Treatment of pulmonary arterial hypertension: recent progress and a look to the future

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The next generation of evidence-based medicine

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Serum and Pulmonary Expression Profiles of the Activin Signaling System in Pulmonary Arterial Hypertension

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Heterogeneity of treatment effects by risk in pulmonary arterial hypertension

(2023) Hao-Min Pan et al.   EUROPEAN RESPIRATORY JOURNAL

Phase 3 Trial of Sotatercept for Treatment of Pulmonary Arterial Hypertension

(2023) Marius M. Hoeper et al.   NEW ENGLAND JOURNAL OF MEDICINE

Is low-risk status a surrogate outcome in pulmonary arterial hypertension? An analysis of three randomised trials

(2023) Bryan S Blette et al.   Lancet Respiratory Medicine

Pulmonary hypertension associated with lung disease: new insights into pathomechanisms, diagnosis, and management

(2023) Karen M Olsson et al.   Lancet Respiratory Medicine

Remote Six-Minute Walk Testing in Patients with Pulmonary Hypertension: A Pilot Study

(2022) Tess LaPatra et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Using the Plasma Proteome for Risk Stratifying Patients with Pulmonary Arterial Hypertension

(2022) Christopher J Rhodes et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Autoimmunity is a Significant Feature of Idiopathic Pulmonary Arterial Hypertension

(2022) Rowena J. Jones et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

An emerging phenotype of pulmonary arterial hypertension patients carrying SOX17 variants

(2022) David Montani et al.   EUROPEAN RESPIRATORY JOURNAL

Idiopathic pulmonary arterial hypertension patients with a high H2FPEF-score: insights from the Amsterdam UMC PAH-cohort

(2022) Azar Kianzad et al.   JOURNAL OF HEART AND LUNG TRANSPLANTATION

Pulmonary hypertension in Europe: a call to action

(2022) Talha Burki   Lancet Respiratory Medicine

Macrophage-NLRP3 Activation Promotes Right Ventricle Failure in Pulmonary Arterial Hypertension

(2022) Ruaa Al-Qazazi et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Endogenous Retroviral Elements Generate Pathologic Neutrophils in Pulmonary Arterial Hypertension

(2022) Shalina Taylor et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Inhaled Seralutinib Exhibits Potent Efficacy in Models of Pulmonary Arterial Hypertension

(2022) Anna Galkin et al.   EUROPEAN RESPIRATORY JOURNAL

What role can decentralized trial designs play to improve rare disease studies?

(2022) J. Moore et al.   Orphanet Journal of Rare Diseases

Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis

(2022) Marius M Hoeper et al.   Lancet Respiratory Medicine

Association of Remote Technology Use and Other Decentralization Tools With Patient Likelihood to Enroll in Cancer Clinical Trials

(2022) Devon V. Adams et al.   JAMA Network Open

Assessment of Clinical Worsening End Points as a Surrogate for Mortality in Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis of Randomized Controlled Trials

(2022) Élodie Tremblay et al.   CIRCULATION

2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

(2022) Marc Humbert et al.   EUROPEAN HEART JOURNAL

2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

(2022) Marc Humbert et al.   EUROPEAN RESPIRATORY JOURNAL

Sotatercept for the treatment of pulmonary arterial hypertension: PULSAR open-label extension

(2022) Marc Humbert et al.   EUROPEAN RESPIRATORY JOURNAL

First histological description of pulmonary and vascular abnormalities of pulmonary hypertension associated withKDRpathogenic variant

(2022) Marianne Riou et al.   EUROPEAN RESPIRATORY JOURNAL

Pulmonary Artery Denervation for Pulmonary Arterial Hypertension: A Sham-Controlled Randomized Trial

(2022) Hang Zhang et al.   JACC-Cardiovascular Interventions

Effect of Ivermectin vs Placebo on Time to Sustained Recovery in Outpatients With Mild to Moderate COVID-19

(2022) Susanna Naggie et al.   JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION

Priorities for pulmonary hypertension research: A James Lind Alliance priority setting partnership

(2022) Jason Weatherald et al.   JOURNAL OF HEART AND LUNG TRANSPLANTATION

Risk stratification and response to therapy in patients with pulmonary arterial hypertension and comorbidities: A COMPERA analysis

