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humans (110) 110
index medicus (93) 93
pulmonary hypertension (89) 89
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1. Full Text Updated Clinical Classification of Pulmonary Hypertension
by Simonneau, Gérald, MD and Robbins, Ivan M., MD and Beghetti, Maurice, MD and Channick, Richard N., MD and Delcroix, Marion, MD, PhD and Denton, Christopher P., MD, PhD and Elliott, C. Gregory, MD and Gaine, Sean P., MD, PhD and Gladwin, Mark T., MD and Jing, Zhi-Cheng, MD and Krowka, Michael J., MD and Langleben, David, MD and Nakanishi, Norifumi, MD, PhD and Souza, Rogério, MD
JACC (Journal of the American College of Cardiology), ISSN 0735-1097, 2009, Volume 54, Issue 1, pp. S43 - S54
The aim of a clinical classification of pulmonary hypertension (PH) is to group together different manifestations of disease sharing similarities in... 
Cardiovascular | Internal Medicine | clinical classification | pulmonary arterial hypertension | pulmonary hypertension | VENOOCCLUSIVE-DISEASE | CHRONIC HEART-FAILURE | CARDIAC & CARDIOVASCULAR SYSTEMS | HEREDITARY HEMORRHAGIC TELANGIECTASIA | CONNECTIVE-TISSUE-DISEASE | SYSTEMIC-SCLEROSIS | INTERSTITIAL LUNG-DISEASE | GLYCOGEN-STORAGE-DISEASE | ARTERIAL-HYPERTENSION | SICKLE-CELL-DISEASE | OF-THE-LITERATURE | Hypertension, Pulmonary - classification | Prognosis | Comorbidity | Humans | Calcium Channel Blockers - therapeutic use | Antihypertensive Agents - administration & dosage | Anticoagulants - therapeutic use | Treatment Outcome | Hypertension, Pulmonary - epidemiology | Evidence-Based Medicine | Scleroderma, Systemic - epidemiology | Randomized Controlled Trials as Topic | Algorithms | Hypertension, Pulmonary - drug therapy | Endothelin Receptor Antagonists | Epoprostenol - administration & dosage | Hypertension | Medical colleges | Micropollutants | Sickle cell anemia | Architecture | Respiratory agents | Congenital heart disease | HIV (Viruses) | Peptide hormones | Coronary heart disease | Gene mutations | Bone morphogenetic proteins | Kidney diseases | Cardiology | Health aspects | Personal video recorders | Anemia | Pulmonary hypertension | Genetic counseling | Surgery | Classification | Clinical trials | Cardiovascular disease | Mutation | Family medical history | Drug therapy | Defects
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2. Full Text Bosentan for Treatment of Inoperable Chronic Thromboembolic Pulmonary Hypertension
by Jaïs, Xavier, MD and D'Armini, Andrea M., MD and Jansa, Pavel, MD and Torbicki, Adam, MD and Delcroix, Marion, MD and Ghofrani, Hossein A., MD and Hoeper, Marius M., MD and Lang, Irene M., MD and Mayer, Eckhard, MD and Pepke-Zaba, Joanna, MD and Perchenet, Loïc, PhD and Morganti, Adele, MSc and Simonneau, Gérald, MD and Rubin, Lewis J., MD and Benefit Study Grp
JACC (Journal of the American College of Cardiology), ISSN 0735-1097, 2008, Volume 52, Issue 25, pp. 2127 - 2134
Objectives Our goal was to investigate the effect of treatment with the oral dual endothelin receptor antagonist bosentan on the hemodynamics and exercise... 
