pulmonary hypertension

Summary

Summary: Increased VASCULAR RESISTANCE in the PULMONARY CIRCULATION, usually secondary to HEART DISEASES or LUNG DISEASES.

Top Publications

  1. ncbi Updated clinical classification of pulmonary hypertension
    Gerald Simonneau
    Centre National de Référence des Maladies Vasculaires Pulmonaires, Universite Paris Sud, Hopital Antoine Beclere, Clamart, France
    J Am Coll Cardiol 54:S43-54. 2009
  2. ncbi Pulmonary arterial hypertension in France: results from a national registry
    Marc Humbert
    UPRES EA 2705, Service de Pneumologie et Reanimation Respiratoire, Hopital Antoine Beclere, Assistance Publique Hopitaux de Paris, Universite Paris Sud, 157 rue de la Porte de Trivaux, 92140 Clamart, France
    Am J Respir Crit Care Med 173:1023-30. 2006
  3. ncbi Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension
    K B Lane
    Vanderbilt University Medical Center, Nashville, Tennessee, USA
    Nat Genet 26:81-4. 2000
  4. ncbi Cellular and molecular pathobiology of pulmonary arterial hypertension
    Marc Humbert
    Service de Pneumologie et Reanimation Respiratoire, Centre des Maladies Vasculaires Pulmonaires, UPRES EA2705, Hopital Antoine Beclere, Universite Paris Sud, Clamart, France
    J Am Coll Cardiol 43:13S-24S. 2004
  5. ncbi Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial
    Gerald Simonneau
    Division of Pulmonary and Critical Care Medicine, Antoine Beclere Hospital, Clamart, Paris Sud University, Clamart, France
    Am J Respir Crit Care Med 165:800-4. 2002
  6. ncbi Pulmonary arterial hypertension
    Harrison W Farber
    Evans Department of Medicine, Boston University School of Medicine, Boston, MA 02118, USA
    N Engl J Med 351:1655-65. 2004
  7. ncbi Primary pulmonary hypertension is associated with reduced pulmonary vascular expression of type II bone morphogenetic protein receptor
    Carl Atkinson
    Department of Medicine, University of Cambridge School of Clinical Medicine, Addenbrooke s Hospital, Cambridge, UK
    Circulation 105:1672-8. 2002
  8. ncbi Detection of pulmonary hypertension with multidetector CT and echocardiography alone and in combination
    Anand Devaraj
    Department of Radiology, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, England
    Radiology 254:609-16. 2010
  9. ncbi Dynamic changes in lung microRNA profiles during the development of pulmonary hypertension due to chronic hypoxia and monocrotaline
    Paola Caruso
    Division of Cardiovascular and Medical Sciences, British Heart Foundation Glasgow Cardiovascular Research Centre, 126 University Ave, University of Glasgow, Glasgow, Scotland
    Arterioscler Thromb Vasc Biol 30:716-23. 2010
  10. ncbi Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study
    Eric Hachulla
    Hopital Claude Huriez, Lille, France
    Arthritis Rheum 52:3792-800. 2005

Detail Information

Publications311 found, 100 shown here

  1. ncbi Updated clinical classification of pulmonary hypertension
    Gerald Simonneau
    Centre National de Référence des Maladies Vasculaires Pulmonaires, Universite Paris Sud, Hopital Antoine Beclere, Clamart, France
    J Am Coll Cardiol 54:S43-54. 2009
    The aim of a clinical classification of pulmonary hypertension (PH) is to group together different manifestations of disease sharing similarities in pathophysiologic mechanisms, clinical presentation, and therapeutic approaches...
  2. ncbi Pulmonary arterial hypertension in France: results from a national registry
    Marc Humbert
    UPRES EA 2705, Service de Pneumologie et Reanimation Respiratoire, Hopital Antoine Beclere, Assistance Publique Hopitaux de Paris, Universite Paris Sud, 157 rue de la Porte de Trivaux, 92140 Clamart, France
    Am J Respir Crit Care Med 173:1023-30. 2006
    ..Pulmonary arterial hypertension (PAH) is an orphan disease for which the trend is for management in designated centers with multidisciplinary teams working in a shared-care approach...
  3. ncbi Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension
    K B Lane
    Vanderbilt University Medical Center, Nashville, Tennessee, USA
    Nat Genet 26:81-4. 2000
    Primary pulmonary hypertension (PPH), characterized by obstruction of pre-capillary pulmonary arteries, leads to sustained elevation of pulmonary arterial pressure (mean >25 mm Hg at rest or >30 mm Hg during exercise)...
  4. ncbi Cellular and molecular pathobiology of pulmonary arterial hypertension
    Marc Humbert
    Service de Pneumologie et Reanimation Respiratoire, Centre des Maladies Vasculaires Pulmonaires, UPRES EA2705, Hopital Antoine Beclere, Universite Paris Sud, Clamart, France
    J Am Coll Cardiol 43:13S-24S. 2004
    ..Disordered proteolysis of the extracellular matrix is also evident in PAH. Future studies are required to find which if any of these abnormalities initiates PAH and which ones are best targeted to cure the disease...
  5. ncbi Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial
    Gerald Simonneau
    Division of Pulmonary and Critical Care Medicine, Antoine Beclere Hospital, Clamart, Paris Sud University, Clamart, France
    Am J Respir Crit Care Med 165:800-4. 2002
    ..Concomitantly, treprostinil significantly improved indices of dyspnea, signs and symptoms of pulmonary hypertension, and hemodynamics...
  6. ncbi Pulmonary arterial hypertension
    Harrison W Farber
    Evans Department of Medicine, Boston University School of Medicine, Boston, MA 02118, USA
    N Engl J Med 351:1655-65. 2004
  7. ncbi Primary pulmonary hypertension is associated with reduced pulmonary vascular expression of type II bone morphogenetic protein receptor
    Carl Atkinson
    Department of Medicine, University of Cambridge School of Clinical Medicine, Addenbrooke s Hospital, Cambridge, UK
    Circulation 105:1672-8. 2002
    Mutations in the type II receptor for bone morphogenetic protein (BMPR-II), a receptor member of the transforming growth factor-beta (TGF-beta) superfamily, underlie many familial and sporadic cases of primary pulmonary hypertension (PPH).
  8. ncbi Detection of pulmonary hypertension with multidetector CT and echocardiography alone and in combination
    Anand Devaraj
    Department of Radiology, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, England
    Radiology 254:609-16. 2010
    To test the reliability of potentially new computed tomographic (CT) indicators of pulmonary hypertension (PH) and to establish whether a combination of CT and echocardiographic measurements was more predictive of PH than either test alone...
  9. ncbi Dynamic changes in lung microRNA profiles during the development of pulmonary hypertension due to chronic hypoxia and monocrotaline
    Paola Caruso
    Division of Cardiovascular and Medical Sciences, British Heart Foundation Glasgow Cardiovascular Research Centre, 126 University Ave, University of Glasgow, Glasgow, Scotland
    Arterioscler Thromb Vasc Biol 30:716-23. 2010
    ..Each miRNA is capable of potentially controlling hundreds of genes. The regulation of miRNAs in the lung during the development of pulmonary arterial hypertension (PAH) is unknown...
  10. ncbi Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study
    Eric Hachulla
    Hopital Claude Huriez, Lille, France
    Arthritis Rheum 52:3792-800. 2005
    ....
  11. pmc Genomewide RNA expression profiling in lung identifies distinct signatures in idiopathic pulmonary arterial hypertension and secondary pulmonary hypertension
    Revathi Rajkumar
    Cardiovascular Institute, Univ of Pittsburgh, PA 15213 2582, USA
    Am J Physiol Heart Circ Physiol 298:H1235-48. 2010
    ..aimed to identify genes involved specifically in the pathogenesis of PAH and not other forms of pulmonary hypertension (PH)...
  12. ncbi Tadalafil therapy for pulmonary arterial hypertension
    Nazzareno Galie
    Institute of Cardiology, University of Bologna, Via Massarenti 9, 40138 Bologna, Italy
    Circulation 119:2894-903. 2009
    ..Treatment options for pulmonary arterial hypertension target the prostacyclin, endothelin, or nitric oxide pathways. Tadalafil, a phosphodiesterase type-5 inhibitor, increases cGMP, the final mediator in the nitric oxide pathway...
  13. pmc Resveratrol prevents monocrotaline-induced pulmonary hypertension in rats
    Anna Csiszar
    Reynolds Oklahoma Center on Aging, Department of Geriatric Medicine, University of Oklahoma Health Science Center, Oklahoma City, OK 73104, USA
    Hypertension 54:668-75. 2009
    ..muscle cells, endothelial dysfunction, oxidative stress, and inflammation promotes the development of pulmonary hypertension. Resveratrol is a polyphenolic compound that exerts antioxidant and anti-inflammatory protective effects ..
