Robyn J Barst

Summary

Affiliation: Columbia University
Country: USA

Publications

  1. ncbi request reprint 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
  2. pmc Fatal pulmonary arterial hypertension associated with phenylpropanolamine exposure
    R J Barst
    Columbia University College of Physicians and Surgeons, New York, New York, USA
    Heart 90:e42. 2004
  3. ncbi request reprint Sitaxsentan therapy for pulmonary arterial hypertension
    Robyn J Barst
    Columbia University College of Physicians and Surgeons, 3959 Broadway, BHN 2 255, New York, NY 10032 1551, USA
    Am J Respir Crit Care Med 169:441-7. 2004
  4. ncbi request reprint Beraprost therapy for pulmonary arterial hypertension
    Robyn J Barst
    Columbia University College of Physicians and Surgeons, New York, New York 10032, USA
    J Am Coll Cardiol 41:2119-25. 2003
  5. pmc A review of pulmonary arterial hypertension: role of ambrisentan
    Robyn J Barst
    New York Presbyterian Pulmonary Hypertension Center, Columbia University College of Physicians and Surgeons, 3959 Broadway, BHN 2 255, NewYork, NY 10032, USA
    Vasc Health Risk Manag 3:11-22. 2007
  6. ncbi request reprint Sitaxsentan: a selective endothelin-A receptor antagonist, for the treatment of pulmonary arterial hypertension
    Robyn J Barst
    Columbia University College of Physicians and Surgeons, 3959 Broadway, BHN 2 255, New York, NY 10032, USA
    Expert Opin Pharmacother 8:95-109. 2007
  7. ncbi request reprint 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
  8. ncbi request reprint Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan
    Robyn J Barst
    Department of Pediatrics, Columbia University Medical Center, New York, New York, USA
    J Am Coll Cardiol 47:2049-56. 2006
  9. pmc PDGF signaling in pulmonary arterial hypertension
    Robyn J Barst
    New York Presbyterian Pulmonary Hypertension Center, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA
    J Clin Invest 115:2691-4. 2005
  10. doi request reprint Effectiveness of transition from intravenous epoprostenol to oral/inhaled targeted pulmonary arterial hypertension therapy in pediatric idiopathic and familial pulmonary arterial hypertension
    Laura Melnick
    Division of Pediatric Cardiology, Columbia University College of Physicians and Surgeons, New York, New York, USA
    Am J Cardiol 105:1485-9. 2010

Detail Information

Publications80

  1. ncbi request reprint 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...
  2. pmc Fatal pulmonary arterial hypertension associated with phenylpropanolamine exposure
    R J Barst
    Columbia University College of Physicians and Surgeons, New York, New York, USA
    Heart 90:e42. 2004
    ....
  3. ncbi request reprint Sitaxsentan therapy for pulmonary arterial hypertension
    Robyn J Barst
    Columbia University College of Physicians and Surgeons, 3959 Broadway, BHN 2 255, New York, NY 10032 1551, USA
    Am J Respir Crit Care Med 169:441-7. 2004
    ..02 for each parameter at both doses). The incidence of elevated aminotransferase values (> three times normal) was 3% for the placebo group, 0% for the 100-mg group, and 10% for the 300-mg group...
  4. ncbi request reprint Beraprost therapy for pulmonary arterial hypertension
    Robyn J Barst
    Columbia University College of Physicians and Surgeons, New York, New York 10032, USA
    J Am Coll Cardiol 41:2119-25. 2003
    ..The purpose of this study was to assess the safety and efficacy of the oral prostacyclin analogue beraprost sodium during a 12-month double-blind, randomized, placebo-controlled trial in patients with pulmonary arterial hypertension (PAH)...
  5. pmc A review of pulmonary arterial hypertension: role of ambrisentan
    Robyn J Barst
    New York Presbyterian Pulmonary Hypertension Center, Columbia University College of Physicians and Surgeons, 3959 Broadway, BHN 2 255, NewYork, NY 10032, USA
    Vasc Health Risk Manag 3:11-22. 2007
    ..The demonstration of clinical efficacy, low incidence of acute hepatic toxicity, and low risk of drug-drug interactions support the role of ambrisentan for the treatment of PAH...
