4.8 Review

Pulmonary Arterial Hypertension

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Article Cardiac & Cardiovascular Systems

A machine learning cardiac magnetic resonance approach to extract disease features and automate pulmonary arterial hypertension diagnosis

Andrew J. Swift et al.

Summary: A tensor-based machine learning approach has been developed to identify diagnostic features in PAH using CMR, showing high diagnostic accuracy and the ability to complete diagnosis quickly. New features associated with PAH were visualized with diagnostic potential.

EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING (2021)

Article Medicine, General & Internal

Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease

Aaron Waxman et al.

Summary: In patients with pulmonary hypertension due to interstitial lung disease, inhaled treprostinil significantly improved exercise capacity and clinical outcomes compared to placebo in a 16-week trial. Adverse events such as cough, headache, dyspnea, dizziness, nausea, fatigue, and diarrhea were commonly reported.

NEW ENGLAND JOURNAL OF MEDICINE (2021)

Article Medicine, General & Internal

Sotatercept for the Treatment of Pulmonary Arterial Hypertension

Marc Humbert et al.

Summary: In this study, patients with pulmonary arterial hypertension receiving Sotatercept treatment showed a significant reduction in pulmonary vascular resistance at 24 weeks, with both Sotatercept groups performing better than the placebo group.

NEW ENGLAND JOURNAL OF MEDICINE (2021)

Article Genetics & Heredity

Rare variant analysis of 4241 pulmonary arterial hypertension cases from an international consortium implicates FBLN2, PDGFD, and rare de novo variants in PAH

Na Zhu et al.

Summary: The study identified seven genes with rare deleterious variants associated with IPAH, including three known genes and two new candidate genes; analysis of pediatric IPAH showed a significant burden of de novo variants, with at least eight novel candidate genes related to lung/heart development.

GENOME MEDICINE (2021)

Letter Critical Care Medicine

Multiple Manifestations of Systemic Sclerosis Affect Walk Distance Reply

Roham T. Zamanian et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2021)

Review Cardiac & Cardiovascular Systems

Molecular genetic framework underlying pulmonary arterial hypertension

Laura Southgate et al.

NATURE REVIEWS CARDIOLOGY (2020)

Article Respiratory System

Risk stratification in pulmonary arteria hypertension using Bayesian analysis

Manreet K. Kanwar et al.

EUROPEAN RESPIRATORY JOURNAL (2020)

Review Cardiac & Cardiovascular Systems

Advanced imaging in pulmonary hypertension: emerging techniques and applications

Fabian Rengier et al.

INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING (2019)

Article Cardiac & Cardiovascular Systems

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

Andrew J. Sweatt et al.

CIRCULATION RESEARCH (2019)

Article Respiratory System

Genetics and genomics of pulmonary arterial hypertension

Nicholas W. Morrell et al.

EUROPEAN RESPIRATORY JOURNAL (2019)

Article Respiratory System

Haemodynamic definitions and updated clinical classification of pulmonary hypertension

Gerald Simonneau et al.

EUROPEAN RESPIRATORY JOURNAL (2019)

Article Respiratory System

Risk stratification and medical therapy of pulmonary arterial hypertension

Nazzareno Galie et al.

EUROPEAN RESPIRATORY JOURNAL (2019)

Article Respiratory System

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

Anton Vonk Noordegraaf et al.

EUROPEAN RESPIRATORY JOURNAL (2019)

Article Medicine, General & Internal

Schistosomiasis - Assessing Progress toward the 2020 and 2025 Global Goals

Arminder K. Deol et al.

NEW ENGLAND JOURNAL OF MEDICINE (2019)

Article Cardiac & Cardiovascular Systems

A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension

David Kylhammar et al.

EUROPEAN HEART JOURNAL (2018)

Article Cardiac & Cardiovascular Systems

REVEAL risk scores applied to riociguat-treated patients in PATENT-2: Impact of changes in risk score on survival

Raymond L. Benza et al.

JOURNAL OF HEART AND LUNG TRANSPLANTATION (2018)

Article Multidisciplinary Sciences

Identification of rare sequence variation underlying heritable pulmonary arterial hypertension

Stefan Graf et al.

NATURE COMMUNICATIONS (2018)

Review Respiratory System

Precapillary Pulmonary Hypertension and Sleep-Disordered Breathing: Is There a Link?

Robert Thurnheer et al.

RESPIRATION (2017)

Article Respiratory System

Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension

Athenais Boucly et al.

EUROPEAN RESPIRATORY JOURNAL (2017)

Article Critical Care Medicine

Plasma proteome analysis in patients with pulmonary arterial hypertension: an observational cohort study

Christopher J. Rhodes et al.

LANCET RESPIRATORY MEDICINE (2017)

Article Cardiac & Cardiovascular Systems

A Multi-Center Study to Improve Understanding of Pulmonary Vascular Disease Through Phenomics

Anna R. Hemnes et al.

CIRCULATION RESEARCH (2017)

Review Respiratory System

Pulmonary hypertension in systemic sclerosis: different phenotypes

David Launay et al.

EUROPEAN RESPIRATORY REVIEW (2017)

Letter Critical Care Medicine

Intrapulmonary Bronchopulmonary Anastomoses and Plexiform Lesions in Idiopathic Pulmonary Arterial Hypertension

Csaba Galambos et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2016)

Review Cardiac & Cardiovascular Systems

Right ventricular dysfunction in heart failure with preserved ejection fraction: a systematic review and meta-analysis

Thomas M. Gorter et al.

EUROPEAN JOURNAL OF HEART FAILURE (2016)

Review Critical Care Medicine

A global view of pulmonary hypertension

Marius M. Hoeper et al.