  14. ncbi Pulmonary hypertension in COPD
    A Chaouat
    Dept for Respiratory Diseases and Respiratory Intensive Care, Centre Hospitalier Universitaire de Nancy, Vandoeuvre les Nancy, France
    Eur Respir J 32:1371-85. 2008
    Mild-to-moderate pulmonary hypertension is a common complication of chronic obstructive pulmonary disease (COPD); such a complication is associated with increased risks of exacerbation and decreased survival...
  15. ncbi Health-related quality of life in patients with pulmonary arterial hypertension
    Shirin Shafazand
    The George Washington University, Washington Hospital Center, Washington, DC, USA
    Chest 126:1452-9. 2004
    ..This study describes HRQOL in a cohort of patients with PAH...
  16. ncbi Inflammation in pulmonary arterial hypertension
    P Dorfmuller
    Centre for Pulmonary Vascular Diseases UPRES EA 2705, Dept of Respiratory and Intensive Care Medicine, South Paris Cytokine Institute, Antoine Beclere Hospital, Assistance Publique Hopitaux de Paris, South Paris University, Clamart, France
    Eur Respir J 22:358-63. 2003
    Inflammatory mechanisms appear to play a significant role in some types of pulmonary hypertension (PH), including monocrotaline-induced PH in rats and pulmonary arterial hypertension of various origins in humans, such as connective tissue ..
  17. ncbi Inhaled iloprost for severe pulmonary hypertension
    Horst Olschewski
    Department of Internal Medicine II, University Clinic, Giessen, Germany
    N Engl J Med 347:322-9. 2002
    Uncontrolled studies suggested that aerosolized iloprost, a stable analogue of prostacyclin, causes selective pulmonary vasodilatation and improves hemodynamics and exercise capacity in patients with pulmonary hypertension.
  18. pmc Reversal of experimental pulmonary hypertension by PDGF inhibition
    Ralph Theo Schermuly
    Department of Internal Medicine, Justus Liebig University Giessen, Giessen, Germany
    J Clin Invest 115:2811-21. 2005
    Progression of pulmonary hypertension is associated with increased proliferation and migration of pulmonary vascular smooth muscle cells. PDGF is a potent mitogen and involved in this process...
  19. pmc Rosiglitazone attenuates chronic hypoxia-induced pulmonary hypertension in a mouse model
    Rachel E Nisbet
    Department of Medicine, Atlanta Veterans Affairs and Emory University Medical Centers, Atlanta, Georgia 30033, USA
    Am J Respir Cell Mol Biol 42:482-90. 2010
    Chronic hypoxia contributes to pulmonary hypertension through complex mechanisms that include enhanced NADPH oxidase expression and reactive oxygen species (ROS) generation in the lung...
  20. ncbi Limitations to the 6-minute walk test in interstitial lung disease and pulmonary hypertension in scleroderma
    Margaret C Garin
    Washington University School of Medicine, St Louis, MO, USA
    J Rheumatol 36:330-6. 2009
    To determine factors that influence 6-minute walk distance (6MWD) in patients with scleroderma (systemic sclerosis, SSc)-interstitial lung disease (ILD), SSc-pulmonary hypertension (PH), and idiopathic pulmonary fibrosis (IPF).
  21. pmc Endothelial-derived FGF2 contributes to the progression of pulmonary hypertension in humans and rodents
    Mohamed Izikki
    INSERM U841, Faculte de Medecine, 8 Avenue du General Sarrail, 94010 Creteil, France
    J Clin Invest 119:512-23. 2009
    b>Pulmonary hypertension (PH) is a progressive, lethal lung disease characterized by pulmonary artery SMC (PA-SMC) hyperplasia leading to right-sided heart failure...
  22. ncbi Epidemiology of pulmonary hypertension: new data from the Swiss registry
    Claudia Tueller
    Pneumology, University Hospital, Berne, Switzerland
    Swiss Med Wkly 138:379-84. 2008
    since 1999 data from pulmonary hypertension (PH) patients from all PH centres in Switzerland were prospectively collected. We analyse the epidemiological aspects of these data.
  23. ncbi Tricuspid annular displacement predicts survival in pulmonary hypertension
    Paul R Forfia
    Division of Pulmonary and Critical Care Medicine, Johns Hopkins Hospital, Asthma and Allergy Center, Baltimore, MD 21224, USA
    Am J Respir Crit Care Med 174:1034-41. 2006
    Right ventricular (RV) function is an important determinant of prognosis in pulmonary hypertension. However, noninvasive assessment of the RV function is often limited by complex geometry and poor endocardial definition.
  24. ncbi Right ventricular function and failure: report of a National Heart, Lung, and Blood Institute working group on cellular and molecular mechanisms of right heart failure
    Norbert F Voelkel
    Pulmonary Hypertension Center, University of Colorado at Denver and Health Sciences Center, 4200 E Ninth Ave, MC C272, Denver, CO 80262, USA
    Circulation 114:1883-91. 2006
  25. ncbi Peroxisome proliferator-activated receptor gamma (PPARgamma) expression is decreased in pulmonary hypertension and affects endothelial cell growth
    Shingo Ameshima
    Pulmonary Hypertension Center, University of Colorado Health Sciences Center, Denver, Colo, USA
    Circ Res 92:1162-9. 2003
    ..We conclude that fluid shear stress decreases the expression of PPARgamma in endothelial cells and that loss of PPARgamma expression characterizes an abnormal, proliferating, apoptosis-resistant endothelial cell phenotype...
  26. pmc Obstructive sleep apnea, cardiovascular disease, and pulmonary hypertension
    Jason M Golbin
    Division of Pulmonary, Mayo Clinic, Rochester, MN 55905, USA
    Proc Am Thorac Soc 5:200-6. 2008
    ..with multiple disorders, such as systemic hypertension, heart failure, cardiac arrhythmias, and pulmonary hypertension. Further data in the form of longitudinal cohort studies and randomized controlled trials are accruing to ..
  27. ncbi Epidermal growth factor receptor blockade mediates smooth muscle cell apoptosis and improves survival in rats with pulmonary hypertension
    Sandra L Merklinger
    Department of Pediatrics, Stanford University School of Medicine, Stanford, CA, USA
    Circulation 112:423-31. 2005
    ..This suggests that blockade of these downstream effectors may also induce regression of PAH...
  28. ncbi The right ventricle under pressure: cellular and molecular mechanisms of right-heart failure in pulmonary hypertension
    Harm J Bogaard
    Department of Pulmonary Medicine, VU University Medical Center, Amsterdam, The Netherlands
    Chest 135:794-804. 2009
    ..with the online supplement, it provides a starting point for new research and aims to encourage the pulmonary hypertension research community to direct some of their attention to the RV, in parallel to their focus on the ..
  29. ncbi The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR): a measure of health-related quality of life and quality of life for patients with pulmonary hypertension
    S P McKenna
    Galen Research, Enterprise House, Manchester Science Park, Lloyd Street North, Manchester M15 6SE, UK
    Qual Life Res 15:103-15. 2006
    No outcome measures specific to pulmonary hypertension (PH) currently exist...
  30. pmc Pathology of pulmonary hypertension
    Rubin M Tuder
    Division of Cardiopulmonary Pathology, Johns Hopkins University School of Medicine, 720 Rutland Avenue, Ross Research Building, Baltimore, MD 21217, USA
    Clin Chest Med 28:23-42, vii. 2007
    The secondary role of pathology in the present clinical management of pulmonary hypertension (PH) reflects to some extent the limitations of the current understanding of the disease...
  31. pmc Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-beta family
    J R Thomson
    Division of Medical Genetics, Departments of Medicine and Genetics, University of Leicester, UK
    J Med Genet 37:741-5. 2000
    Primary pulmonary hypertension (PPH), resulting from occlusion of small pulmonary arteries, is a devastating condition...
  32. pmc Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension
    Micah R Fisher
    Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University, Baltimore, Maryland, USA
    Am J Respir Crit Care Med 179:615-21. 2009
    Transthoracic Doppler echocardiography is recommended for screening for the presence of pulmonary hypertension (PH)...
  33. ncbi A randomised, controlled trial of bosentan in severe COPD
    D Stolz
    Clinic of Pulmonary Medicine and Respiratory Cell Research, University Hospital Basel, Petersgraben 4, CH 4031 Basel, Switzerland
    Eur Respir J 32:619-28. 2008
    b>Pulmonary hypertension during exercise is common in severe chronic obstructive pulmonary disease (COPD)...