  6. ncbi request reprint Sitaxsentan: a selective endothelin-A receptor antagonist, for the treatment of pulmonary arterial hypertension
    Robyn J Barst
    Columbia University College of Physicians and Surgeons, 3959 Broadway, BHN 2 255, New York, NY 10032, USA
    Expert Opin Pharmacother 8:95-109. 2007
    ..The demonstration of clinical efficacy and low incidence of acute hepatotoxicity support the potential use of sitaxsentan for the treatment of PAH...
  7. ncbi request reprint 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
    ..Close follow-up at a center specializing in management of PH is recommended, with careful periodic reassessment and adjustment of therapy...
  8. ncbi request reprint Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan
    Robyn J Barst
    Department of Pediatrics, Columbia University Medical Center, New York, New York, USA
    J Am Coll Cardiol 47:2049-56. 2006
    ..We sought to determine the optimal dose of the selective endothelin A (ET(A)) receptor antagonist sitaxsentan for the treatment of pulmonary arterial hypertension (PAH); for observation only, an open-label (OL) bosentan arm was included...
  9. pmc PDGF signaling in pulmonary arterial hypertension
    Robyn J Barst
    New York Presbyterian Pulmonary Hypertension Center, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA
    J Clin Invest 115:2691-4. 2005
    ..These studies provide preclinical proof of concept for the clinical development of a PDGF inhibitor as a targeted therapy for PAH patients...
  10. doi request reprint Effectiveness of transition from intravenous epoprostenol to oral/inhaled targeted pulmonary arterial hypertension therapy in pediatric idiopathic and familial pulmonary arterial hypertension
    Laura Melnick
    Division of Pediatric Cardiology, Columbia University College of Physicians and Surgeons, New York, New York, USA
    Am J Cardiol 105:1485-9. 2010
    ....
  11. ncbi request reprint Effects of long-term bosentan in children with pulmonary arterial hypertension
    Erika Berman Rosenzweig
    Division of Pediatric Cardiology, New York Presbyterian Hospital, New York, New York 10032, USA
    J Am Coll Cardiol 46:697-704. 2005
    ..This study investigated the long-term outcome of children with pulmonary arterial hypertension (PAH) treated with bosentan therapy, with or without concomitant prostanoid therapy...
  12. ncbi request reprint Outcomes in children with idiopathic pulmonary arterial hypertension
    Delphine Yung
    Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, NY 10032, USA
    Circulation 110:660-5. 2004
    ..We sought to determine parameters associated with survival and treatment success...
  13. pmc Short- and long-term effects of inhaled iloprost therapy in children with pulmonary arterial hypertension
    D Dunbar Ivy
    The Pulmonary Hypertension Program and Pediatric Heart Lung Center, Department of Pediatrics, The University of Colorado School of Medicine and The Children s Hospital, Denver, Colorado 80045, USA
    J Am Coll Cardiol 51:161-9. 2008
    ..This study investigated the short- and long-term outcome of children with pulmonary arterial hypertension (PAH) treated with inhaled iloprost...
  14. pmc Exercise capacity and haemodynamics in patients with sickle cell disease with pulmonary hypertension treated with bosentan: results of the ASSET studies
    Robyn J Barst
    Department of Pediatric Cardiology, Columbia University, New York, NY, USA
    Br J Haematol 149:426-35. 2010
    ..Limited data in SCD-PH suggest that a low 6MWD predicts a low CO. Standard-dose bosentan appears to be well tolerated. Further investigation is warranted. Clinicaltrials.gov registration numbers NCT00310830, NCT00313196, NCT00360087...
  15. ncbi request reprint Idiopathic pulmonary arterial hypertension in children
    Erika Berman Rosenzweig
    Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA
    Curr Opin Pediatr 17:372-80. 2005
    ..A review of some of the latest medical advances will provide the reader with a better understanding of the most current treatment options for children with idiopathic pulmonary arterial hypertension...
  16. doi request reprint Clinical implications of determining BMPR2 mutation status in a large cohort of children and adults with pulmonary arterial hypertension
    Erika B Rosenzweig
    Department of Pediatric Cardiology, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA
    J Heart Lung Transplant 27:668-74. 2008
    ..We investigated the role of BMPR2 mutations on acute vasoreactivity and disease severity in IPAH/FPAH children and adults...