Cardiovascular | Internal Medicine | bosentan | pulmonary endarterectomy | operability | hemodynamics | INHALED ILOPROST | CARDIAC & CARDIOVASCULAR SYSTEMS | SILDENAFIL | FOLLOW-UP | THROMBOENDARTERECTOMY | ENDARTERECTOMY | THERAPY | ARTERIAL-HYPERTENSION | OUTCOMES | UP-REGULATION | PARAMETER | Hypertension | Medical colleges | Care and treatment | Micropollutants | Endothelin | Respiratory tract diseases | Antitubercular agents | Cardiology | Public health | Personal video recorders | Natriuretic peptides | Tuberculosis | Pulmonary hypertension
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3. Full Text Long-term safety and efficacy of imatinib in pulmonary arterial hypertension
by Frost, Adaani E., MD and Barst, Robyn J., MD and Hoeper, Marius M., MD and Chang, Hyuk-Jae, MD and Frantz, Robert P., MD and Fukumoto, Yoshihiro, MD and Galié, Nazzareno, MD and Hassoun, Paul M., MD and Klose, Hans, MD and Matsubara, Hiromi, MD and Morrell, Nicholas W., MD and Peacock, Andrew J., MD and Pfeifer, Michael, MD and Simonneau, Gérald, MD and Tapson, Victor F., MD and Torres, Fernando, MD and Dario Vizza, Carmine, MD and Lawrence, David, PhD and Yang, Wei, MD and Felser, James M., MD and Quinn, Deborah A., MD and Ghofrani, Hossein-Ardeschir, MD
Journal of Heart and Lung Transplantation, ISSN 1053-2498, 2015, Volume 34, Issue 11, pp. 1366 - 1375
Background Imatinib is an oral inhibitor of several protein kinases implicated in the pathophysiology of pulmonary hypertension. Treatment with imatinib... 
Surgery | imatinib | pulmonary arterial hypertension | safety | efficacy | long-term | SURVIVAL | SURGERY | CONTROLLED-TRIAL | CARDIAC & CARDIOVASCULAR SYSTEMS | 6-MINUTE WALK TEST | TREPROSTINIL | PROSTACYCLIN | OPEN-LABEL | TRANSPLANTATION | MESYLATE | THERAPY | RESPIRATORY SYSTEM | DOUBLE-BLIND | GROWTH-FACTOR EXPRESSION | Double-Blind Method | Follow-Up Studies | Humans | Middle Aged | Hypertension, Pulmonary - physiopathology | Male | Treatment Outcome | Dose-Response Relationship, Drug | Young Adult | Protein Kinase Inhibitors - administration & dosage | Time Factors | Exercise Tolerance - physiology | Adolescent | Imatinib Mesylate - administration & dosage | Adult | Female | Aged | Hemodynamics - drug effects | Hypertension, Pulmonary - drug therapy | Retrospective Studies | Antimitotic agents | Medical colleges | Hospitals | Antineoplastic agents | Phosphotransferases | Pulmonary hypertension | Cardiovascular agents
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4. Full Text Macitentan Improves Health-Related Quality of Life for Patients With Pulmonary Arterial Hypertension
by Mehta, Sanjay, MD and Sastry, Bhagavatula Kutumba Srinivasa, MD and Souza, Rogério, MD and Torbicki, Adam, MD and Ghofrani, Hossein-Ardeschir, MD and Channick, Richard N., MD and Delcroix, Marion, MD and Pulido, Tomás, MD and Simonneau, Gérald, MD and Wlodarczyk, John, PhD and Rubin, Lewis J., MD and Jansa, Pavel, MD and Hunsche, Elke, PhD and Galiè, Nazzareno, MD and Perchenet, Loïc, PhD and Sitbon, Olivier, MD
Chest, ISSN 0012-3692, 2016, Volume 151, Issue 1, pp. 106 - 118
Background Pulmonary arterial hypertension (PAH) leads to reduced health-related quality of life (HRQoL). The objectives of this analysis were to evaluate the... 
Pulmonary/Respiratory | pulmonary arterial hypertension | pharmacotherapy | health-related quality of life | DIAGNOSIS | REPORTED OUTCOMES | THERAPY | RESPIRATORY SYSTEM | DISEASE | CRITICAL CARE MEDICINE
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5. Full Text Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases
by Dorfmüller, Peter, MD, PhD and Humbert, Marc, MD, PhD and Perros, Frédéric, MSC and Sanchez, Olivier, MD and Simonneau, Gérald, MD and Müller, Klaus-Michael, MD and Capron, Frédérique, MD, PhD
Human Pathology, ISSN 0046-8177, 2007, Volume 38, Issue 6, pp. 893 - 902
Summary Pulmonary arterial hypertension is a severe complication of connective tissue diseases. It is currently well established that pulmonary arterial... 