  34. ncbi Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia
    R C Trembath
    Department of Medicine, University of Leicester, United Kingdom
    N Engl J Med 345:325-34. 2001
    Most patients with familial primary pulmonary hypertension have defects in the gene for bone morphogenetic protein receptor II (BMPR2), a member of the transforming growth factor beta (TGF-beta) superfamily of receptors...
  35. pmc BMPR2 haploinsufficiency as the inherited molecular mechanism for primary pulmonary hypertension
    R D Machado
    Division of Medical Genetics, Departments of Medicine and Genetics, University of Leicester, Leicester, England LE1 7RH
    Am J Hum Genet 68:92-102. 2001
    Primary pulmonary hypertension (PPH) is a potentially lethal disorder, because the elevation of the pulmonary arterial pressure may result in right-heart failure...
  36. ncbi Rescue of monocrotaline-induced pulmonary arterial hypertension using bone marrow-derived endothelial-like progenitor cells: efficacy of combined cell and eNOS gene therapy in established disease
    Yidan D Zhao
    Terrence Donnelly Vascular Biology Laboratories, St Michael s Hospital and the McLaughlin Center for Molecular Medicine, University of Toronto, Canada
    Circ Res 96:442-50. 2005
    ..Therefore, the regeneration of lung vascular endothelium by injection of progenitor cells may represent a novel treatment paradigm for patients with PAH...
  37. pmc S100A4/Mts1 produces murine pulmonary artery changes resembling plexogenic arteriopathy and is increased in human plexogenic arteriopathy
    Steven Greenway
    Division of Cardiovascular Research, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
    Am J Pathol 164:253-62. 2004
    ..analyze S100A4/Mts1 expression in pulmonary arteries in surgical lung biopsies from children with pulmonary hypertension secondary to congenital heart disease...
  38. ncbi End-tidal PCO2 abnormality and exercise limitation in patients with primary pulmonary hypertension
    Yuji Yasunobu
    Department of Medicine, Los Angeles Biomedical Research Institute, Harbor UCLA Medical Center, Torrance, CA 90509 2910, USA
    Chest 127:1637-46. 2005
    Primary pulmonary hypertension (PPH) is a pulmonary vasculopathy resulting in exercise intolerance, usually due to dyspnea...
  39. ncbi Interleukin-6 overexpression induces pulmonary hypertension
    M Kathryn Steiner
    Division of Pulmonary Critical Care Medicine, University of Massachusetts Memorial Medical Center, Worcester, MA 01655, USA
    Circ Res 104:236-44, 28p following 244. 2009
    ..These findings suggest that IL-6 promotes the development and progression of pulmonary vascular remodeling and PAH through proproliferative antiapoptotic mechanisms...
  40. ncbi Expression of angiogenesis-related molecules in plexiform lesions in severe pulmonary hypertension: evidence for a process of disordered angiogenesis
    R M Tuder
    Department of Pathology, Division of Pulmonary Sciences and Critical Care Medicine, Pulmonary Hypertension Center, University of Colorado Health Sciences Center, Colorado, USA
    J Pathol 195:367-74. 2001
    Pulmonary arteries of patients with severe pulmonary hypertension (SPH) presenting in an idiopathic form (primary PH-PPH) or associated with congenital heart malformations or collagen vascular diseases show plexiform lesions...
  41. pmc An antiproliferative BMP-2/PPARgamma/apoE axis in human and murine SMCs and its role in pulmonary hypertension
    Georg Hansmann
    Department of Pediatrics, Stanford University School of Medicine, Stanford, California 94305 5162, USA
    J Clin Invest 118:1846-57. 2008
    ..Thus, PPARgamma-mediated events could protect against PAH, and PPARgamma agonists may reverse PAH in patients with or without BMP-RII dysfunction...
  42. ncbi Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing
    Roland Wensel
    Department of Cardiothoracic Surgery, Deutsches Herzzentrum Berlin, Germany
    Circulation 106:319-24. 2002
    Primary pulmonary hypertension (PPH) is a life-threatening disease. Prognostic assessment is an important factor in determining medical treatment and lung transplantation...
  43. ncbi Endothelial dysfunction in pulmonary hypertension
    Rohit Budhiraja
    Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, Tufts New England Medical Center, Tufts University School of Medicine, Boston, Mass, USA
    Circulation 109:159-65. 2004
  44. ncbi Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease
    Selim M Arcasoy
    Division of Pulmonary, Allergy and Critical Care, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA
    Am J Respir Crit Care Med 167:735-40. 2003
    Doppler echocardiography is commonly used to estimate systolic pulmonary artery pressure and to diagnose pulmonary hypertension, but data relating to its utility in patients with advanced lung disease are limited...
  45. ncbi Sustained improvement with iloprost in a COPD patient with severe pulmonary hypertension
    Matthew J Hegewald
    Division of Pulmonary and Critical Care Medicine Intermountain Medical Center, University of Utah School of Medicine, Salt Lake City, UT 84157 7000, USA
    Chest 135:536-7. 2009
    b>Pulmonary hypertension is an important complication of COPD. A small subset of patients with COPD have severe pulmonary hypertension (PH) that is out of proportion to the mild increase in pulmonary arterial pressure observed commonly...
  46. pmc Genetics and genomics of pulmonary arterial hypertension
    Rajiv D Machado
    Department of Medical and Molecular Genetics, King s College London School of Medicine, Guy s Hospital, London, United Kingdom
    J Am Coll Cardiol 54:S32-42. 2009
    ....
  47. pmc The cancer paradigm of severe pulmonary arterial hypertension
    Pradeep R Rai
    Department of Pathology, University of Colorado at Denver and Health Sciences Center, Denver, Colorado, USA
    Am J Respir Crit Care Med 178:558-64. 2008
    ..The concept of the endothelial cell as a "quasi-malignant" cell provides a new framework for antiproliferative, antiangiogenic therapy in severe PAH...
  48. ncbi Exercise pathophysiology in patients with primary pulmonary hypertension
    X G Sun
    Department of Medicine, Harbor-UCLA Medical Center, Torrance, California, USA
    Circulation 104:429-35. 2001
    BACKGROUND: Patients with primary pulmonary hypertension (PPH) have a pulmonary vasculopathy that leads to exercise intolerance due to dyspnea and fatigue...
  49. ncbi Diagnosis and differential assessment of pulmonary arterial hypertension
    Robyn J Barst
    Columbia University College of Physicians and Surgeons, New York, New York, USA
    J Am Coll Cardiol 43:40S-47S. 2004
    ..that are essential for making the diagnosis, and by additional tests to clarify the category of pulmonary hypertension (PH)...
  50. pmc Defects in caveolin-1 cause dilated cardiomyopathy and pulmonary hypertension in knockout mice
    You Yang Zhao
    University of California at San Diego, Institute of Molecular Medicine, 9500 Gilman Drive, BSB 0623, La Jolla, CA 92093, USA
    Proc Natl Acad Sci U S A 99:11375-80. 2002
    ..of pulmonary artery pressure and histological analysis revealed that the cav-1(-/-) mice exhibit pulmonary hypertension, which may contribute to the right ventricle hypertrophy...
  51. ncbi Increased CD62e(+) endothelial microparticle levels predict poor outcome in pulmonary hypertension patients
    Nicolas Amabile
    Department of Medicine, Division of Cardiology, University of California at San Francisco, San Francisco, California, USA
    J Heart Lung Transplant 28:1081-6. 2009
    ..and leukocytes-derived microparticles (EMPs and LMPs, respectively) are increased in patients with pulmonary hypertension (PH). We hypothesized that the levels of circulating EMPs and LMPs could predict outcome in these patients.
  52. ncbi Obstructive sleep apnea and pulmonary hypertension
    Dimitar Sajkov
    Flinders Medical Center, Bedford Park, SA, Australia
    Prog Cardiovasc Dis 51:363-70. 2009
    ..hypoxia for several hours per day to mimic OSA developed pulmonary vascular remodeling and sustained pulmonary hypertension and right ventricular hypertrophy within a few weeks...
  53. ncbi An epidemiological study of pulmonary arterial hypertension
    A J Peacock
    Scottish Pulmonary Vascular Unit, Western Infirmary, Glasgow, G61 2SW, UK
    Eur Respir J 30:104-9. 2007
    ..Scottish Morbidity Record scheme, data from all adults aged 16-65 yrs admitted with PAH (idiopathic PAH, pulmonary hypertension associated with congenital heart abnormalities and pulmonary hypertension associated with connective ..
