Circulation, Vol 70, 580-587, Copyright © 1984 by American Heart Association
V Fuster, PM Steele, WD Edwards, BJ Gersh, MD McGoon and RL Frye
A long-term retrospective follow-up study was made of 120 patients (33
male, 87 female patients) with primary pulmonary hypertension-- diagnosed
by strict clinical and hemodynamic criteria--to obtain a better
understanding of the natural history and possible pathogenetic mechanisms
of the disease. The mean age at diagnosis was 34 (3 to 64) years, but only
24 patients (21%) remained alive 5 years later. Lung tissue obtained at
autopsy from 56 patients revealed two major pathologic types:
thromboembolic pulmonary hypertension in 32 patients (57%) and plexogenic
pulmonary arteriopathy in 18 (32%). Thus, in more than half the patients
undergoing autopsy the major histologic feature was thrombi without any
evidence of plexiform lesions. The two groups were similar with respect to
their clinical and hemodynamic features and short survival. Of the
variables tested for prognostic importance by stepwise multivariate
analysis, only two were significant: pulmonary arterial oxygen saturation
(p less than .00001) and anticoagulant therapy (p = .01). Anticoagulant
therapy is recommended for patients with primary pulmonary hypertension.
ARTICLES
Primary pulmonary hypertension: natural history and the importance of thrombosis
This article has been cited by other articles:
 |
|
 |
|
  The Task Force for the Diagnosis and Treatment of, N. Galie, M. M. Hoeper, M. Humbert, A. Torbicki, J-L. Vachiery, J. A. Barbera, M. Beghetti, P. Corris, S. Gaine, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension Eur. Respir. J., December 1, 2009; 34(6): 1219 - 1263. [Full Text] [PDF]
|
|
|
|
 |
|
 Authors/Task Force Members, N. Galie, M. M. Hoeper, M. Humbert, A. Torbicki, J.-L. Vachiery, J. A. Barbera, M. Beghetti, P. Corris, S. Gaine, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT) Eur. Heart J., October 2, 2009; 30(20): 2493 - 2537. [Full Text] [PDF]
|
|
|
|
 |
|
 D. B. Badesch, H. C. Champion, M. A. Gomez Sanchez, M. M. Hoeper, J. E. Loyd, A. Manes, M. McGoon, R. Naeije, H. Olschewski, R. J. Oudiz, et al. Diagnosis and assessment of pulmonary arterial hypertension. J. Am. Coll. Cardiol., June 30, 2009; 54(1 Suppl): S55 - S66. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. J. Barst, J. S. R. Gibbs, H. A. Ghofrani, M. M. Hoeper, V. V. McLaughlin, L. J. Rubin, O. Sitbon, V. F. Tapson, and N. Galie Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J. Am. Coll. Cardiol., June 30, 2009; 54(1 Suppl): S78 - S84. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
V. V. McLaughlin, D. B. Badesch, M. Delcroix, T. R. Fleming, S. P. Gaine, N. Galie, J. S. R. Gibbs, N. H. Kim, R. J. Oudiz, A. Peacock, et al. End Points and Clinical Trial Design in pulmonary arterial hypertension. J. Am. Coll. Cardiol., June 30, 2009; 54(1 Suppl): S97 - 107. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
V. V. McLaughlin, S. L. Archer, D. B. Badesch, R. J. Barst, H. W. Farber, J. R. Lindner, M. A. Mathier, M. D. McGoon, M. H. Park, R. S. Rosenson, et al. ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension: A Report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association Developed in Collaboration With the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association J. Am. Coll. Cardiol., April 28, 2009; 53(17): 1573 - 1619. [Full Text] [PDF]
|
|
|
|
 |
|
 Writing Committee Members, V. V. McLaughlin, S. L. Archer, D. B. Badesch, R. J. Barst, H. W. Farber, J. R. Lindner, M. A. Mathier, M. D. McGoon, M. H. Park, et al. ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension: A Report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: Developed in Collaboration With the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association Circulation, April 28, 2009; 119(16): 2250 - 2294. [Full Text] [PDF]
|
|
|
|
 |
|