Pathology | Thrombotic lesion | Pulmonary arterial hypertension | Venoocclusive disease | Connective tissue diseases | Intimal fibrosis | venoocclusive disease | EPOPROSTENOL | SECONDARY | SCLEROSIS | intimal fibrosis | CAPILLARY HEMANGIOMATOSIS | PATHOLOGY | connective tissue diseases | PATHOGENESIS | SCLERODERMA | THERAPY | INFLAMMATION | PLEXIFORM LESIONS | pulmonary arterial hypertension | thrombotic lesion | Immunohistochemistry | Lung - pathology | Humans | Middle Aged | Pulmonary Veno-Occlusive Disease - etiology | Male | Connective Tissue Diseases - pathology | Adolescent | Adult | Female | Aged | Lung - blood supply | Hypertension, Pulmonary - etiology | Hypertension, Pulmonary - pathology | Connective Tissue Diseases - complications | Pulmonary Artery - pathology | Pulmonary Veno-Occlusive Disease - pathology | Hypertension | Autoantibodies | Lungs | Pulmonary arteries | Veins & arteries
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6. Full Text EPITOME-2: An open-label study assessing the transition to a new formulation of intravenous epoprostenol in patients with pulmonary arterial hypertension
by Sitbon, Olivier, MD and Delcroix, Marion, MD and Bergot, Emmanuel, MD and Boonstra, Anco B., MD and Granton, John, MD and Langleben, David, MD and Subías, Pilar Escribano, MD and Galiè, Nazzareno, MD and Pfister, Thomas, PhD and Lemarié, Jean-Christophe, MSc and Simonneau, Gérald, MD
American Heart Journal, ISSN 0002-8703, 2014, Volume 167, Issue 2, pp. 210 - 217
Background Continuous infusion of epoprostenol is the treatment of choice in patients with pulmonary arterial hypertension in functional classes III to IV.... 
Cardiovascular | SODIUM | CARDIAC & CARDIOVASCULAR SYSTEMS | NATRIURETIC PEPTIDE | TREPROSTINIL | PROSTACYCLIN | Single-Blind Method | Prospective Studies | Follow-Up Studies | Humans | Middle Aged | Antihypertensive Agents - administration & dosage | Hypertension, Pulmonary - physiopathology | Familial Primary Pulmonary Hypertension | Male | Treatment Outcome | Hemodynamics - physiology | Exercise Tolerance - drug effects | Patient Satisfaction | Dose-Response Relationship, Drug | Young Adult | Cardiac Catheterization | Adult | Female | Surveys and Questionnaires | Aged | Hemodynamics - drug effects | Hypertension, Pulmonary - drug therapy | Infusions, Intravenous | Epoprostenol - administration & dosage | Medicine, Experimental | Medical research | Care and treatment | Analysis | Pulmonary hypertension | Hypertension | Heart | Heart rate | Heart attacks | Aqueous solutions | Pulmonary arteries | Blood pressure | Drug therapy | Veins & arteries | Quality of life | Index Medicus | Abridged Index Medicus
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7. Full Text Potts Shunt in Children With Idiopathic Pulmonary Arterial Hypertension: Long-Term Results
by Baruteau, Alban-Elouen, MD and Serraf, Alain, MD, PhD and Lévy, Maryline, MD, PhD and Petit, Jérôme, MD and Bonnet, Damien, MD, PhD and Jais, Xavier, MD and Vouhé, Pascal, MD, PhD and Simonneau, Gérald, MD and Belli, Emre, MD and Humbert, Marc, MD, PhD
Annals of Thoracic Surgery, ISSN 0003-4975, 2012, Volume 94, Issue 3, pp. 817 - 824
Background Idiopathic pulmonary arterial hypertension (IPAH) remains a progressive fatal disease. Palliative Potts shunt has been proposed in children... 