  54. ncbi Advances in understanding the pathogenesis of chronic thromboembolic pulmonary hypertension
    Irene Lang
    Division of Cardiology, Department of Internal Medicine II, Vienna General Hospital, Medical University of Vienna, Vienna, Austria
    Br J Haematol 149:478-83. 2010
    Chronic thromboembolic pulmonary hypertension (CTEPH) comprises organizing thrombotic obstructions in the pulmonary arteries. While roughly 40% of CTEPH cases are not preceded by a venous thromboembolic event, 0.1-5...
  55. ncbi Combined tyrosine and serine/threonine kinase inhibition by sorafenib prevents progression of experimental pulmonary hypertension and myocardial remodeling
    Martina Klein
    Cardiology Research, Bayer Schering Pharma, Wuppertal, Germany
    Circulation 118:2081-90. 2008
    ..growth factor receptor, can reduce pulmonary arterial pressure in experimental and clinical pulmonary hypertension. We hypothesized that inhibition of the serine/threonine kinases Raf-1 (also termed c-Raf) and b-Raf in ..
  56. pmc Irreversible pulmonary hypertension associated with the use of interferon alpha for chronic hepatitis C
    Sonu Dhillon
    Department of Medicine, Rush University Medical Center, Ste 158 Professional Building, Chicago, IL 60612, USA
    Dig Dis Sci 55:1785-90. 2010
    ..Pulmonary side effects including pneumonitis, pulmonary fibrosis, and reversible pulmonary hypertension have been reported...
  57. ncbi Opposite effects of training in rats with stable and progressive pulmonary hypertension
    M L Handoko
    Department of Pulmonology, VU University Medical Center Institute for Cardiovascular Research, Amsterdam, The Netherlands
    Circulation 120:42-9. 2009
    ..However, it is still unclear whether training is beneficial for all PH patients. We hypothesized that right ventricular adaptation plays a pivotal role in the response to training...
  58. ncbi Impact of pulmonary hypertension on gas exchange and exercise capacity in patients with pulmonary fibrosis
    Sven Glaser
    Division of Pulmonary Medicine and Infectious Diseases, University of Greifswald, Sauerbruchstrasse, 17475 Greifswald, Germany
    Respir Med 103:317-24. 2009
    b>Pulmonary hypertension is a relevant interceding morbidity in patients with pulmonary fibrosis that has significant impact on exercise tolerance and outcome...
  59. ncbi Circulating endothelial cells: a new candidate biomarker of irreversible pulmonary hypertension secondary to congenital heart disease
    David M Smadja
    Paris Descartes University, Faculty of Pharmacy, INSERM U765, Paris, France
    Circulation 119:374-81. 2009
    ..The objective of the present study was to identify noninvasive biomarkers of endothelial turnover that could be used to identify congenital heart disease patients at risk of irreversible PAH...
  60. ncbi Selected Contribution: Pulmonary hypertension in mice following intermittent hypoxia
    K A Fagan
    Cardiovascular Pulmonary Research Laboratory, Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, Denver, Colorado 80262, USA
    J Appl Physiol (1985) 90:2502-7. 2001
    ..with systemic hypertension and increased mortality from cardiovascular disease, but the relationship to pulmonary hypertension is uncertain...
  61. ncbi Safety and efficacy of IV treprostinil for pulmonary arterial hypertension: a prospective, multicenter, open-label, 12-week trial
    Victor F Tapson
    Division of Pulmonary and Critical Care Medicine, Room 351 Bell Building, Duke University Medical Center, Durham, NC 27710, USA
    Chest 129:683-8. 2006
    ..With continuous IV treprostinil having potential advantages over both of the above therapies, we investigated the safety and efficacy of this regimen in patients with PAH...
  62. pmc Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension
    Anastasia Anthi
    Vascular Medicine Branch, Clinical Center, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 2089, USA
    Am J Respir Crit Care Med 175:1272-9. 2007
    Although pulmonary hypertension (PH) is a common complication of sickle cell disease (SCD) associated with high mortality, there exist few data characterizing hemodynamics and cardiopulmonary function in this population.
  63. ncbi Autoimmunity and pulmonary hypertension: a perspective
    M R Nicolls
    Department of Medicine, University of Colorado Health Sciences Center, Denver, CO 80262, USA
    Eur Respir J 26:1110-8. 2005
    ..Two striking similarities between conditions associated with severe angioproliferative pulmonary hypertension are a defect in the CD4 T-cell compartment and auto-antibody production...
  64. ncbi Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006
    S G Haworth
    UCL Institute of Child Health, University College London, 30 Guilford Street, London, UK
    Heart 95:312-7. 2009
    A retrospective study of the UK Pulmonary Hypertension Service for Children for the first 5-year period of its existence.
  65. pmc The role of NADPH oxidase in chronic intermittent hypoxia-induced pulmonary hypertension in mice
    Rachel E Nisbet
    Department of Medicine, Atlanta Veterans Affairs and Emory University Medical Center, Atlanta, Georgia, USA
    Am J Respir Cell Mol Biol 40:601-9. 2009
    ..characterized by intermittent periods of hypoxemia, is an independent risk factor for the development of pulmonary hypertension. However, the exact mechanisms of this disorder remain to be defined...
  66. ncbi Prevalence and correlates of septal delayed contrast enhancement in patients with pulmonary hypertension
    Javier Sanz
    The Zena and Michael A Wiener Cardiovascular Institute and Marie Josee and Henry R Kravis Center for Cardiovascular Health, Mount Sinai School of Medicine, New York, New York, USA
    Am J Cardiol 100:731-5. 2007
    ..in the ventricular septum at the level of the right ventricular insertion points in patients with pulmonary hypertension (PH). The aim of this study was to investigate the prevalence, extent, and correlates of this finding...
  67. ncbi Role of epidermal growth factor inhibition in experimental pulmonary hypertension
    Bhola Kumar Dahal
    Max Planck Institute for Heart and Lung Research, Parkstrasse 1, 61231 Bad Nauheim, Germany
    Am J Respir Crit Care Med 181:158-67. 2010
    ..Epidermal growth factor (EGF) and its receptors play a role in cell proliferation and survival and are implicated in the pathobiology of pulmonary arterial hypertension (PAH)...
  68. ncbi Short-term oral administration of L-arginine improves hemodynamics and exercise capacity in patients with precapillary pulmonary hypertension
    N Nagaya
    Department of Internal Medicine and Department of Pharmacy, National Cardiovascular Center, and Osaka Seamen's Insurance Hospital, Osaka, Japan
    Am J Respir Crit Care Med 163:887-91. 2001
    ..of L-arginine, the precursor of nitric oxide (NO), on hemodynamics and exercise capacity in patients with pulmonary hypertension. Acute hemodynamic responses to oral L-arginine (0...
  69. ncbi Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension
    Derliz Mereles
    Department of Cardiology and Pneumology, University of Heidelberg, Heidelberg, Germany
    Circulation 114:1482-9. 2006
    b>Pulmonary hypertension (PH) is associated with restricted physical capacity, limited quality of life, and a poor prognosis because of right heart failure...
  70. ncbi High levels of nitric oxide in individuals with pulmonary hypertension receiving epoprostenol therapy
    M Ozkan
    Departments of Pulmonary and Critical Care Medicine, Lerner Research Institute, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA
    Lung 179:233-43. 2001
    Lack of vasodilator substances, such as nitric oxide (NO), has been implicated in the development of pulmonary hypertension, but the pathogenesis of the disease remains speculative...
  71. ncbi Dichloroacetate, a metabolic modulator, prevents and reverses chronic hypoxic pulmonary hypertension in rats: role of increased expression and activity of voltage-gated potassium channels
    Evangelos D Michelakis
    Department of Medicine, University of Alberta, Edmonton, Canada
    Circulation 105:244-50. 2002
    Chronic hypoxic pulmonary hypertension (CH-PHT) is associated with suppressed expression and function of voltage-gated K(+) channels (Kv) in pulmonary artery (PA) smooth muscle cells (SMCs) and a shift in cellular redox balance toward a ..
  72. ncbi Serotonin increases susceptibility to pulmonary hypertension in BMPR2-deficient mice
    Lu Long
    Department of Medicine, University of Cambridge School of Clinical Medicine, Addenbrooke s and Papworth Hospitals, Cambridge, United Kingdom
    Circ Res 98:818-27. 2006
    ..These findings provide the first evidence for an interaction between BMPR-II-mediated signaling and the serotonin pathway, perturbation of which may be critical to the pathogenesis of PAH...
