high autoantibody', 'low autoantibody', and a small 'intermediate' cluster exhibiting high levels of RNP-complex. The high autoantibody cluster had worse haemodynamics but improved survival. A small subset of patients demonstrated immunoglobulin reactivity to BMPR2. CONCLUSIONS: This study establishes aberrant immune regulation and presence of autoantibodies as a key feature in the profile of a significant proportion of IPAH patients and is associated with clinical outcomes. This article is open access and distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/).
Toshner M, Church C, Harbaum L, et al., 2022, Mendelian randomisation and experimental medicine approaches to interleukin-6 as a drug target in pulmonary arterial hypertension (vol 59, 2002463, 2022), EUROPEAN RESPIRATORY JOURNAL, Vol: 60, ISSN: 0903-1936
Hoeper MM, Pausch C, Olsson KM, et al., 2022, Prognostic value of improvement endpoints in pulmonary arterial hypertension trials: A COMPERA analysis, JOURNAL OF HEART AND LUNG TRANSPLANTATION, Vol: 41, Pages: 971-981, ISSN: 1053-2498
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- Citations: 6
Hoeper MM, Pausch C, Gruenig E, et al., 2022, Temporal trends in pulmonary arterial hypertension: results from the COMPERA registry, EUROPEAN RESPIRATORY JOURNAL, Vol: 59, ISSN: 0903-1936
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- Citations: 32
Humbert M, Mclaughlin VV, Gibbs SR, et al., 2022, PULSAR Open-Label Extension: Long-Term Efficacy and Safety of Sotatercept for the Treatment of Pulmonary Arterial Hypertension (PAH), International Conference of the American-Thoracic-Society, Publisher: AMER THORACIC SOC, ISSN: 1073-449X
Leong K, Howard L, Lo Giudice F, et al., 2022, MRI Feature Tracking Strain in Pulmonary Hypertension: Utility of Combined Left Atrial Volumetric and Deformation Assessment in Distinguishing Post- From Pre-capillary Physiology, FRONTIERS IN CARDIOVASCULAR MEDICINE, Vol: 9, ISSN: 2297-055X
Kariotis S, Jammeh E, Swietlik EM, et al., 2021, Biological heterogeneity in idiopathic pulmonary arterial hypertension identified through unsupervised transcriptomic profiling of whole blood, Nature Communications, Vol: 12, Pages: 1-14, ISSN: 2041-1723
Idiopathic pulmonary arterial hypertension (IPAH) is a rare but fatal disease diagnosed by right heart catheterisation and the exclusion of other forms of pulmonary arterial hypertension, producing a heterogeneous population with varied treatment response. Here we show unsupervised machine learning identification of three major patient subgroups that account for 92% of the cohort, each with unique whole blood transcriptomic and clinical feature signatures. These subgroups are associated with poor, moderate, and good prognosis. The poor prognosis subgroup is associated with upregulation of the ALAS2 and downregulation of several immunoglobulin genes, while the good prognosis subgroup is defined by upregulation of the bone morphogenetic protein signalling regulator NOG, and the C/C variant of HLA-DPA1/DPB1 (independently associated with survival). These findings independently validated provide evidence for the existence of 3 major subgroups (endophenotypes) within the IPAH classification, could improve risk stratification and provide molecular insights into the pathogenesis of IPAH.