Surgery | Cardiothoracic Surgery | SURVIVAL | SURGERY | CARDIAC & CARDIOVASCULAR SYSTEMS | ATRIAL SEPTOSTOMY | DISEASE MANAGEMENT | EISENMENGER-SYNDROME | HEART | LUNG TRANSPLANTATION | THERAPY | RESPIRATORY SYSTEM | EXPERIENCE | REGISTRY | INSIGHTS | Age Factors | Follow-Up Studies | Humans | Echocardiography - methods | Child, Preschool | Critical Illness | Hypertension, Pulmonary - diagnostic imaging | Infant | Male | Pulmonary Artery - surgery | Cause of Death | Hospital Mortality - trends | Blalock-Taussig Procedure - methods | Time Factors | Statistics, Nonparametric | Female | Anastomosis, Surgical - methods | Retrospective Studies | Child | Hypertension, Pulmonary - mortality | Risk Assessment | Hypertension, Pulmonary - surgery | Treatment Outcome | Aorta, Thoracic - surgery | Sex Factors | Survival Analysis | Hypertension, Pulmonary - congenital | Cohort Studies | Palliative Care - methods | Pulmonary hypertension
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8. Full Text Loss of Vascular Distensibility During Exercise Is an Early Hemodynamic Marker of Pulmonary Vascular Disease
by Lau, Edmund M.T., MD, PhD and Chemla, Denis, MD, PhD and Godinas, Laurent, MD and Zhu, Kaixian, MSc and Sitbon, Olivier, MD, PhD and Savale, Laurent, MD, PhD and Montani, David, MD, PhD and Jaïs, Xavier, MD and Celermajer, David S., MD, PhD and Simonneau, Gérald, MD and Humbert, Marc, MD, PhD and Hervé, Philippe, MD, PhD
Chest, ISSN 0012-3692, 2016, Volume 149, Issue 2, pp. 353 - 361
Background Exercise can distend the normally compliant, thin-walled pulmonary vessels. Loss of distensibility has been suggested as an early marker of... 
Pulmonary/Respiratory | exercise | pulmonary arterial hypertension | pulmonary hypertension | hemodynamics | Pulmonary arterial hypertension | Exercise | Hemodynamics | Pulmonary hypertension | DIAGNOSIS | STRESS ECHOCARDIOGRAPHY | CIRCULATION | ALTITUDE | LEVEL | FLOW | PRESSURE | RESPIRATORY SYSTEM | ARTERIAL-HYPERTENSION | CRITICAL CARE MEDICINE | Severity of Illness Index | Hypertension, Pulmonary - diagnosis | Vascular Resistance | Follow-Up Studies | Humans | Middle Aged | Hypertension, Pulmonary - physiopathology | Male | Pulmonary Artery - physiopathology | Pulmonary Circulation - physiology | Cardiac Catheterization | Exercise Test | Time Factors | Pulmonary Wedge Pressure - physiology | Female | Exercise - physiology | Retrospective Studies | Stroke (Disease) | Care and treatment | Analysis | Health aspects | Risk factors
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9. Full Text Clinical phenotypes and outcomes of heritable and sporadic pulmonary veno-occlusive disease: a population-based study
by Montani, David, MD and Girerd, Barbara, PhD and Jaïs, Xavier, MD and Levy, Marilyne, MD and Amar, David, MD and Savale, Laurent, MD and Dorfmüller, Peter, MD and Seferian, Andrei, MD and Lau, Edmund M, MD and Eyries, Mélanie, PhD and Le Pavec, Jérôme, MD and Parent, Florence, MD and Bonnet, Damien, MD and Soubrier, Florent, MD and Fadel, Elie, MD and Sitbon, Olivier, MD and Simonneau, Gérald, MD and Humbert, Marc, Prof
Lancet Respiratory Medicine, The, ISSN 2213-2600, 2017, Volume 5, Issue 2, pp. 125 - 134
Summary Background Bi-allelic mutations of the EIF2AK4 gene cause heritable pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis... 