  17. doi request reprint Pulmonary arterial hypertension in children: a medical update
    Erika B Rosenzweig
    Department of Pediatrics, Columbia University College of Physician and Surgeons, New York, NY 10032, USA
    Indian J Pediatr 76:77-81. 2009
    ..A comprehensive review of the most recent literature over the past year on available and emerging novel therapies as well as an approach to target pediatric populations provides insights into the management of pediatric PAH patients...
  18. ncbi request reprint New predictors of outcome in idiopathic pulmonary arterial hypertension
    Steven M Kawut
    Department of Internal Medicine, Joseph L Mailman School of Public Health, Columbia University, 622 West 168th Street, New York, NY 10032, USA
    Am J Cardiol 95:199-203. 2005
    ..Race is identified as a new risk factor, which may be attributable to biologic or socioeconomic differences. Cardiac function and acute reactivity of the pulmonary vascular bed remain strong independent predictors of outcome...
  19. ncbi request reprint Clinical efficacy of sitaxsentan, an endothelin-A receptor antagonist, in patients with pulmonary arterial hypertension: open-label pilot study
    Robyn J Barst
    Department of Pediatrics, Columbia University, College of Physicians and Surgeons, New York, NY, USA
    Chest 121:1860-8. 2002
    ..To evaluate the safety and efficacy of sitaxsentan, an endothelin-A receptor antagonist, in a 12-week, open-label trial of patients with pulmonary arterial hypertension (PAH)...
  20. pmc Updated evidence-based treatment algorithm in pulmonary arterial hypertension
    Robyn J Barst
    Columbia University, New York, New York 10583, USA
    J Am Coll Cardiol 54:S78-84. 2009
    ..Atrial septostomy and lung transplantation are indicated for refractory patients or where medical treatment is unavailable...
  21. ncbi request reprint von Willebrand factor independently predicts long-term survival in patients with pulmonary arterial hypertension
    Steven M Kawut
    Department of Medicine, College of Physicians and Surgeons, Joseph L Mailma School of Public Health, Columbia University, New York, NY, USA
    Chest 128:2355-62. 2005
    ....
  22. ncbi request reprint Sitaxsentan: a novel endothelin-A receptor antagonist for pulmonary arterial hypertension
    Allison C Widlitz
    Pulmonary Hypertension Center, Columbia University College of Physicians and Surgeons, BHN 2 255, New York, NY 10032, USA
    Expert Rev Cardiovasc Ther 3:985-91. 2005
    ..Initial studies using the selective oral endothelin-A receptor antagonist sitaxsentan in pulmonary arterial hypertension patients have revealed a favorable risk-benefit therapeutic profile with the 100 mg once-daily dose...
  23. doi request reprint Vasodilator testing with nitric oxide and/or oxygen in pediatric pulmonary hypertension
    Robyn J Barst
    Division of Pediatric Cardiology, Columbia University, Scarsdale, New York, NY 10583, USA
    Pediatr Cardiol 31:598-606. 2010
    ..One-year survival data show similar rates between the iNO/O(2) and the O(2) alone groups; however, the combination may be more effective than O(2) alone in discriminating survivors versus nonsurvivors at long-term follow-up...
  24. ncbi request reprint Sitaxsentan, a selective endothelin-A receptor antagonist for the treatment of pulmonary arterial hypertension
    Evelyn M Horn
    Center for Advanced Cardiac Care, Division of Cardiology, PH 12, 622 West 168th Street, New York, NY 10032, USA
    Expert Opin Investig Drugs 13:1483-92. 2004
    ..Long-term comparative studies are necessary to determine whether there is a clinically meaningful difference between selective ET(A) receptor antagonism and ET(A)/ET(B) receptor antagonism...
  25. pmc Determinants of right ventricular ejection fraction in pulmonary arterial hypertension
    Steven M Kawut
    Department of Medicine, Columbia University, New York, NY, USA
    Chest 135:752-9. 2009
    ..Right ventricular function is a key determinant of exercise capacity and survival in pulmonary arterial hypertension (PAH). We aimed to study the predictors of right ventricular ejection fraction (RVEF) in patients with newly diagnosed PAH...