  73. ncbi Pulmonary arterial hypertension
    Vallerie V McLaughlin
    University of Michigan Health System, Ann Arbor, MI 48109 0273, USA
    Circulation 114:1417-31. 2006
  74. ncbi Mitochondrial metabolism, redox signaling, and fusion: a mitochondria-ROS-HIF-1alpha-Kv1.5 O2-sensing pathway at the intersection of pulmonary hypertension and cancer
    Stephen L Archer
    University of Chicago, Section of Cardiology, IL 60637, USA
    Am J Physiol Heart Circ Physiol 294:H570-8. 2008
    ..Mitochondrial abnormalities that disturb the ROS-HIF-1alpha-Kv1.5 O(2)-sensing pathway contribute to the pathogenesis of PAH and cancer and constitute promising therapeutic targets...
  75. ncbi Primary pulmonary hypertension
    James R Runo
    Division of Allergy, Pulmonary and Critical Care Medicine, Vanderbilt University Medical Center, T 1217 Medical Center North, Nashville, TN 37232 2650, USA
    Lancet 361:1533-44. 2003
    Primary pulmonary hypertension (PPH) is a rare disorder characterised by raised pulmonary-artery pressure in the absence of secondary causes...
  76. ncbi Pulmonary vascular remodeling: a target for therapeutic intervention in pulmonary hypertension
    T K Jeffery
    Department of PhysiologyPharmacology, The University of Queensland, St Lucia, Brisbane, Queensland, 4072, Australia
    Pharmacol Ther 92:1-20. 2001
    Pulmonary vascular remodelling is an important pathological feature of pulmonary hypertension, leading to increased pulmonary vascular resistance and reduced compliance...
  77. ncbi A noninvasive assessment of pulmonary perfusion abnormality in patients with primary pulmonary hypertension
    H Ting
    Division of Respiratory and Critical Care Physiology and Medicine, Department of Medicine, Harbor-UCLA Medical Center, Torrance, CA, USA
    Chest 119:824-32. 2001
    ..ratio of minute ventilation [VE] to carbon dioxide output [VCO(2)]) is increased in patients with primary pulmonary hypertension (PPH) consequent to an increase in physiologic dead space and alveolar ventilation...
  78. pmc Hemolysis in sickle cell mice causes pulmonary hypertension due to global impairment in nitric oxide bioavailability
    Lewis L Hsu
    Marian Anderson Sickle Cell Center at St Christopher s Hospital for Children, Drexel University College of Medicine, Philadelphia, PA 19134, USA
    Blood 109:3088-98. 2007
    b>Pulmonary hypertension is a highly prevalent complication of sickle cell disease and is a strong risk factor for early mortality. However, the pathophysiologic mechanisms leading to pulmonary vasculopathy remain unclear...
  79. ncbi Cellular and molecular mechanisms of pulmonary vascular remodeling: role in the development of pulmonary hypertension
    Mehran Mandegar
    Department of Medicine, School of Medicine, University of California, San Diego, La Jolla 92093, USA
    Microvasc Res 68:75-103. 2004
    ....
  80. pmc Evidence for angiotensin-converting enzyme 2 as a therapeutic target for the prevention of pulmonary hypertension
    Anderson J Ferreira
    Department of Physiology and Functional Genomics, College of Medicine, Gainesville, FL 32610, USA
    Am J Respir Crit Care Med 179:1048-54. 2009
    ..and vasodilator mechanisms involving the pulmonary circulation leading to the development of pulmonary hypertension (PH)...
  81. pmc Vasoactive intestinal peptide as a new drug for treatment of primary pulmonary hypertension
    Ventzislav Petkov
    Department of Internal Medicine, University of Vienna, Medical School, Vienna, Austria
    J Clin Invest 111:1339-46. 2003
    Primary pulmonary hypertension is a fatal disease causing progressive right heart failure within 3 years after diagnosis...
  82. ncbi Pathologic assessment of vasculopathies in pulmonary hypertension
    Giuseppe G Pietra
    Department of Pathology, University of Pennsylvania, School of Medicine, Philadelphia, Pennsylvania, USA
    J Am Coll Cardiol 43:25S-32S. 2004
    Pulmonary arterial hypertension (PAH) includes various forms of pulmonary hypertension of different etiology but similar clinical presentation and functional derangement...
  83. ncbi Anxiety and depression in patients with pulmonary hypertension
    Bernd Löwe
    Department of General Internal and Psychosomatic Medicine, University of Heidelberg, Medical Center, Im Neuenheimer Feld 410, D 69120 Heidelberg, Germany
    Psychosom Med 66:831-6. 2004
    ..the prevalence and actual treatment of anxiety, depression, and other mental disorders in patients with pulmonary hypertension (PH)...
  84. pmc Pulmonary hypertension in heart failure with preserved ejection fraction: a community-based study
    Carolyn S P Lam
    Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA
    J Am Coll Cardiol 53:1119-26. 2009
    This study sought to define the prevalence, severity, and significance of pulmonary hypertension (PH) in heart failure with preserved ejection fraction (HFpEF) in the general community.
  85. ncbi Altered bone morphogenetic protein and transforming growth factor-beta signaling in rat models of pulmonary hypertension: potential for activin receptor-like kinase-5 inhibition in prevention and progression of disease
    Lu Long
    Division of Respiratory Medicine, Department of Medicine, Box 157, Addenbrooke s Hospital, Hills Rd, Cambridge CB2 2QQ, United Kingdom
    Circulation 119:566-76. 2009
    ..It remains unclear whether alterations in these pathways contribute to other forms of pulmonary hypertension and to what extent these changes can be exploited for therapeutic intervention.
  86. ncbi Dichloroacetate prevents and reverses pulmonary hypertension by inducing pulmonary artery smooth muscle cell apoptosis
    M Sean McMurtry
    Department of Medicine and Pediatrics, University of Alberta, Edmonton, Canada
    Circ Res 95:830-40. 2004
    ..5 in the media. We identify mitochondria-dependent apoptosis as a potential target for therapy and DCA as an effective and selective treatment for PAH...
  87. ncbi Altered growth responses of pulmonary artery smooth muscle cells from patients with primary pulmonary hypertension to transforming growth factor-beta(1) and bone morphogenetic proteins
    N W Morrell
    Department of Medicine, University of Cambridge, Addenbrooke s and Papworth Hospitals, Cambridge, United Kingdom
    Circulation 104:790-5. 2001
    ..growth factor-beta (TGF-beta) superfamily, underlie many cases of familial and sporadic primary pulmonary hypertension (PPH)...
  88. pmc VEGF ameliorates pulmonary hypertension through inhibition of endothelial apoptosis in experimental lung fibrosis in rats
    Laszlo Farkas
    Department of Medicine, McMaster University, Firestone Institute for Respiratory Health and St Joseph s Healthcare, Hamilton, Ontario, Canada
    J Clin Invest 119:1298-311. 2009
    Idiopathic pulmonary fibrosis (IPF) can lead to the development of secondary pulmonary hypertension (PH) and ultimately death. Despite this known association, the precise mechanism of disease remains unknown...
  89. ncbi Evaluation of pulmonary artery stiffness in pulmonary hypertension with cardiac magnetic resonance
    Javier Sanz
    Clinical Cardiac MR CT Program, Zena and Michael A Wiener Cardiovascular Institute and Marie Josee and Henry R Kravis Center for Cardiovascular Health, Mount Sinai School of Medicine, New York, New York 10029, USA
    JACC Cardiovasc Imaging 2:286-95. 2009
    This study sought to evaluate indexes of pulmonary artery (PA) stiffness in patients with pulmonary hypertension (PH) using same-day cardiac magnetic resonance (CMR) and right heart catheterization (RHC).
  90. pmc Expression profiling of laser-microdissected intrapulmonary arteries in hypoxia-induced pulmonary hypertension
    Grazyna Kwapiszewska
    Department of Pathology, Justus Liebig University Giessen, Germany
    Respir Res 6:109. 2005
    Chronic hypoxia influences gene expression in the lung resulting in pulmonary hypertension and vascular remodelling...
  91. ncbi Idiopathic pulmonary fibrosis and pulmonary hypertension: connecting the dots
    Steven D Nathan
    Medical Director, Advanced Lung Disease and Transplant Program, Inova Heart and Vascular Institute, Inova Fairfax Hospital, Falls Church, VA 22042, USA
    Am J Respir Crit Care Med 175:875-80. 2007
    Idiopathic pulmonary fibrosis (IPF) has a poor prognosis and a course that is unpredictable. Pulmonary hypertension may complicate the course of IPF and potentially impact prognosis...
  92. ncbi Contrast enhanced-cardiovascular magnetic resonance imaging in patients with pulmonary hypertension
    Kevin G Blyth
    Scottish Pulmonary Vascular Unit, Level 8, Western Infirmary, Glasgow G11 6NT, UK
    Eur Heart J 26:1993-9. 2005
    To determine the presence and extent of delayed contrast enhancement (DCE) in patients with pulmonary hypertension (PHT) using contrast enhanced-cardiovascular magnetic resonance imaging (ce-CMR).