Pulmonary/Respiratory | MODELS | GENE | RESPIRATORY SYSTEM | ARTERIAL-HYPERTENSION | MUTATION | COMPETING RISKS | CRITICAL CARE MEDICINE | Hemangioma, Capillary - therapy | Lung Neoplasms - mortality | Humans | Middle Aged | Child, Preschool | Infant | Male | Familial Primary Pulmonary Hypertension - therapy | Hemangioma, Capillary - genetics | Hypertension, Pulmonary - therapy | Young Adult | Pulmonary Veno-Occlusive Disease - genetics | Lung Transplantation | Hemangioma, Capillary - mortality | Aged, 80 and over | Adult | Female | Child | Infant, Newborn | Lung Neoplasms - genetics | Hypertension, Pulmonary - mortality | Pulmonary Veno-Occlusive Disease - therapy | Pulmonary Veno-Occlusive Disease - mortality | Protein-Serine-Threonine Kinases - genetics | Familial Primary Pulmonary Hypertension - genetics | Hypertension, Pulmonary - genetics | Treatment Outcome | Lung Neoplasms - therapy | Disease-Free Survival | Phenotype | Familial Primary Pulmonary Hypertension - mortality | Adolescent | Age of Onset | Alleles | Aged | Mutation | Genetic aspects | Research | Gene mutations | Pulmonary hypertension | Patient outcomes
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10. Full Text Usefulness of first-line combination therapy with epoprostenol and bosentan in pulmonary arterial hypertension: An observational study
by Kemp, Kristina, MD and Savale, Laurent, MD, PhD and O'Callaghan, Dermot S., MD and Jaïs, Xavier, MD and Montani, David, MD, PhD and Humbert, Marc, MD, PhD and Simonneau, Gérald, MD and Sitbon, Olivier, MD, PhD
Journal of Heart and Lung Transplantation, ISSN 1053-2498, 2012, Volume 31, Issue 2, pp. 150 - 158
Background Recent guidelines have proposed first-line combination therapy as a potential strategy for the treatment of functional class IV pulmonary arterial... 
Surgery | prostacyclin | pulmonary | endothelin receptor antagonist | combination therapy | hypertension | pulmonary arterial hypertension | observational study | SURVIVAL | SURGERY | CARDIAC & CARDIOVASCULAR SYSTEMS | SILDENAFIL | EFFICACY | TREPROSTINIL | GUIDELINES | TRANSPLANTATION | RESPIRATORY SYSTEM | RECEPTOR ANTAGONIST BOSENTAN | Severity of Illness Index | Injections, Intravenous | Humans | Middle Aged | Antihypertensive Agents - administration & dosage | Hypertension, Pulmonary - physiopathology | Familial Primary Pulmonary Hypertension | Male | Treatment Outcome | Antihypertensive Agents - therapeutic use | Case-Control Studies | Antihypertensive Agents - adverse effects | Epoprostenol - therapeutic use | Sulfonamides - therapeutic use | Epoprostenol - adverse effects | Sulfonamides - adverse effects | Adult | Female | Hypertension, Pulmonary - drug therapy | Retrospective Studies | Drug Therapy, Combination | Sulfonamides - administration & dosage | Epoprostenol - administration & dosage | Pulmonary hypertension | Endothelin
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11. Full Text Impact of High-Priority Allocation on Lung and Heart-Lung Transplantation for Pulmonary Hypertension
by Savale, Laurent, MD, PhD and Le Pavec, Jérôme, MD, PhD and Mercier, Olaf, MD, PhD and Mussot, Sacha, MD and Jaïs, Xavier, MD and Fabre, Dominique, MD and O’Connell, Caroline, MD and Montani, David, MD, PhD and Stephan, François, MD and Sitbon, Olivier, MD, PhD and Simonneau, Gérald, MD and Dartevelle, Philippe, MD and Humbert, Marc, MD, PhD and Fadel, Elie, MD, PhD
Annals of Thoracic Surgery, ISSN 0003-4975, 2017, Volume 104, Issue 2, pp. 404 - 411
Background Since 2006 and 2007, patients in France with severe pulmonary hypertension (PH) who are at imminent risk of death, despite optimal treatment in the... 