  26. doi request reprint PET imaging may provide a novel biomarker and understanding of right ventricular dysfunction in patients with idiopathic pulmonary arterial hypertension
    Sabahat Bokhari
    Division of Cardiology, Department of Medicine, New York Presbyterian Hospital at Columbia University Medical Center, New York, NY 10032, USA
    Circ Cardiovasc Imaging 4:641-7. 2011
    ..The objective is to develop PET metabolic imaging of the RV as a noninvasive tool in patients with PAH...
  27. doi request reprint Tadalafil monotherapy and as add-on to background bosentan in patients with pulmonary arterial hypertension
    Robyn J Barst
    Department of Pediatrics and Medicine, Columbia University, New York, New York, USA
    J Heart Lung Transplant 30:632-43. 2011
    ..Inclusion criteria included the option for background bosentan. Analyses of tadalafil in treatment-naive patients and as add-on to bosentan were pre-specified. Objectives were to provide safety and efficacy data for both groups...
  28. ncbi request reprint Eisenmenger's syndrome: current management
    Erika Berman Berman
    Division of Pediatric Cardiology, Department of Pediatrics, Columbia University, College of Physicians and Surgeons, New York, NY, USA
    Prog Cardiovasc Dis 45:129-38. 2002
    ..Although there is no cure for this condition at present, recent advances in management have improved the quality of life for many patients with Eisenmenger's syndrome...
  29. doi request reprint A randomized, double-blind, placebo-controlled, dose-ranging study of oral sildenafil citrate in treatment-naive children with pulmonary arterial hypertension
    Robyn J Barst
    Columbia University, New York, NY, USA
    Circulation 125:324-34. 2012
    ..Safe, effective therapy is needed for pediatric pulmonary arterial hypertension...
  30. doi request reprint Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management
    Robyn J Barst
    Columbia University College of Physicians and Surgeons, New York, NY, USA
    Circulation 125:113-22. 2012
    ..Pulmonary arterial hypertension (PAH) is a rare but important cause of morbidity and mortality in children...
  31. pmc Plasma serotonin levels are normal in pulmonary arterial hypertension
    David J Lederer
    Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY, USA
    Pulm Pharmacol Ther 21:112-4. 2008
    ..Higher 5-HT levels correlated with increased six-minute walk distance (r=0.55, p=0.04). These data suggest that plasma 5-HT levels are normal in PAH. Plasma 5-HT may therefore not be a useful biomarker in this condition...
  32. ncbi request reprint Pulmonary arterial hypertension in children
    Erika Berman Rosenzweig
    Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, New York 10027, USA
    Pediatr Pulmonol 38:2-22. 2004
    ..This report is a review the current state of the art for pulmonary arterial hypertension in 2004, with an emphasis on childhood pulmonary arterial hypertension and specific recommendations for current practice and future directions...
  33. pmc Doppler-defined pulmonary hypertension and the risk of death in children with sickle cell disease followed for a mean of three years
    Margaret T Lee
    Division of Pediatric Hematology, Columbia University College of Physicians and Surgeons, New York, NY, USA
    Br J Haematol 146:437-41. 2009
    ..PH may be a manifestation of progressive organ damage from chronic haemolysis and systemic vasculopathy that ultimately leads to early death in adulthood...
  34. ncbi request reprint Treprostinil therapy for pulmonary artery hypertension
    Evelyn M Horn
    Departments of Medicine and Pediatrics, New York Presbyterian Pulmonary Hypertension Center, New York, NY, USA
    Expert Opin Investig Drugs 11:1615-22. 2002
    ....
  35. ncbi request reprint Identical twins with primary pulmonary hypertension: beraprost vs epoprostenol
    Erika Berman Rosenzweig
    Children s Hospital of New York, Columbia University, College of Physicians and Surgeons, New York, NY, USA
    Chest 125:1157-60. 2004
    ..The course of 12-year-old, homozygotic twins with primary pulmonary hypertension (PPH) treated with different vasoactive agents, beraprost vs epoprostenol, is described...