  93. ncbi Pulmonary hypertension in COPD: epidemiology, significance, and management: pulmonary vascular disease: the global perspective
    Omar A Minai
    Department of Pulmonary and Critical Care Medicine, Cleveland Clinic, Cleveland, OH 44195, USA
    Chest 137:39S-51S. 2010
    b>Pulmonary hypertension (PH) associated with parenchymal lung diseases is one of the most common forms of PH...
  94. ncbi Long-term outcome in pulmonary arterial hypertension patients treated with subcutaneous treprostinil
    R J Barst
    Columbia University College of Physicians and Surgeons, 3959 Broadway, BHN 2 255, New York, NY 10032, USA
    Eur Respir J 28:1195-203. 2006
    ..In contrast, predicted survival was 69-38% over 1-4 yrs. The safety profile for long-term subcutaneous treprostinil was consistent with previous short-term trials with no unexpected adverse events...
  95. ncbi Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome
    V Cottin
    Hospices Civils de Lyon, Hopital Louis Pradel, Service de pneumologie Centre de référence des maladies pulmonaires rares, Universite de Lyon, Université Lyon I, UCBL INRA ENVL EPHE, UMR754, IFR128, Lyon, France
    Eur Respir J 35:105-11. 2010
    This study aims to describe the haemodynamic and survival characteristics of patients with pulmonary hypertension in the recently individualised syndrome of combined pulmonary fibrosis and emphysema...
  96. doi Emerging concepts and translational priorities in pulmonary arterial hypertension
    Evangelos D Michelakis
    Department of Medicine, Cardiology Division, Pulmonary Hypertension Program, University of Alberta, Edmonton, Alberta, Canada
    Circulation 118:1486-95. 2008
  97. ncbi Bosentan therapy for pulmonary arterial hypertension
    Lewis J Rubin
    Division of Pulmonary and Critical Care Medicine, University of California at San Diego, La Jolla 92037 1330, USA
    N Engl J Med 346:896-903. 2002
    ..The present trial investigated the effect of bosentan on exercise capacity in a larger number of patients and compared two doses...
  98. pmc Persistent eNOS activation secondary to caveolin-1 deficiency induces pulmonary hypertension in mice and humans through PKG nitration
    You Yang Zhao
    Department of Pharmacology and 2Center for Lung and Vascular Biology, University of Illinois College of Medicine, Chicago, Illinois, USA
    J Clin Invest 119:2009-18. 2009
    b>Pulmonary hypertension (PH) is an unremitting disease defined by a progressive increase in pulmonary vascular resistance leading to right-sided heart failure...
  99. ncbi Perspectives on endothelial-to-mesenchymal transition: potential contribution to vascular remodeling in chronic pulmonary hypertension
    Enrique Arciniegas
    Laboratorio de Microscopía Electrónica, Servicio Autónomo Instituto de Biomedicina, Facultad de Medicina, Universidad Central de Venezuela, Caracas, Venezuela
    Am J Physiol Lung Cell Mol Physiol 293:L1-8. 2007
    All forms of pulmonary hypertension are characterized by structural changes in pulmonary arteries. Increased numbers of cells expressing alpha-smooth muscle (alpha-SM) actin is a nearly universal finding in the remodeled artery...
  100. ncbi Sildenafil citrate therapy for pulmonary arterial hypertension
    Nazzareno Galie
    Institute of Cardiology, University of Bologna, Bologna, Italy
    N Engl J Med 353:2148-57. 2005
    ....
  101. pmc Early right ventricular systolic dysfunction in patients with systemic sclerosis without pulmonary hypertension: a Doppler Tissue and Speckle Tracking echocardiography study
    Sebastian Schattke
    Universitatsmedizin Berlin, Medical Clinic for Cardiology and Angiology, Charite Campus Mitte, Chariteplatz 1, 10117 Berlin, Germany
    Cardiovasc Ultrasound 8:3. 2010
    ..The aim of this study was to evaluate IVA as an early parameter for the detection of right ventricular (RV) systolic dysfunction in patients with systemic sclerosis (SSc) without pulmonary hypertension.

Research Grants72

  1. Prevention of Complications in Thalassemia
    Elliott P Vichinsky; Fiscal Year: 2011
    ..goals include expanded screening for cardiac iron deposition using newly available MRI technology and pulmonary hypertension. Indications of research hypotheses and research questions to be addressed and answered throughout the ..
  2. Iron-mediated vascular disease in sickle cell disease.
    John C Wood; Fiscal Year: 2010
    ..SCD patients suffer from chronic, progressive vasculopathy leading to pulmonary hypertension, renal failure and stroke...
  3. Right ventricular contractile dysfunction after pressure overload
    CLIFFORD RUSSELL GREYSON; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Right ventricular (RV) contractile failure from acute pulmonary hypertension is an important cause of morbidity and mortality in conditions such as massive pulmonary embolism, hypoxic pulmonary ..
  4. Regulation of mural cells during pulmonary capillary formation
    Stephen E McGowan; Fiscal Year: 2013
    ..microvasculature is also an important therapeutic goal for interstitial fibrosis and other causes of pulmonary hypertension. A critical step towards achieving this goal is to identify factors which regulate capillary expansion and ..
  5. DNA methylation of extracellular superoxide dismutase in pulmonary hypertension
    EVA NOZIK GRAYCK; Fiscal Year: 2012
    ..in transgenic mice or through adenoviral gene delivery attenuates pulmonary vascular remodeling and pulmonary hypertension. The regulation of EC-SOD in IPAH has not been investigated...
  6. REGULATION OF PULMONARY CIRCULATION IN FETUS AND NEWBORN
    Girija G Konduri; Fiscal Year: 2013
    Project Summary Persistent pulmonary hypertension of the newborn (PPHN) is a condition that results from failure of pulmonary vasodilation to occur at birth...
  7. Mechanisms and Consequences of Reduced PPAR gamma in Pulmonary Hypertension
    C Michael Hart; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Pulmonary hypertension (PH) is a devastating cardiopulmonary disorder with significant morbidity and mortality that frequently complicates disorders that affect the veteran patient population...
  8. Platelet TSP1 Mediates Vascular Disease and pulmonary hypertension in Sickle Cell
    Enrico M Novelli; Fiscal Year: 2013
    ..These processes lead to platelet and hemostatic activation, pulmonary hypertension and vascular disease...
  9. Effectiveness and Safety of Antithrombotic Strategies after Joint Replacement
    Elaine Hylek; Fiscal Year: 2013
    ..The long-term sequelae of VTE include post thrombotic syndrome and pulmonary hypertension which are each associated with low health-related quality of life (HRQOL), reduced survival, and high ..
  10. Antenatal Betamethasone in Late Preterm Gestation Lambs
    SATYANARAYANA LAKSHMINRUSIMHA; Fiscal Year: 2010
    ..Respiratory morbidity from disorders of transition including pulmonary hypertension (impaired pulmonary vasodilation), transient tachypnea of newborn (inadequate lung liquid clearance) and ..
  11. BMP signaling in vertebrate development
    Ken W Y Cho; Fiscal Year: 2013
    ..g., juvenile polyposis syndrome, primary pulmonary hypertension, Cowden's syndrome) and developmental abnormalities...
  12. Tadalafil for Pulmonary Hypertension Associated with Chronic Lung Disease
    SHARON IRENE SMITH ROUNDS; Fiscal Year: 2013
    ..with tadalafil at 40 mg daily over 12 months on exercise capacity in patients with at least moderate pulmonary hypertension (PH) (mean pulmonary artery pressure [mPAP] >30 mm Hg, pulmonary vascular resistance [PVR]>3...
  13. Fate of Lung Stem Cells During Pulmonary Disease
    Susan M Majka; Fiscal Year: 2012
    ..PUBLIC HEALTH RELEVANCE: Pulmonary hypertension, chronic obstructive pulmonary disease, interstitial pulmonary fibrosis and other adult lung conditions ..
  14. The unique role of B-Raf in murine cardiac development and postnatal function
    Natasha N Chattergoon; Fiscal Year: 2013
    ..I studied vascular smooth muscle growth in the context of primary pulmonary hypertension for my doctoral dissertation...
  15. Nitric Oxide in Pulmonary Hypertension
    Serpil C Erzurum; Fiscal Year: 2013
    ..Overall our goals are to define the pathophysiology of the abnormal vascular growth in PAH, and in so doing, apply the knowledge to improve the care of patients. ..
  16. PlGF-HIF1a-miRNA Axis in Sickle Pulmonary Hypertension
    Punam Malik; Fiscal Year: 2013
    Project Summary Pulmonary hypertension (PHT) occurs in ~30% of patients with sickle cell anemia (SCA) and results in ~50% mortality within 2 years of diagnosis...