Johnson S, Gordeuk VR, Machado R, et al., 2021, Exercise-induced changes of vital signs in adults with sickle cell disease, AMERICAN JOURNAL OF HEMATOLOGY, Vol: 96, Pages: 1630-1638, ISSN: 0361-8609
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- Citations: 2
Babu V, Little JA, Morris CR, et al., 2021, Targeted Proteomics of Pulmonary Hypertension in Sickle Cell Disease, 63rd Annual Meeting and Exposition of the American-Society-of-Hematology (ASH), Publisher: AMER SOC HEMATOLOGY, ISSN: 0006-4971
Chin KM, Sitbon O, Doelberg M, et al., 2021, Three- Versus Two-Drug Therapy for Patients With Newly Diagnosed Pulmonary Arterial Hypertension, JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, Vol: 78, Pages: 1393-1403, ISSN: 0735-1097
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- Citations: 53
Coghlan GJ, Gaine S, Channick RN, et al., 2021, Treatment effect of selexipag on time to disease progression when initiated early in pulmonary arterial hypertension (PAH) patients: GRIPHON and TRITON pooled analysis, Publisher: OXFORD UNIV PRESS, Pages: 1964-1964, ISSN: 0195-668X
Toshner M, Church C, Harbaum L, et al., 2021, Mendelian randomisation and experimental medicine approaches to IL-6 as a drug target in PAH, European Respiratory Journal, Vol: 59, Pages: 1-11, ISSN: 0903-1936
Inflammation and dysregulated immunity are important in the development of pulmonary arterial hypertension. Compelling preclinical data supports the therapeutic blockade of interleukin-6 signalling.We conducted an open-label phase-II study of intravenous tocilizumab (8 mg·kg-1) over 6 months in group 1 pulmonary arterial hypertension. Co-primary endpoints were safety, defined by incidence and severity of adverse events, and change in pulmonary vascular resistance. Separately, a Mendelian randomisation study was undertaken on 11,744 individuals with European ancestry including 2085 patients with idiopathic/heritable disease for the IL6R variant (rs7529229), known to associate with circulating IL6R levels.Twenty-nine patients (M/F 10/19; mean age 54.9[SD11.4]) were recruited. Nineteen had heritable/idiopathic and ten connective tissue disease associated pulmonary arterial hypertension. Six were withdrawn prior to drug administration. Twenty-three patients received at least one dose of tocilizumab. Tocilizumab was discontinued in 4 patients due to serious adverse events. There were no deaths. Despite evidence of target engagement in plasma interleukin-6 and C-reactive protein levels, both intention-to-treat and modified intention-to-treat analyses demonstrated no change in pulmonary vascular resistance. Inflammatory markers did not predict treatment response. Mendelian randomisation did not support an effect of the lead IL6R variant on risk of pulmonary arterial hypertension (OR 0.99, p=0.88).Adverse events were consistent with the known safety profile of tocilizumab. Tocilizumab did not show any consistent treatment effect.
Badesch D, Gibbs S, Gomberg-Maitland M, et al., 2021, PULSAR open-label extension: interim results from a phase 2 study of the efficacy and safety of sotatercept when added to standard of care for the treatment of pulmonary arterial hypertension (PAH), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Vizza CD, Hoeper MM, Huscher D, et al., 2021, Pulmonary Hypertension in Patients With COPD Results From the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA), CHEST, Vol: 160, Pages: 678-689, ISSN: 0012-3692
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- Citations: 39
Hoeper MM, Al-Hiti H, Benza RL, et al., 2021, Switching to riociguat versus maintenance therapy with phosphodiesterase-5 inhibitors in patients with pulmonary arterial hypertension (REPLACE): a multicentre, open-label, randomised controlled trial, LANCET RESPIRATORY MEDICINE, Vol: 9, Pages: 573-584, ISSN: 2213-2600
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- Citations: 57
Badesch DB, Gibbs S, Gomberg-Maitland M, et al., 2021, PULSAR Study Open-Label Extension: Interim Results from a Phase 2 Study of the Efficacy and Safety of Sotatercept When Added to Standard of Care for the Treatment of Pulmonary Arterial Hypertension (PAH), International Conference of the American-Thoracic-Society (ATS), Publisher: AMER THORACIC SOC, ISSN: 1073-449X
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- Citations: 1
Humbert M, McLaughlin V, Gibbs JSR, et al., 2021, Sotatercept for the Treatment of Pulmonary Arterial Hypertension, NEW ENGLAND JOURNAL OF MEDICINE, Vol: 384, Pages: 1204-1215, ISSN: 0028-4793
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- Citations: 146
McLaughlin V, Chin K, Doelberg M, et al., 2021, Initial Triple Oral Therapy in Pulmonary Arterial Hypertension (PAH): Extended Long-Term Outcome Data from TRITON, Publisher: ELSEVIER SCIENCE INC, Pages: S108-S108, ISSN: 1053-2498
Lewis RA, Armstrong I, Bergbaum C, et al., 2021, EmPHasis-10 health-related quality of life score predicts outcomes in patients with idiopathic and connective tissue disease-associated pulmonary arterial hypertension: results from a UK multicentre study, EUROPEAN RESPIRATORY JOURNAL, Vol: 57, ISSN: 0903-1936
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- Citations: 21
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