  36. pmc Novel loci interacting epistatically with bone morphogenetic protein receptor 2 cause familial pulmonary arterial hypertension
    Laura Rodriguez-Murillo
    Division of Statistical Genetics, Department of Biostatistics, Columbia University Medical Center, New York, New York, USA
    J Heart Lung Transplant 29:174-80. 2010
    ..Our goal in this study was to identify genetic loci that may influence FPAH expression in BMPR2 mutation carriers...
  37. ncbi request reprint Selective serotonin reuptake inhibitor use and outcomes in pulmonary arterial hypertension
    Steven M Kawut
    Department of Medicine, College of Physicians and Surgeons, Columbia University, New York City, NY, USA
    Pulm Pharmacol Ther 19:370-4. 2006
    ..We therefore hypothesized that PAH patients treated with high-affinity selective serotonin reuptake inhibitors (SSRIs) would have a reduced risk of death compared to PAH patients not treated with SSRIs...
  38. ncbi request reprint Validation of SPECT equilibrium radionuclide angiographic right ventricular parameters by cardiac magnetic resonance imaging
    Kenneth Nichols
    Division of Cardiology, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY 10032, USA
    J Nucl Cardiol 9:153-60. 2002
    ..The purpose of this investigation was to determine the accuracy of RV functional parameters assessed by single photon emission computed tomography (SPECT) equilibrium radionuclide angiography (ERNA)...
  39. doi request reprint Congenital heart disease and pulmonary hypertension: pharmacology and feasibility of late surgery
    Erika B Rosenzweig
    Columbia University College of Physicians and Surgeons, New York, NY 10032, USA
    Prog Cardiovasc Dis 55:128-33. 2012
    ..Careful evaluation and an understanding of the predominant physiologic features will help guide the management of these complex patients and whether late surgical repair is feasible...
  40. pmc Polymorphism in the angiotensin II type 1 receptor (AGTR1) is associated with age at diagnosis in pulmonary arterial hypertension
    Wendy K Chung
    Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, New York, USA
    J Heart Lung Transplant 28:373-9. 2009
    ..The renin angiotensin aldosterone system (RAAS) provides a set of candidate genes that could modulate pulmonary vascular disease similar to its effects on renal and peripheral vasculature...
  41. ncbi request reprint Lower airway obstruction, bronchial hyperresponsiveness, and primary pulmonary hypertension in children
    Deepa Rastogi
    Division of Pediatric Pulmonology, Department of Pediatrics, College of Physicians and Surgeons of Columbia University and the Morgan Stanley Children s Hospital of New York Presbyterian, New York, New York 10032, USA
    Pediatr Pulmonol 37:50-5. 2004
    ..Our findings suggest that reversible LAO is the most common lung function abnormality among pediatric patients with PPH. Whether airway reactivity is a cause of or the result of PPH remains to be determined...
  42. ncbi request reprint The effect of atrial septostomy on the concentration of brain-type natriuretic peptide in patients with idiopathic pulmonary arterial hypertension
    Erika S Berman Rosenzweig
    Columbia University College of Physicians and Surgeons New York, New York, USA
    Cardiol Young 17:557-9. 2007
    ....
  43. ncbi request reprint Bone morphogenetic protein receptor 2 mutations in adults and children with idiopathic pulmonary arterial hypertension: association with thyroid disease
    Kari E Roberts
    Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY, USA
    Chest 128:618S. 2005
  44. doi request reprint Pulmonary arterial hypertension in children: a medical update
    Erika B Rosenzweig
    Columbia University College of Physicians and Surgeons, New York, NY 10032, USA
    Curr Opin Pediatr 20:288-93. 2008
    ....
  45. doi request reprint Functional class improvement and 3-year survival outcomes in patients with pulmonary arterial hypertension in the REVEAL Registry
    Robyn J Barst
    Columbia University College of Physicians and Surgeons, New York, NY, USA
    Chest 144:160-8. 2013
    ..We assessed whether patients with PAH who improve from FC III to FC I/II have improved survival vs patients who remain at FC III or worsen to FC IV...
  46. ncbi request reprint 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
    ..We conclude that chronic subcutaneous infusion of treprostinil is an effective treatment with an acceptable safety profile in patients with pulmonary arterial hypertension...