Surgery | Cardiothoracic Surgery | SURVIVAL | SURGERY | CARDIAC & CARDIOVASCULAR SYSTEMS | MANAGEMENT | SCORE | ADULT LUNG | INTERNATIONAL SOCIETY | WAITING-LIST | FAILURE | RESPIRATORY SYSTEM | ARTERIAL-HYPERTENSION | CYSTIC-FIBROSIS | REGISTRY | France - epidemiology | Survival Rate - trends | Follow-Up Studies | Heart-Lung Transplantation | Humans | Middle Aged | Hypertension, Pulmonary - surgery | Tissue and Organ Procurement | Male | Treatment Outcome | Postoperative Complications - epidemiology | Incidence | Patient Selection | Time Factors | Adult | Female | Registries | Waiting Lists | Retrospective Studies
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12. Full Text Characteristics of Pulmonary Arterial Hypertension in Affected Carriers of a Mutation Located in the Cytoplasmic Tail of Bone Morphogenetic Protein Receptor Type 2
by Girerd, Barbara, PhD and Coulet, Florence, PharmD, PhD and Jaïs, Xavier, MD and Eyries, Mélanie, PhD and Van Der Bruggen, Cathelijne, BSc and De Man, Frances, PhD and Houweling, Arjan, MD, PhD and Dorfmüller, Peter, MD, PhD and Savale, Laurent, MD, PhD and Sitbon, Olivier, MD, PhD and Vonk-Noordegraaf, Anton, MD, PhD and Soubrier, Florent, MD, PhD and Simonneau, Gérald, MD and Humbert, Marc, MD, PhD and Montani, David, MD, PhD
Chest, ISSN 0012-3692, 2015, Volume 147, Issue 5, pp. 1385 - 1394
BACKGROUND Mutations in BMPR2 encoding bone morphogenetic protein receptor type 2 (BMPRII) is the main genetic risk factor for heritable pulmonary arterial... 
Pulmonary/Respiratory | DIAGNOSIS | LONG-TERM RESPONSE | DOMAIN | BMPR2 MUTATIONS | RESPIRATORY SYSTEM | II RECEPTOR | GUIDELINES | CALCIUM-CHANNEL BLOCKERS | CLINICAL-OUTCOMES | CRITICAL CARE MEDICINE | Bone Morphogenetic Protein Receptors, Type II - genetics | Humans | Adult | Female | Heterozygote | Hypertension, Pulmonary - genetics | Male | Mutation | Cohort Studies | Bone morphogenetic proteins | Care and treatment | Research | Gene mutations | Pulmonary hypertension | Risk factors
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13. Full Text Long-term outcomes of pulmonary arterial hypertension under specific drug therapy in eisenmenger syndrome
by Hascoët, Sébastien, MD and Baruteau, Alban-Elouen, MD, PhD and Humbert, Marc, MD, PhD and Simonneau, Gérald, MD, PhD and Jais, Xavier, MD, PhD and Petit, Jérôme, MD and Laux, Daniela, MD and Sitbon, Olivier, MD, PhD and Lambert, Virginie, MD, PhD and Capderou, André, MD, PhD
Journal of Heart and Lung Transplantation, ISSN 1053-2498, 2016, Volume 36, Issue 4, pp. 386 - 398
Background The long-term effectiveness of pulmonary artery hypertension-specific drug therapy (PAH-SDT) in Eisenmenger syndrome is controversial. We... 
Surgery | catheterization | drug therapy | Eisenmenger syndrome | pulmonary arterial hypertension | outcome | congenital heart diseases | CARDIAC-OUTPUT | SURGERY | OXYGEN-CONSUMPTION | DEFECTS | CARDIAC & CARDIOVASCULAR SYSTEMS | SILDENAFIL | GUIDELINES | BOSENTAN THERAPY | CHILDREN | TRANSPLANTATION | BREATHE-5 | RESPIRATORY SYSTEM | CONGENITAL HEART-DISEASE | Vascular Resistance | Drug Administration Schedule | Humans | Middle Aged | Hypertension, Pulmonary - physiopathology | Male | Treatment Outcome | Eisenmenger Complex - complications | Eisenmenger Complex - physiopathology | Antihypertensive Agents - therapeutic use | Young Adult | Adolescent | Exercise Tolerance | Adult | Female | Hypertension, Pulmonary - drug therapy | Hypertension, Pulmonary - etiology | Cohort Studies | Drug therapy | Pulmonary hypertension | Medical research | Genetic disorders | Catheterization | Analysis | Cardiac patients | Medicine, Experimental | Transplantation of organs, tissues, etc | Congenital heart disease | Heart diseases
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