(2022) Stephan Rosenkranz et al.   JOURNAL OF HEART AND LUNG TRANSPLANTATION

Cytokines as Prognostic Biomarkers in Pulmonary Arterial Hypertension

(2022) Athénaïs Boucly et al.   EUROPEAN RESPIRATORY JOURNAL

Circulating markers of inflammation and angiogenesis and clinical outcomes across subtypes of pulmonary arterial hypertension

(2022) Kellen Hirsch et al.   JOURNAL OF HEART AND LUNG TRANSPLANTATION

The evolving landscape of pulmonary arterial hypertension clinical trials

(2022) Jason Weatherald et al.   LANCET

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(2022) Tian-Yu Lian et al.   Science Bulletin

Safety and Efficacy of B-Cell Depletion with Rituximab for the Treatment of Systemic Sclerosis Associated Pulmonary Arterial Hypertension: A Multi-center, Double-blind, Randomized, Placebo-controlled Trial

(2021) Roham T Zamanian et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Sotatercept for the Treatment of Pulmonary Arterial Hypertension

(2021) Marc Humbert et al.   NEW ENGLAND JOURNAL OF MEDICINE

Switching to riociguat versus maintenance therapy with phosphodiesterase-5 inhibitors in patients with pulmonary arterial hypertension (REPLACE): a multicentre, open-label, randomised controlled trial

(2021) Marius M Hoeper et al.   Lancet Respiratory Medicine

Association Between Initial Treatment Strategy and Long-term Survival in Pulmonary Arterial Hypertension

(2021) Athénaïs Boucly et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Severe Pulmonary Arterial Hypertension Is Characterized by Increased Neutrophil Elastase and Relative Elafin Deficiency

(2021) Andrew J. Sweatt et al.   CHEST

The role of machine learning in clinical research: transforming the future of evidence generation

(2021) E. Hope Weissler et al.   Trials

The impact of comorbidities on selexipag treatment effect in patients with pulmonary arterial hypertension: insights from the GRIPHON study

(2021) Stephan Rosenkranz et al.   EUROPEAN JOURNAL OF HEART FAILURE

Mortality trends in pulmonary arterial hypertension in canada: a temporal analysis of survival per ESC/ERS Guideline Era

(2021) Jason G.E. Zelt et al.   EUROPEAN RESPIRATORY JOURNAL

Temporal trends in pulmonary arterial hypertension: Results from the COMPERA registry

(2021) Marius M. Hoeper et al.   EUROPEAN RESPIRATORY JOURNAL

Mendelian randomisation and experimental medicine approaches to IL-6 as a drug target in PAH

(2021) Mark Toshner et al.   EUROPEAN RESPIRATORY JOURNAL

External validation of a refined 4-strata risk assessment score from the French pulmonary hypertension Registry

(2021) Athénaïs Boucly et al.   EUROPEAN RESPIRATORY JOURNAL

Sex and gender in pulmonary arterial hypertension

(2021) Céline Cheron et al.   European Respiratory Review

Cellular sources of IL-6 and associations with clinical phenotypes and outcomes in PAH

(2020) Catherine E. Simpson et al.   EUROPEAN RESPIRATORY JOURNAL

Mild parenchymal lung disease and/or low diffusion capacity impacts survival and treatment response in patients diagnosed with idiopathic pulmonary arterial hypertension

(2020) Robert A. Lewis et al.   EUROPEAN RESPIRATORY JOURNAL

Phenotype and outcome of PAH patients carrying a TBX4 mutation

(2020) Pierre Thoré et al.   EUROPEAN RESPIRATORY JOURNAL

Risk assessment in pulmonary arterial hypertension: Insights from the GRIPHON study

(2020) Olivier Sitbon et al.   JOURNAL OF HEART AND LUNG TRANSPLANTATION

Drug Treatment of Pulmonary Hypertension in Children

(2020) Catherine M. Avitabile et al.   PEDIATRIC DRUGS

Intravascular Ultrasound Pulmonary Artery Denervation to Treat Pulmonary Arterial Hypertension (TROPHY1)

(2020) Alexander M.K. Rothman et al.   JACC-Cardiovascular Interventions

Targeting Transforming Growth Factor Beta Receptors in Pulmonary Hypertension

(2020) Christophe Guignabert et al.   EUROPEAN RESPIRATORY JOURNAL

Enrichment Benefits of Risk Algorithms for Pulmonary Arterial Hypertension Clinical Trials