  47. doi request reprint Continuous hemodynamic monitoring in patients with pulmonary arterial hypertension
    Robert P Frantz
    Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA
    J Heart Lung Transplant 27:780-8. 2008
    ..The purpose of this study was to determine whether an implantable hemodynamic monitor (IHM) could be used to judge the response of pulmonary arterial hypertension (PAH) patients to changes in therapy...
  48. doi request reprint Sitaxsentan for the treatment of pulmonary arterial hypertension: a 1-year, prospective, open-label observation of outcome and survival
    Raymond L Benza
    University of Alabama at Birmingham, 1900 University Blvd, Tinsley Harrison Towers, Room 328A, Birmingham, AL 35294, USA
    Chest 134:775-82. 2008
    ..Long-term efficacy evaluations are needed to guide therapeutic management. The purpose of this study is to present 1-year observational data with two endothelin antagonists, sitaxsentan and bosentan, in a prospective, open-label study...
  49. doi request reprint Design of the REVEAL registry for US patients with pulmonary arterial hypertension
    Michael D McGoon
    Division of Cardiovascular Diseases, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA
    Mayo Clin Proc 83:923-31. 2008
    ..REVEAL will be the richest source of data on patients with World Health Organization group I PAH...
  50. ncbi request reprint Endothelin receptor antagonists in pulmonary arterial hypertension
    Richard N Channick
    Pulmonary and Critical Care Division, University of California, San Diego, California, USA
    J Am Coll Cardiol 43:62S-67S. 2004
    ..The selective endothelin receptor-A antagonists sitaxsentan and ambrisentan are currently undergoing investigation...
  51. ncbi request reprint Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial
    Gerald Simonneau
    Service de Pneumologie, Hopital Antoine Beclere, Universite Paris Sud, Clamart, France
    Ann Intern Med 149:521-30. 2008
    ..Oral sildenafil and intravenous epoprostenol have independently been shown to be effective in patients with pulmonary arterial hypertension...
  52. ncbi request reprint Prostanoid therapy for pulmonary arterial hypertension
    David B Badesch
    University of Colorado Health Sciences Center, Denver, Colorado, USA
    J Am Coll Cardiol 43:56S-61S. 2004
    ..In conclusion, though treatment with prostanoids is complicated by their generally short half-lives and complicated drug delivery systems, they continue to play an important role in the treatment of PAH...
  53. ncbi request reprint Efficacy and safety of treprostinil: an epoprostenol analog for primary pulmonary hypertension
    Vallerie V McLaughlin
    Rush Presbyterian St Luke s Medical Center, Chicago, Illinois 60612, USA
    J Cardiovasc Pharmacol 41:293-9. 2003
    ..Subcutaneous treprostinil has favorable hemodynamic effects when given acutely and in the short term. Treprostinil can be given safely to an ambulatory patient with a novel subcutaneous delivery pump system...
  54. ncbi request reprint Pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension
    Robyn J Barst
    Actelion Pharmaceuticals Ltd, Department of Clinical Pharmacology, Gewerbestrasse, Allscwil, Switzerland
    Clin Pharmacol Ther 73:372-82. 2003
    ..Little is known about the effects of bosentan in children. This study was conducted to investigate the pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension...
  55. ncbi request reprint Prognostic factors for survival in human immunodeficiency virus-associated pulmonary arterial hypertension
    Hilario Nunes
    UPRES 2705, Centre des Maladies Vasculaires Pulmonaires, Service de Pneumologie et Reanimation Respiratoire, Hopital Antoine Beclere, Assistance Publique Hopitaux de Paris, Universite Paris Sud, Clamart, France
    Am J Respir Crit Care Med 167:1433-9. 2003
    ..These results suggest that patients with severe human immunodeficiency virus-associated PAH should be considered for long-term epoprostenol infusion in association with CART...
  56. ncbi request reprint Transition from intravenous epoprostenol to intravenous treprostinil in pulmonary hypertension
    Mardi Gomberg-Maitland
    Pulmonary Hypertension Center, University of Chicago Hospitals, 5841 South Maryland Avenue, MC 2016, Chicago, IL 60637, USA
    Am J Respir Crit Care Med 172:1586-9. 2005
    ..With the demonstration of bioequivalence between subcutaneous and intravenous treprostinil, intravenous treprostinil may have an overall better risk-benefit profile than intravenous epoprostenol...