 S G Haworth and A A Hislop Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006 Heart, February 1, 2009; 95(4): 312 - 317. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. D. McGoon and G. C. Kane Pulmonary Hypertension: Diagnosis and Management Mayo Clin. Proc., February 1, 2009; 84(2): 191 - 207. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 N. Galiè and A. Manes CHAPTER 24 Pulmonary Hypertension ESC Textbook of Cardiovascular Medicine, January 1, 2009; 2(1): med-9780199566990-chapter - med-9780199566990-chapter. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. M. Majka, M. Skokan, L. Wheeler, J. Harral, S. Gladson, E. Burnham, J. E. Loyd, K. R. Stenmark, M. Varella-Garcia, and J. West Evidence for cell fusion is absent in vascular lesions associated with pulmonary arterial hypertension Am J Physiol Lung Cell Mol Physiol, December 1, 2008; 295(6): L1028 - L1039. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A U Wells, N Hirani, and on behalf of the BTS Interstitial Lung Disease Gui Interstitial lung disease guideline Thorax, September 1, 2008; 63(Suppl_5): v1 - v58. [Full Text] [PDF]
|
|
|
|
 |
|
 K. Boutet, D. Montani, X. Jais, A. Yaici, O. Sitbon, G. Simonneau, and M. Humbert Review: Therapeutic advances in pulmonary arterial hypertension Therapeutic Advances in Respiratory Disease, August 1, 2008; 2(4): 249 - 265. [Abstract] [PDF]
|
|
|
|
|
|
National Pulmonary Hypertension Centres of the UK Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland Heart, March 1, 2008; 94(Suppl_1): i1 - i41. [Full Text] [PDF]
|
|
|
|
|
|
National Pulmonary Hypertension Centres of the UK Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland Thorax, March 1, 2008; 63(Suppl_2): ii1 - ii41. [Full Text] [PDF]
|
|
|
|
 |
|
 K. I. Ataga, C. G. Moore, C. A. Hillery, S. Jones, H. C. Whinna, D. Strayhorn, C. Sohier, A. Hinderliter, L. V. Parise, and E. P. Orringer Coagulation activation and inflammation in sickle cell disease-associated pulmonary hypertension Haematologica, January 1, 2008; 93(1): 20 - 26. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. M. Chin, R. N. Channick, N. H. Kim, and L. J. Rubin Central Venous Blood Oxygen Saturation Monitoring in Patients With Chronic Pulmonary Arterial Hypertension Treated With Continuous IV Epoprostenol: Correlation With Measurements of Hemodynamics and Plasma Brain Natriuretic Peptide Levels Chest, September 1, 2007; 132(3): 786 - 792. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. J. White, D. F. Meoli, R. F. Swarthout, D. Y. Kallop, I. I. Galaria, J. L. Harvey, C. M. Miller, B. C. Blaxall, C. M. Hall, R. A. Pierce, et al. Plexiform-like lesions and increased tissue factor expression in a rat model of severe pulmonary arterial hypertension Am J Physiol Lung Cell Mol Physiol, September 1, 2007; 293(3): L583 - L590. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. Suntharalingam, R. D Machado, L. D Sharples, M. R Toshner, K. K Sheares, R. J Hughes, D. P Jenkins, R. C Trembath, N. W Morrell, and J. Pepke-Zaba Demographic features, BMPR2 status and outcomes in distal chronic thromboembolic pulmonary hypertension Thorax, July 1, 2007; 62(7): 617 - 622. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Takaoka, J. L. Faul, and R. Doyle Current Therapies for Pulmonary Arterial Hypertension Seminars in Cardiothoracic and Vascular Anesthesia, June 1, 2007; 11(2): 137 - 148. [Abstract] [PDF]
|
|
|
|
|
|
K. G. Blyth, B. A. Groenning, P. B. Mark, T. N. Martin, J. E. Foster, T. Steedman, J. J. Morton, H. J. Dargie, and A. J. Peacock NT-proBNP can be used to detect right ventricular systolic dysfunction in pulmonary hypertension Eur. Respir. J., April 1, 2007; 29(4): 737 - 744. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. R. Johnson, S. Mehta, and J. T. Granton Anticoagulation in pulmonary arterial hypertension: a qualitative systematic review. Eur. Respir. J., November 1, 2006; 28(5): 999 - 1004. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D J Fox and R S Khattar Pulmonary arterial hypertension: classification, diagnosis and contemporary management Postgrad. Med. J., November 1, 2006; 82(973): 717 - 722. [Full Text] [PDF]