(2020) Jacqueline V. Scott et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

United States Pulmonary Hypertension Scientific Registry

(2020) Jessica B. Badlam et al.   CHEST

Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry

(2020) Marius M. Hoeper et al.   JOURNAL OF HEART AND LUNG TRANSPLANTATION

Normal and Abnormal Relationships of Pulmonary Artery to Wedge Pressure During Exercise

(2020) Robert F. Bentley et al.   Journal of the American Heart Association

Discovery of Distinct Immune Phenotypes Using Machine Learning in Pulmonary Arterial Hypertension

(2019) Andrew J Sweatt et al.   CIRCULATION RESEARCH

Use of Balloon Atrial Septostomy in Patients with Advanced Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis

(2019) Muhammad Shahzeb Khan et al.   CHEST

New Strategies for the Conduct of Clinical Trials in Pediatric Pulmonary Arterial Hypertension: Outcome of a Multistakeholder Meeting With Patients, Academia, Industry, and Regulators, Held at the European Medicines Agency on Monday, June 12, 2017

(2019) Cécile Ollivier et al.   Journal of the American Heart Association

Macitentan for the treatment of portopulmonary hypertension (PORTICO): a multicentre, randomised, double-blind, placebo-controlled, phase 4 trial

(2019) Olivier Sitbon et al.   Lancet Respiratory Medicine

PULMONARY ARTERIAL HYPERTENSION PATIENTS WITH AND WITHOUT CARDIOVASCULAR RISK FACTORS: RESULTS FROM THE AMBITION TRIAL

(2019) Vallerie V. McLaughlin et al.   JOURNAL OF HEART AND LUNG TRANSPLANTATION

Characterization of GDF2 Mutations and Levels of BMP9 and BMP10 in Pulmonary Arterial Hypertension

(2019) Joshua Hodgson et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Chronic Inflammation Within the Vascular Wall in Pulmonary Arterial Hypertension: More than a Spectator

(2019) Alice Huertas et al.   CARDIOVASCULAR RESEARCH

The Low-Risk Profile in Pulmonary Arterial Hypertension. Time for a Paradigm Shift to Goal-oriented Clinical Trial Endpoints?

(2018) Jason Weatherald et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Predictors of Favorable Responses to Immunosuppressive Treatment in Pulmonary Arterial Hypertension Associated With Connective Tissue Disease

(2018) Hidekata Yasuoka et al.   CIRCULATION JOURNAL

Impact of age and comorbidity on risk stratification in idiopathic pulmonary arterial hypertension

(2018) Clara Hjalmarsson et al.   EUROPEAN RESPIRATORY JOURNAL

Interleukin-1 (IL-1) Blockade Reduces Inflammation in Pulmonary Arterial Hypertension and Right Ventricular Failure: A Single-Arm, Open-Label, Phase IB/II Pilot Study

(2018) Cory R Trankle et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Potts Shunt Improves Right Ventricular Function and Coupling With Pulmonary Circulation in Children With Suprasystemic Pulmonary Arterial Hypertension

(2018) Manish Aggarwal et al.   Circulation-Cardiovascular Imaging

Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives

(2018) Marc Humbert et al.   EUROPEAN RESPIRATORY JOURNAL

Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management

(2018) Erika B. Rosenzweig et al.   EUROPEAN RESPIRATORY JOURNAL

Pulmonary hypertension in chronic lung disease and hypoxia

(2018) Steven D. Nathan et al.   EUROPEAN RESPIRATORY JOURNAL

Clinical trial design and new therapies for pulmonary arterial hypertension

(2018) Olivier Sitbon et al.   EUROPEAN RESPIRATORY JOURNAL

Pathophysiology of the right ventricle and of the pulmonary circulation in pulmonary hypertension: an update

(2018) Anton Vonk Noordegraaf et al.   EUROPEAN RESPIRATORY JOURNAL

Genetic determinants of risk in pulmonary arterial hypertension: international genome-wide association studies and meta-analysis

(2018) Christopher J Rhodes et al.   Lancet Respiratory Medicine

Rare respiratory diseases are ready for primetime: from Rare Disease Day to the European Reference Networks