LANCET RESPIRATORY MEDICINE (2016)

Article Respiratory System

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

Nazzareno Galie et al.

EUROPEAN RESPIRATORY JOURNAL (2015)

Article Biochemistry & Molecular Biology

Selective enhancement of endothelial BMPR-II with BMP9 reverses pulmonary arterial hypertension

Lu Long et al.

NATURE MEDICINE (2015)

Article Medicine, General & Internal

Selexipag for the Treatment of Pulmonary Arterial Hypertension

Olivier Sitbon et al.

NEW ENGLAND JOURNAL OF MEDICINE (2015)

Article Medicine, General & Internal

Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension

N. Galie et al.

NEW ENGLAND JOURNAL OF MEDICINE (2015)

Article Cardiac & Cardiovascular Systems

RV-pulmonary arterial coupling predicts outcome in patients referred for pulmonary hypertension

Rebecca R. Vanderpool et al.

Article Rheumatology

Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study

J. Gerry Coghlan et al.

ANNALS OF THE RHEUMATIC DISEASES (2014)

Article Health Care Sciences & Services

The economic burden of pulmonary arterial hypertension (PAH) in the US on payers and patients

Mirko Sikirica et al.

BMC HEALTH SERVICES RESEARCH (2014)

Article Critical Care Medicine

EIF2AK4 Mutations in Pulmonary Capillary Hemangiomatosis

D. Hunter Best et al.

Article Cardiac & Cardiovascular Systems

Advances in Therapeutic Interventions for Patients With Pulmonary Arterial Hypertension

Marc Humbert et al.

CIRCULATION (2014)

Article Cardiac & Cardiovascular Systems

Protein Changes Contributing to Right Ventricular Cardiomyocyte Diastolic Dysfunction in Pulmonary Arterial Hypertension

Silvia Rain et al.

JOURNAL OF THE AMERICAN HEART ASSOCIATION (2014)

Article Cardiac & Cardiovascular Systems

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

Marius M. Hoeper et al.

INTERNATIONAL JOURNAL OF CARDIOLOGY (2013)

Article Medicine, Research & Experimental

FK506 activates BMPR2 rescues endothelial dysfunction, and reverses pulmonary hypertension

Edda Spiekerkoetter et al.

JOURNAL OF CLINICAL INVESTIGATION (2013)

Article Cardiac & Cardiovascular Systems

Accurate assessment of load-independent right ventricular systolic function in patients with pulmonary hypertension

Pia Trip et al.

JOURNAL OF HEART AND LUNG TRANSPLANTATION (2013)

Article Genetics & Heredity

TBX4 mutations (small patella syndrome) are associated with childhood-onset pulmonary arterial hypertension

Wilhelmina S. Kerstjens-Frederikse et al.

JOURNAL OF MEDICAL GENETICS (2013)

Article Medicine, General & Internal

Macitentan and Morbidity and Mortality in Pulmonary Arterial Hypertension

Tomas Pulido et al.

NEW ENGLAND JOURNAL OF MEDICINE (2013)

Article Medicine, General & Internal

A Novel Channelopathy in Pulmonary Arterial Hypertension

Lijiang Ma et al.

NEW ENGLAND JOURNAL OF MEDICINE (2013)

Review Respiratory System

The need to move from 6-minute walk distance to outcome trials in pulmonary arterial hypertension

Sean Gaine et al.

EUROPEAN RESPIRATORY REVIEW (2013)

Article Critical Care Medicine

Pulmonary Lymphoid Neogenesis in Idiopathic Pulmonary Arterial Hypertension

Frederic Perros et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2012)

Article Critical Care Medicine

Inflammation in Pulmonary Arterial Hypertension

Laura C. Price et al.

Article Cardiac & Cardiovascular Systems

Whole Exome Sequencing to Identify a Novel Gene (Caveolin-1) Associated With Human Pulmonary Arterial Hypertension

Eric D. Austin et al.

CIRCULATION-CARDIOVASCULAR GENETICS (2012)

Article Critical Care Medicine

Sex Hormones Are Associated with Right Ventricular Structure and Function The MESA-Right Ventricle Study

Corey E. Ventetuolo et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2011)

Article Respiratory System

Outcomes of hospitalisation for right heart failure in pulmonary arterial hypertension

A. Campo et al.

EUROPEAN RESPIRATORY JOURNAL (2011)

Article Cardiac & Cardiovascular Systems

A meta-analysis of randomized controlled trials in pulmonary arterial hypertension

Nazzareno Galie et al.

EUROPEAN HEART JOURNAL (2009)

Article Cardiac & Cardiovascular Systems

Inflammation, Growth Factors, and Pulmonary Vascular Remodeling

Paul M. Hassoun et al.

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY (2009)

Article Cardiac & Cardiovascular Systems

Evaluation of Pulmonary Artery Stiffness in Pulmonary Hypertension With Cardiac Magnetic Resonance

Javier Sanz et al.

JACC-CARDIOVASCULAR IMAGING (2009)

Article Critical Care Medicine

Pulmonary arterial hypertension in France - Results from a national registry

M Humbert et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2006)

Article Cardiac & Cardiovascular Systems

Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension

O Sitbon et al.

CIRCULATION (2005)

Article Cardiac & Cardiovascular Systems

Transforming growth factor-β receptor mutations and pulmonary arterial hypertension in childhood

RE Harrison et al.

CIRCULATION (2005)

Review Cardiac & Cardiovascular Systems

Endothelial dysfunction in pulmonary hypertension

R Budhiraja et al.

CIRCULATION (2004)

Article Medicine, General & Internal

Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia

RC Trembath et al.

NEW ENGLAND JOURNAL OF MEDICINE (2001)