|
|
|
|
|
|
E. Cenedese, R. Speich, L. Dorschner, S. Ulrich, M. Maggiorini, R. Jenni, and M. Fischler Measurement of quality of life in pulmonary hypertension and its significance Eur. Respir. J., October 1, 2006; 28(4): 808 - 815. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H. A. Ghofrani, R. Voswinckel, F. Reichenberger, N. Weissmann, R. T. Schermuly, W. Seeger, and F. Grimminger Hypoxia- and non-hypoxia-related pulmonary hypertension - Established and new therapies Cardiovasc Res, October 1, 2006; 72(1): 30 - 40. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 O. Distler and A. Pignone Pulmonary arterial hypertension and rheumatic diseases--from diagnosis to treatment Rheumatology, October 1, 2006; 45(suppl_4): iv22 - iv25. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
V. V. McLaughlin and M. D. McGoon Pulmonary Arterial Hypertension Circulation, September 26, 2006; 114(13): 1417 - 1431. [Full Text] [PDF]
|
|
|
|
 |
|
 E. Gurbanov and X. Shiliang The key role of apoptosis in the pathogenesis and treatment of pulmonary hypertension. Eur. J. Cardiothorac. Surg., September 1, 2006; 30(3): 499 - 507. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Ulrich, M. Fischler, R. Speich, V. Popov, and M. Maggiorini Chronic thromboembolic and pulmonary arterial hypertension share acute vasoreactivity properties. Chest, September 1, 2006; 130(3): 841 - 846. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. R. Johnson, J. T. Granton, and S. Mehta Thrombotic Arteriopathy and Anticoagulation in Pulmonary Hypertension. Chest, August 1, 2006; 130(2): 545 - 552. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. H. Caramuru, A. A. Lopes, N. Y. Maeda, V. D. Aiello, and C. C. Filho Long-term Behavior of Endothelial and Coagulation Markers in Eisenmenger Syndrome Clinical and Applied Thrombosis/Hemostasis, April 1, 2006; 12(2): 175 - 183. [Abstract] [PDF]
|
|
|
|
 |
|
 J G Coghlan and C Handler Connective tissue associated pulmonary arterial hypertension Lupus, March 1, 2006; 15(3): 138 - 142. [Abstract] [PDF]
|
|
|
|
|
|
S. Mahapatra, R. A. Nishimura, P. Sorajja, S. Cha, and M. D. McGoon Relationship of Pulmonary Arterial Capacitance and Mortality in Idiopathic Pulmonary Arterial Hypertension J. Am. Coll. Cardiol., February 21, 2006; 47(4): 799 - 803. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. J. Rubin Pulmonary arterial hypertension. Proceedings of the ATS, January 1, 2006; 3(1): 111 - 115. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S Mehta and G J Shoemaker Improving survival in idiopathic pulmonary arterial hypertension: revisiting the "kingdom of the near-dead" Thorax, December 1, 2005; 60(12): 981 - 983. [Full Text] [PDF]
|
|
|
|
|
|
A. Ogawa, K. Nakamura, H. Matsubara, H. Fujio, T. Ikeda, K. Kobayashi, I. Miyazaki, M. Asanuma, K. Miyaji, D. Miura, et al. Prednisolone Inhibits Proliferation of Cultured Pulmonary Artery Smooth Muscle Cells of Patients With Idiopathic Pulmonary Arterial Hypertension Circulation, September 20, 2005; 112(12): 1806 - 1812. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. J. Rubin and D. B. Badesch Evaluation and Management of the Patient with Pulmonary Arterial Hypertension Ann Intern Med, August 16, 2005; 143(4): 282 - 292. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T. Humpl, J. T. Reyes, H. Holtby, D. Stephens, and I. Adatia Beneficial Effect of Oral Sildenafil Therapy on Childhood Pulmonary Arterial Hypertension: Twelve-Month Clinical Trial of a Single-Drug, Open-Label, Pilot Study Circulation, June 21, 2005; 111(24): 3274 - 3280. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. Ford Pulmonary artery hypertension: new drug treatment in children Arch. Dis. Child. Ed. Pract., June 1, 2005; 90(1): ep15 - ep20. [Full Text] [PDF]
|
|
|
|
 |
|