  17. Zipcode based nano-imaging of hypertensive pulmonary arteries
    Rubin M Tuder; Fiscal Year: 2010
    ..Severe pulmonary hypertension (SPH) is an irreversible, malignant elevation of pulmonary artery pressures, often associated with high ..
  18. Neprilysin and Pulmonary Vascular Remodeling: Cellular and Molecular Mechanisms
    EDWARD CHARLES DEMPSEY; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): PROJECT ABSTRACT Chronic hypoxic pulmonary hypertension (PHTN) is a major clinical problem, in the general and Veteran populations, and complicates most lung and heart disorders, including chronic ..
  19. TRANSFORMING GROWTH FACTOR - BETA MEDIATES EFFECTS OF HYPOXIA IN NEWBORN LUNG
    Namasivayam Ambalavanan; Fiscal Year: 2012
    ..PAR contributes to persistent pulmonary hypertension of the newborn (PPHN), and both IAD and PAR are seen in BPD...
  20. Genetic Basis for Impaired Angiogenic Signaling in BPD
    STEVEN HERBERT ABMAN; Fiscal Year: 2012
    ..decreased NO bioavailability due to high oxidant stress increase susceptibility of the developing lung for pulmonary hypertension, impaired angiogenesis and reduced alveolarization...
  21. Antenatal betamethasone for persistent pulmonary hypertension of newborn
    Girija G Konduri; Fiscal Year: 2010
    DESCRIPTION (provided by applicant): Persistent pulmonary hypertension of the newborn (PPHN) occurs when the pulmonary vascular resistance fails to decrease at birth...
  22. Role of VEGF in Perinatal Hypertension
    STEVEN HERBERT ABMAN; Fiscal Year: 2013
    ..recurrent respiratory exacerbations, impaired lung function into adulthood, exercise intolerance, and pulmonary hypertension in survivors...
  23. Transplantation tolerance and immune function following mTOR inhibition.
    Jonathan D Powell; Fiscal Year: 2013
    ..in stable mixed chimerism that corrects their hematological phenotype of sickle cell anemia and reverses pulmonary hypertension. Based on this success, a second clinical trial was developed to expand the potential donor pool to ..
  24. Translational Approaches to Reducing Health Disparities in Lung Disease
    Joe G N Garcia; Fiscal Year: 2012
    ..The proposed conference will focus on five major thematic areas: 1. Pulmonary Hypertension (including PH associated with sickle cell disease);2. Airway Diseases (Asthma and COPD);3...
  25. HIV and CD16+ monocytes as cellular reservoir &HIV/pulmonary hypertension
    Sharilyn Almodovar; Fiscal Year: 2013
    ..HIV+ patients are more frequently diagnosed with HIV-related pulmonary hypertension (HRPAH) compared to the general population, regardless of suppressive HAART...
  26. Statins for Pulmonary and Cardiac Complications of Chronic HIV (The SPARC Trial)
    Stephen R Wisniewski; Fiscal Year: 2013
    ..HIV-uninfected population, COPD frequently co-exists with cardiac disease including atherosclerosis and pulmonary hypertension. Our work has demonstrated that a syndrome of "cardiopulmonary dysfunction" exists even in non-smoking or ..
  27. Role of Microbial Translocation in Cardiopulmonary Disease in Children with HIV
    Alison Morris; Fiscal Year: 2013
    ..We find that MT is correlated with pulmonary hypertension on echocardiography, increased airway obstruction, impairment of diffusing capacity for carbon monoxide, ..
  28. NFATc3 in chronic hypoxic pulmonary hypertension
    Laura V Gonzalez Bosc; Fiscal Year: 2012
    ..A better understanding of this mechanisms in PAH will lead to the development of novel therapeutic approaches to prevent and cure this debilitating disease. ..
  29. Renin-angiotensin and fibrinolysis interaction in humans
    NANCY JOAN BROWN; Fiscal Year: 2013
    ..PDE5 inhibitors have been approved for the long-term treatment of pulmonary hypertension, improve many biomarkers of endothelial function and appear to be safe in patients at risk for ..
  30. Bifunctional Redox Agent for the Treatment of PPHN
    GARRY JOHN SOUTHAN; Fiscal Year: 2011
    DESCRIPTION (provided by applicant): Persistent pulmonary hypertension of the newborn (PPHN) is a life-threatening neonatal condition wherein an elevation in pulmonary vascular resistance after birth diverts pulmonary blood flow via a ..
  31. (INTRODUCTION) MECHANISMS OF ACCLIMATIZATION: FETUS AND ADULT
    Lawrence D Longo; Fiscal Year: 2013
    ..For newborn they relate to altered cerebrovascular blood flow with intracerebral hemorrhage and pulmonary hypertension. 2) The studies also will contribute to understanding mechanisms of maternal cardiovascular disorders and ..
  32. Cardioprotective Signaling following Phosphodiesterase-5 Inhibition
    Rakesh C Kukreja; Fiscal Year: 2012
    ..indications in addition to their current clinical use for treatment of erectile dysfunction and pulmonary hypertension. LAY NARRATIVE Acute myocardial infarction (AMI) continues to be a major cause of morbidity and mortality ..
  33. Toll-like receptor signaling in pathogenesis of pulmonary hypertension
    Yabing Chen; Fiscal Year: 2013
    ....
  34. Purinergic signaling in sleep apnea
    Elzbieta Kaczmarek; Fiscal Year: 2012
    ..sleepiness, OSA has been implicated in the pathogenesis of cardiovascular diseases, including systemic and pulmonary hypertension, coronary artery disease, congestive heart failure, stroke, cardiac arrhythmias, and sudden cardiac death...
  35. Prostacyclin Synthase and Receptors in Pulmonary Arterial Hypertension
    Mark W Geraci; Fiscal Year: 2011
    ..We will sequence the PGIS promoters from familial pulmonary hypertension, correlating specific haplotypes with disease on-set, severity, and morbidity...
  36. The role of the Wnt/planar cell polarity pathway in pulmonary angiogenesis
    Vinicio A de Jesus Perez; Fiscal Year: 2013
    ..vascular biology research began with my first clinical encounters with patients suffering from idiopathic pulmonary hypertension (IPAH)...
  37. Pulmonary Vasoreactivity Following Chronic Hypoxia
    Thomas C Resta; Fiscal Year: 2012
    Chronic hypoxia (CH) associated with obstructive lung diseases and sleep apnea leads to pulmonary hypertension. Elevated basal vascular smooth muscle (VSM) tone and enhanced vasoconstrictor reactivity play a prominent role in mediating ..
  38. Free Hemoglobin Potentiates Pulmonary Vascular Dysfunction in Acute Lung Injury
    JULIE ANNE BASTARACHE; Fiscal Year: 2013
    ..Hemoglobin-induced pulmonary vascular injury and pulmonary hypertension in ALI/ARDS may be driven by the potent pro-oxidant effects of hemoglobin...
  39. The Impact of Oxidative Stress on HIV-induced Lung Disease
    Jesse Roman; Fiscal Year: 2013
    ..with ART, however, pulmonary infections are less frequent and noninfectious lung disorders, such as COPD, pulmonary hypertension, lung cancer, and pulmonary fibrosis are emerging as important causes of illness...
  40. Genetic Analysis of Murine Chronic Hypoxia-Induced Pulmonary Hypertension
    William C Nichols; Fiscal Year: 2013
    ..There are approximately 100,000 new pulmonary hypertension (PH) patients annually in the U.S...
  41. Role of Bone Morphogenetic Protein 4 in Hypoxic Pulmomary Hypertension
    Jian Wang; Fiscal Year: 2013
    ..in vascular tone and pulmonary vascular remodeling play key roles in the pathogenesis of chronic hypoxic pulmonary hypertension (CHPH)...
  42. Heme Oxygenase Polymorphisms and Hepatopulmonary Syndrome
    KARI EVAN ROBERTS; Fiscal Year: 2012
    ..to the genetic epidemiology of known and cryptic polymorphisms in heme oxygenase, an enzyme implicated in the the pathogenesis of numerous disorders including pulmonary hypertension, asthma and ischemic heart disease.
  43. Pulmonary Vascular Changes in Early Chronic Obstructive Pulmonary Disease (COPD)
    R Graham Barr; Fiscal Year: 2011
    ..This study is designed to test a novel hypothesis of endothelial dysfunction and pulmonary hypertension early in the pathogenesis of COPD...