  57. ncbi request reprint 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...
  58. ncbi request reprint Micrococcus-associated central venous catheter infection in patients with pulmonary arterial hypertension
    Ronald J Oudiz
    Division of Cardiology, Research and Education Institute, Harbor UCLA Medical Center, Torrance, CA 90502, USA
    Chest 126:90-4. 2004
    ....
  59. doi request reprint Bloodstream infections in patients given treatment with intravenous prostanoids
    Alexander J Kallen
    Division of Healthcare Quality Promotion, National Center for Preparedness, Detection, and Control of Infectious Diseases, Office of Workforce and Career Development, Centers for Disease Control and Prevention, Atlanta, Georgia, USA
    Infect Control Hosp Epidemiol 29:342-9. 2008
    ..An investigation was conducted to determine rates of prostanoid-associated BSI in this patient population and possible risk factors for infection...
  60. ncbi request reprint Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines
    David B Badesch
    University of Colorado Health Sciences Center, Denver, CO 80262, USA
    Chest 126:35S-62S. 2004
    ..Due to the complexity of the diagnostic evaluation required, and the treatment options available, it is strongly recommended that consideration be given to referral of patients with PAH to a specialized center...
  61. ncbi request reprint Cardiopulmonary exercise testing and six-minute walk correlations in pulmonary arterial hypertension
    Ronald J Oudiz
    Saint John s Cardiovascular Research Center, Los Angeles Biomedical Research Institute at Harbor UCLA Medical Center, Torrance, California, USA
    Am J Cardiol 97:123-6. 2006
    ..Weight adjustment of the 6MW improved its correlation with peak VO2. In conclusion, in future multicenter trials, CPET expertise should be validated at all sites before subject enrollment...
  62. ncbi request reprint 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
    ....
  63. ncbi request reprint 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...
  64. ncbi request reprint Sustained symptomatic, functional, and hemodynamic benefit with the selective endothelin-A receptor antagonist, sitaxsentan, in patients with pulmonary arterial hypertension: a 1-year follow-up study
    David Langleben
    Center for Pulmonary Vascular Disease, Room E 258, Sir Mortimer B Davis Jewish General Hospital, 3755 Cote Ste Catherine, Montreal, Quebec, Canada H3T 1E2
    Chest 126:1377-81. 2004
    ..To examine the long-term efficacy and safety of the selective endothelin-A receptor (ET-A) antagonist, sitaxsentan sodium, after 1 year of therapy in patients with pulmonary arterial hypertension (PAH)...
  65. ncbi request reprint Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension
    Vallerie V McLaughlin
    The University of Michigan Health System, Ann Arbor, Michigan, USA
    Am J Respir Crit Care Med 174:1257-63. 2006
    ..Small, open-label studies suggest that combinations of existing therapies may be effective for pulmonary arterial hypertension (PAH)...
  66. ncbi request reprint 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
  67. ncbi request reprint Reproducibility of cardiopulmonary exercise measurements in patients with pulmonary arterial hypertension
    James E Hansen
    Department of Medicine, Harbor UCLA Medical Center, Torrance, CA 90509, USA
    Chest 126:816-24. 2004
    ..Setting and patients: Over a 15-month period at two tertiary-care teaching hospitals, 42 patients with PAH underwent repeated, paired CPETs using cycle ergometry...
  68. pmc Serotonin transporter polymorphisms in familial and idiopathic pulmonary arterial hypertension
    Elisabeth D Willers
    Division of Allergy, Pulmonary, and Critical Care Medicine, Department of Medicine, Vanderbilt University Medical Center, Medical Center North T 1218, Nashville, TN 37232 2650, USA
    Am J Respir Crit Care Med 173:798-802. 2006
    ....
  69. ncbi request reprint Sildenafil for pulmonary arterial hypertension associated with connective tissue disease
    David B Badesch
    Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, Denver, Colorado 80262, USA
    J Rheumatol 34:2417-22. 2007
    ..The phosphodiesterase-5 inhibitor sildenafil citrate enhances vasodilatation, has antiproliferative effects, and is effective in the treatment of PAH. We examined the efficacy and safety of oral sildenafil in patients with PAH-CTD...