(2017) Marc Humbert et al.   EUROPEAN RESPIRATORY JOURNAL

Randomised placebo-controlled safety and tolerability trial of FK506 (tacrolimus) for pulmonary arterial hypertension

(2017) Edda Spiekerkoetter et al.   EUROPEAN RESPIRATORY JOURNAL

Master Protocols to Study Multiple Therapies, Multiple Diseases, or Both

(2017) Janet Woodcock et al.   NEW ENGLAND JOURNAL OF MEDICINE

Clinical phenotypes and outcomes of heritable and sporadic pulmonary veno-occlusive disease: a population-based study

(2017) David Montani et al.   Lancet Respiratory Medicine

Treatment response in patients with idiopathic pulmonary arterial hypertension and a severely reduced diffusion capacity

(2017) Cathelijne E. van der Bruggen et al.   Pulmonary Circulation

Prognostic Value of Follow-Up Hemodynamic Variables After Initial Management in Pulmonary Arterial Hypertension

(2017) Jason Weatherald et al.   CIRCULATION

Pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability

(2017) Sabine Revuz et al.   PLoS One

Bisoprolol in idiopathic pulmonary arterial hypertension: an explorative study

(2016) Jasmijn S.J.A. van Campen et al.   EUROPEAN RESPIRATORY JOURNAL

Incidence and prevalence of pulmonary arterial hypertension in Germany

(2016) Marius M. Hoeper et al.   INTERNATIONAL JOURNAL OF CARDIOLOGY

MicroRNA-140-5p and SMURF1 regulate pulmonary arterial hypertension

(2016) Alexander M.K. Rothman et al.   JOURNAL OF CLINICAL INVESTIGATION

Pre-Capillary, Combined, and Post-Capillary Pulmonary Hypertension

(2016) Christian F. Opitz et al.   JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

Adaptive Designs for Clinical Trials

(2016) Deepak L. Bhatt et al.   NEW ENGLAND JOURNAL OF MEDICINE

Estradiol improves right ventricular function in rats with severe angioproliferative pulmonary hypertension: effects of endogenous and exogenous sex hormones

(2015) Andrea L. Frump et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Elafin Reverses Pulmonary Hypertension via Caveolin-1–Dependent Bone Morphogenetic Protein Signaling

(2015) Nils P. Nickel et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Long-term safety and efficacy of imatinib in pulmonary arterial hypertension

(2015) Adaani E. Frost et al.   JOURNAL OF HEART AND LUNG TRANSPLANTATION

Balloon atrial septostomy in pulmonary arterial hypertension: Effect on survival and associated outcomes

(2015) Joanne S. Chiu et al.   JOURNAL OF HEART AND LUNG TRANSPLANTATION

Selexipag for the Treatment of Pulmonary Arterial Hypertension

(2015) Olivier Sitbon et al.   NEW ENGLAND JOURNAL OF MEDICINE

Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension

(2015) Nazzareno Galiè et al.   NEW ENGLAND JOURNAL OF MEDICINE

Effect of Macitentan on Hospitalizations

(2015) Richard N. Channick et al.   JACC-Heart Failure

Are Hemodynamics Surrogate End Points in Pulmonary Arterial Hypertension?

(2014) Corey E. Ventetuolo et al.   CIRCULATION

Exercise haemodynamics may unmask the diagnosis of diastolic dysfunction among patients with pulmonary hypertension

(2014) Elad Maor et al.   EUROPEAN JOURNAL OF HEART FAILURE

Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension

(2014) P.-Y. Courand et al.   EUROPEAN RESPIRATORY JOURNAL

Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study

(2014) O. Sitbon et al.   EUROPEAN RESPIRATORY JOURNAL

The Right Ventricle Explains Sex Differences in Survival in Idiopathic Pulmonary Arterial Hypertension

(2013) Wouter Jacobs et al.   CHEST

Efficacy and Safety of Oral Treprostinil Monotherapy for the Treatment of Pulmonary Arterial Hypertension

(2013) Zhi-Cheng Jing et al.   CIRCULATION

Imatinib Mesylate as Add-on Therapy for Pulmonary Arterial Hypertension

(2013) Marius M. Hoeper et al.   CIRCULATION

High Prevalence of Occult Pulmonary Venous Hypertension Revealed by Fluid Challenge in Pulmonary Hypertension