  44. Variations in estrogen exposures may modify pulmonary arterial hypertension
    ERIC DOUGLAS AUSTIN; Fiscal Year: 2013
    ..that benefits from my experienced mentor group, as well as the collaborative milieu of Vanderbilt and our Pulmonary Hypertension Research Group, to provide a comprehensive mentored educational and research experience. Primary Mentor Dr...
  45. 2013 Elastin, Elastic Fibers &Microfibrils Gordon Research Conference &Gordon R
    Zsolt Urban; Fiscal Year: 2013
    ..atherosclerosis, aneurysms, chronic obstructive pulmonary disease, vascular remodeling in systemic and pulmonary hypertension, and skin aging mechanisms...
  46. Protein biomarkers of pulmonary hypertension in sickle cell disease
    Mark E McComb; Fiscal Year: 2013
    ..An elevated tricuspid regurgitant jet velocity (TRV), suggestive of pulmonary hypertension (PH) occurs in up to 1/3 of adults with sickle cell anemia (HbSS) and carries with it a 6-10-fold ..
  47. PITCH HF (CCC) Lead Application
    Marc J Semigran; Fiscal Year: 2013
    ..in heart failure (HF) patients with left ventricular (LV) systolic dysfunction (LVSD) and secondary pulmonary hypertension (PH)...
  48. Redox Regulation of Vascular cGMP Signaling in Neonatal Lungs
    Kathryn N Farrow; Fiscal Year: 2013
    ..Recently, pulmonary hypertension (PH) and right-sided heart failure have been recognized as complications in infants with moderate or ..
  49. Rho-kinase signaling in lung development and persistent pulmonary hypertension
    Jason Gien; Fiscal Year: 2013
    ..From early in my career I have been interested in persistent pulmonary hypertension of the newborn (PPHN) and mechanisms responsible for decreased responsiveness of some newborns with severe ..
  50. Regulation of Rho Signaling by S-Nitrosothiols
    A Richard Whorton; Fiscal Year: 2012
    ..whose over-activity is commonly associated with cardiovascular disease and contributes to systemic and pulmonary hypertension, vascular inflammation, and atherosclerosis...
  51. Regulation and function of cGMP dependent protein kinase in cardiac hypertrophy
    Eiki Takimoto; Fiscal Year: 2013
    ..PDE5A inhibitors are currently widely used to treat erectile dysfunction and pulmonary hypertension. Reports from us and others supporting cardiac benefits from cGMP enhancement by PDE5A inhibitors have ..
  52. Intrauterine chronic hypoxia and ryanodine receptors in fetal pulmonary arteries
    Sean M Wilson; Fiscal Year: 2013
    ..living, placental insufficiency, and smoking puts infants of certain populations at risk o developing pulmonary hypertension, high altitude pulmonary edema, and increases the risk for development of idiopathic pulmonary ..
  53. Impact of Insufficient Vascular EC-SOD in Pulmonary Hypertension
    EVA NOZIK GRAYCK; Fiscal Year: 2013
    ..and reactive oxygen species signaling are now recognized to have a critical role in the pathogenesis of pulmonary hypertension (PH). The sole extracellular enzymatic defense against superoxide (O2...
  54. Calpain Activates Intracellular TGF-beta1 in Pulmonary Hypertension
    Yunchao Su; Fiscal Year: 2013
    ..the first time that global knockout of calpain prevents pulmonary vascular remodeling in hypoxia-induced pulmonary hypertension and that the calpain inhibitor MDL28170 prevents the progression of established pulmonary hypertension ..
  55. Investigation into Cold-induced Pulmonary Vascular Inflammation and Dysfunction
    Zhongjie Sun; Fiscal Year: 2013
    ..pulmonary arterial hypertension and right ventricular (RV) hypertrophy in rats, namely cold-induced pulmonary hypertension (CIPH)...
  56. Identification of Novel Genes for Congenital Diaphragmatic Hernia
    Wendy K Chung; Fiscal Year: 2012
    ..of genetic analyses and survival to discharge and 2 years of age, associated birth defects, severity of pulmonary hypertension at 1 and 3 months, weight and height at 2 years, and Bayley Scales of Infant Development-II and Vineland ..
  57. PITCH HF (DCC)
    Flora Sandra Siami; Fiscal Year: 2013
    ..in heart failure (HF) patients with left ventricular (LV) systolic dysfunction (LVSD) and secondary pulmonary hypertension (PH)...
  58. PPAR gamma and Nox4 in pulmonary hypertension
    Roy L Sutliff; Fiscal Year: 2013
    Despite existing therapies, pulmonary hypertension (PH) causes significant morbidity and mortality. This proposal focuses on peroxisome proliferator-activated receptor gamma (PPARg) as a new target in PH therapy...
  59. Pathogenetics of the FOX transcription factor gene cluster on 16q24.1
    Pawel Stankiewicz; Fiscal Year: 2013
    ..Infants affected with ACD/MPV develop severe respiratory distress with pulmonary hypertension within the first few days of life and despite intensive care they usually die soon thereafter...
  60. Properties of Clca Channels in Vascular Myocytes
    Normand Leblanc; Fiscal Year: 2009
    ..The etiology of pulmonary hypertension (PH) in humans is still poorly understood although it is becoming increasingly clear that defective ionic ..
  61. Ameliorating Sickle Nephropathy and Pulmonary Hypertension
    Punam Malik; Fiscal Year: 2013
    ..SN and animal data that supports an interventional trial with losartan to ameliorate UCD, albuminuria and pulmonary hypertension in sickle cell disease...
  62. Building Core Programs in Cardiovascular and Metabolic Disease
    Paul G McGuire; Fiscal Year: 2010
    ..Vascular Biology Research and Training Core, which has a thematic focus on the mechanisms of systemic and pulmonary hypertension, and metabolic disease-related vascular pathology...
  63. PO2 MODULATION OF NO AND ET1 IN PULMONARY HYPERTENSION
    Karen Fagan; Fiscal Year: 2002
    b>Pulmonary hypertension (PHT), both primary and secondary, leads to significant morbidity and mortality with few acceptable therapeutic options...
  64. POLYAMINES AND MONOCROTALINE PNEUMOTOXICITY (AHR-F1)
    JACK OLSON; Fiscal Year: 1999
    ..MCT) pneumotoxicity is associated with a spectrum of pulmonary vascular lesions reminiscent of idiopathic pulmonary hypertension in humans...
  65. Genetic Risk Factors for Portopulmonary Hypertension
    Steven Kawut; Fiscal Year: 2005
    Patients with portal hypertension are at increased risk of pulmonary hypertension (termed portopulmonary hypertension). Patients with portopulmonary hypertension suffer from increased morbidity and mortality...
  66. NO/CGMP RELAXATION IN PULMONARY HYPERTENSION
    Brian Fouty; Fiscal Year: 2000
    DESCRIPTION (Adapted from the applicant's abstract) Pulmonary hypertension (PHT) represents an important clinical problem in the United States...
  67. Endothelial Dysfunction and the RV in the Multi-Ethnic Study of Atherosclerosis
    Steven M Kawut; Fiscal Year: 2010
    b>Pulmonary hypertension is an elevation in pulmonary artery pressure which may result from a variety of heart and lung diseases...
  68. PULMONARY HYPERTENSION--MECHANISMS AND FAMILY REGISTRY
    James Loyd; Fiscal Year: 2003
    Primary pulmonary hypertension (PPH) is a serious disease of unknown cause in which small arteries in the lungs become obstructed. Mean survival is less than 3 years, and women develop PPH twice as commonly as men...
  69. VASODILATOR FOR NEONATAL PULMONARY HYPERTENSION
    Garry Southan; Fiscal Year: 1999
    b>Pulmonary hypertension is a major cause of morbidity and mortality in neonates in the setting of congenital diaphragmatic hernia, meconium aspiration, respiratory distress syndrome of prematurity, and Group B streptococcal pneumonia...
  70. Characterization and Development of a New Animal Model of Pulmonary Hypertension
    Sylvia McCune; Fiscal Year: 2007
    unreadable] DESCRIPTION (provided by applicant): Currently, a major obstacle facing basic pulmonary hypertension (PH) and right ventricular (RV) dysfunction/failure research is the lack of good, truly representative animal models for ..
  71. Regulation of Kv Channels by Anorexigens
    Bruce Pitt; Fiscal Year: 2007
    ..Kv) channels in pulmonary arterial smooth muscle cells (PASMCs) have been implicated in the initiation of pulmonary hypertension: inhibition of these channels results in membrane depolarization and an increase in intracellular Ca2+ ..
  72. CLONING OF FAMILIAL PRIMARY PULMONARY HYPERTENSION GENE
    William Nichols; Fiscal Year: 2006
    Primary pulmonary hypertension (PPH) is characterized clinically by elevated pulmonary artery pressures in the absence of a secondary cause...