  70. ncbi request reprint Sitaxsentan treatment for patients with pulmonary arterial hypertension discontinuing bosentan
    Raymond L Benza
    Division of Cardiology, University of Alabama at Birmingham, Birmingham, Alabama 35294 0006, USA
    J Heart Lung Transplant 26:63-9. 2007
    ..Sitaxsentan, an oral, ET(A)-selective endothelin antagonist currently under investigation, may be an alternative treatment option. In this study we evaluate the safety and efficacy of sitaxsentan in patients discontinuing bosentan...
  71. ncbi request reprint Creation of a model comparing 6-minute walk test to metabolic equivalent in evaluating treatment effects in pulmonary arterial hypertension
    Mardi Gomberg-Maitland
    Section of Cardiology, Department of Medicine, University of Chicago, Chicago, Illinois 60637, USA
    J Heart Lung Transplant 26:732-8. 2007
    ..We compared the Naughton-Balke treadmill test reported in exercise metabolic equivalents (METs) with the 6MWT to evaluate whether MET could be a useful tool to assess exercise capacity...
  72. pmc Sildenafil therapy in patients with sickle cell disease and pulmonary hypertension
    Roberto F Machado
    Vascular Therapeutics Section, Cardiovascular Branch, National Heart Lung and Blood Institute, Bethesda, MD, USA
    Br J Haematol 130:445-53. 2005
    ....
  73. pmc Selective endothelin A receptor antagonism with sitaxsentan for pulmonary arterial hypertension associated with connective tissue disease
    Reda E Girgis
    Johns Hopkins University, School of Medicine, Baltimore, Maryland, USA
    Ann Rheum Dis 66:1467-72. 2007
    ..Short-term clinical and haemodynamic effects and longer-term follow-up data are presented...
  74. ncbi request reprint Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease
    Ronald J Oudiz
    Research and Education Institute, Harbor UCLA Medical Center, Torrance, CA 90502, USA
    Chest 126:420-7. 2004
    ..To assess the efficacy and safety of continuous subcutaneous infusion of treprostinil, a stable prostacyclin analogue, for treating pulmonary arterial hypertension (PAH) in patients with connective tissue disease (CTD)...
  75. ncbi request reprint Right ventricular pressure waveform and wave reflection analysis in patients with pulmonary arterial hypertension
    Mustafa Karamanoglu
    NT and D Research, Medtronic Inc, 7000 Central Ave NE, CW320, Fridley, MN 55432, USA
    Chest 132:37-43. 2007
    ..The aim of this study was to investigate whether the RV pressure waveform utilizing an IHM can be used to estimate the magnitude of pressure wave reflection and cardiac index in patients with IPAH in acute settings...
  76. ncbi request reprint Effects of the thromboxane synthetase inhibitor and receptor antagonist terbogrel in patients with primary pulmonary hypertension
    David Langleben
    Jewish General Hospital, McGill University, Montreal, Quebec, Canada
    Am Heart J 143:E4. 2002
    ..Circulating mediators, including thromboxane A2, the vasoconstrictor, platelet aggregant, and smooth muscle mitogen, may contribute to the progression of vascular narrowing in primary pulmonary hypertension (PPH)...
  77. ncbi request reprint Temporal trends and drug exposures in pulmonary hypertension: an American experience
    Alexander M Walker
    Harvard School of Public Health, Boston, MA, USA
    Am Heart J 152:521-6. 2006
    ..We also studied whether use of anorexigens and other drugs differed among patients with pulmonary hypertension of different etiologies...
  78. ncbi request reprint Evaluation and treatment for angina in pulmonary arterial hypertension
    Robyn J Barst
    Am J Med 116:427-8. 2004
  79. ncbi request reprint The clinician as investigator: participating in clinical trials in the practice setting
    Ellis W Lader
    Circulation 109:2672-9. 2004
    ..Finally, the appendices review basic elements of study design and statistical principles, which may be of interest to a potential investigator...
  80. ncbi request reprint Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers
    Marius M Hoeper
    Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany
    J Am Coll Cardiol 48:2546-52. 2006
    ..This study sought to assess the risks associated with right heart catheter procedures in patients with pulmonary hypertension...