(2013) Ivan M. Robbins et al.   Circulation-Heart Failure

Severely reduced diffusion capacity in idiopathic pulmonary arterial hypertension: patient characteristics and treatment responses

(2013) Pia Trip et al.   EUROPEAN RESPIRATORY JOURNAL

Sex and haemodynamics in pulmonary arterial hypertension

(2013) C. E. Ventetuolo et al.   EUROPEAN RESPIRATORY JOURNAL

FK506 activates BMPR2, rescues endothelial dysfunction, and reverses pulmonary hypertension

(2013) Edda Spiekerkoetter et al.   JOURNAL OF CLINICAL INVESTIGATION

Transcatheter Potts shunt creation in patients with severe pulmonary arterial hypertension: Initial clinical experience

(2013) Jesse J. Esch et al.   JOURNAL OF HEART AND LUNG TRANSPLANTATION

Macitentan and Morbidity and Mortality in Pulmonary Arterial Hypertension

(2013) Tomás Pulido et al.   NEW ENGLAND JOURNAL OF MEDICINE

Riociguat for the Treatment of Pulmonary Arterial Hypertension

(2013) Hossein-Ardeschir Ghofrani et al.   NEW ENGLAND JOURNAL OF MEDICINE

Usefulness of Beta-Blocker Therapy and Outcomes in Patients With Pulmonary Arterial Hypertension

(2012) Petsy Pui-Sze So et al.   AMERICAN JOURNAL OF CARDIOLOGY

Potts Shunt in Children With Idiopathic Pulmonary Arterial Hypertension: Long-Term Results

(2012) Alban-Elouen Baruteau et al.   ANNALS OF THORACIC SURGERY

An Evaluation of Long-term Survival From Time of Diagnosis in Pulmonary Arterial Hypertension From the REVEAL Registry

(2012) Raymond L. Benza et al.   CHEST

Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: Results from the COMPERA registry

(2012) Marius M. Hoeper et al.   INTERNATIONAL JOURNAL OF CARDIOLOGY

Do Changes of 6-Minute Walk Distance Predict Clinical Events in Patients With Pulmonary Arterial Hypertension?

(2012) Gianluigi Savarese et al.   JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

Characteristics and Outcome After Hospitalization for Acute Right Heart Failure in Patients With Pulmonary Arterial Hypertension

(2011) François Haddad et al.   Circulation-Heart Failure

Effect of atrial septostomy on the survival of patients with severe pulmonary arterial hypertension

(2011) J. Sandoval et al.   EUROPEAN RESPIRATORY JOURNAL

Imatinib in Pulmonary Arterial Hypertension Patients with Inadequate Response to Established Therapy

(2010) Hossein A. Ghofrani et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Elevated Levels of Inflammatory Cytokines Predict Survival in Idiopathic and Familial Pulmonary Arterial Hypertension

(2010) Elaine Soon et al.   CIRCULATION

Addition of Inhaled Treprostinil to Oral Therapy for Pulmonary Arterial Hypertension

(2010) Vallerie V. McLaughlin et al.   JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

Tadalafil Therapy for Pulmonary Arterial Hypertension

(2009) Nazzareno Galiè et al.   CIRCULATION

Prognostic factors of acute heart failure in patients with pulmonary arterial hypertension

(2009) B. Sztrymf et al.   EUROPEAN RESPIRATORY JOURNAL

Iloprost for pulmonary vasodilator testing in idiopathic pulmonary arterial hypertension

(2009) Z-C. Jing et al.   EUROPEAN RESPIRATORY JOURNAL

Exercise improvement and plasma biomarker changes with intravenous treprostinil therapy for pulmonary arterial hypertension: A placebo-controlled trial

(2009) Jagdish Hiremath et al.   JOURNAL OF HEART AND LUNG TRANSPLANTATION

Platelet-derived Growth Factor Expression and Function in Idiopathic Pulmonary Arterial Hypertension

(2008) Frédéric Perros et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Immunosuppressive therapy in lupus- and mixed connective tissue disease–associated pulmonary arterial hypertension: A retrospective analysis of twenty-three cases

(2008) Xavier Jais et al.   ARTHRITIS AND RHEUMATISM

Ambrisentan for the Treatment of Pulmonary Arterial Hypertension

(2008) Nazzareno Galiè et al.   CIRCULATION