 L Robertson, A Pignone, O Kowal-Bielecka, G Fiori, C P Denton, and M Matucci-Cerinic Pulmonary arterial hypertension in systemic sclerosis: diagnostic pathway and therapeutic approach Ann Rheum Dis, June 1, 2005; 64(6): 804 - 807. [Full Text] [PDF]
|
|
|
|
|
|
V. V. McLaughlin, O. Sitbon, D. B. Badesch, R. J. Barst, C. Black, N. Galie, M. Rainisio, G. Simonneau, and L. J. Rubin Survival with first-line bosentan in patients with primary pulmonary hypertension Eur. Respir. J., February 1, 2005; 25(2): 244 - 249. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Task Force members, N. Galie, A. Torbicki, R. Barst, P. Dartevelle, S. Haworth, T. Higenbottam, H. Olschewski, A. Peacock, G. Pietra, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension: The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology Eur. Heart J., December 2, 2004; 25(24): 2243 - 2278. [Full Text] [PDF]
|
|
|
|
 |
|
 M. Humbert, O. Sitbon, and G. Simonneau Treatment of Pulmonary Arterial Hypertension N. Engl. J. Med., September 30, 2004; 351(14): 1425 - 1436. [Full Text] [PDF]
|
|
|
|
|
|
D. Yung, A. C. Widlitz, E. B. Rosenzweig, D. Kerstein, G. Maislin, and R. J. Barst Outcomes in Children With Idiopathic Pulmonary Arterial Hypertension Circulation, August 10, 2004; 110(6): 660 - 665. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. B. Badesch, S. H. Abman, G. S. Ahearn, R. J. Barst, D. C. McCrory, G. Simonneau, and V. V. McLaughlin Medical Therapy For Pulmonary Arterial Hypertension: ACCP Evidence-Based Clinical Practice Guidelines Chest, July 1, 2004; 126(1_suppl): 35S - 62S. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
V. V. McLaughlin, K. W. Presberg, R. L. Doyle, S. H. Abman, D. C. McCrory, T. Fortin, and G. Ahearn Prognosis of Pulmonary Arterial Hypertension*: ACCP Evidence-Based Clinical Practice Guidelines Chest, July 1, 2004; 126(1_suppl): 78S - 92S. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. H. Newman, B. L. Fanburg, S. L. Archer, D. B. Badesch, R. J. Barst, J. G.N. Garcia, P. N. Kao, J. A. Knowles, J. E. Loyd, M. D. McGoon, et al. Pulmonary Arterial Hypertension: Future Directions: Report of a National Heart, Lung and Blood Institute/Office of Rare Diseases Workshop Circulation, June 22, 2004; 109(24): 2947 - 2952. [Full Text] [PDF]
|
|
|
|
|
|
N. Galie, W. Seeger, R. Naeije, G. Simonneau, and L. J. Rubin Comparative analysis of clinical trials and evidence-based treatment algorithm in pulmonary arterial hypertension J. Am. Coll. Cardiol., June 16, 2004; 43(12_Suppl_S): 81S - 88S. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. L. Eells Advances in Prostacyclin Therapy for Pulmonary Arterial Hypertension Crit. Care Nurse, April 1, 2004; 24(2): 42 - 54. [Full Text] [PDF]
|
|
|
|
|
|
P. Dartevelle, E. Fadel, S. Mussot, A. Chapelier, P. Herve, M. de Perrot, J. Cerrina, F.L. Ladurie, D. Lehouerou, M. Humbert, et al. Chronic thromboembolic pulmonary hypertension Eur. Respir. J., April 1, 2004; 23(4): 637 - 648. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. H. Leuchte, M. Schwaiblmair, R. A. Baumgartner, C. F. Neurohr, T. Kolbe, and J. Behr Hemodynamic Response to Sildenafil, Nitric Oxide, and Iloprost in Primary Pulmonary Hypertension Chest, February 1, 2004; 125(2): 580 - 586. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. K. Silversides, J. T. Granton, E. Konen, M. A. Hart, G. D. Webb, and J. Therrien Pulmonary thrombosis in adults with Eisenmenger syndrome J. Am. Coll. Cardiol., December 3, 2003; 42(11): 1982 - 1987. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Mehta Drug Therapy for Pulmonary Arterial Hypertension: What's on the Menu Today? Chest, December 1, 2003; 124(6): 2045 - 2049. [Full Text] [PDF]
|
|
|
|
|
|
K. B. Highland, C. Strange, J. Mazur, and K. N. Simpson Treatment of Pulmonary Arterial Hypertension: A Preliminary Decision Analysis Chest, December 1, 2003; 124(6): 2087 - 2092. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. H. Caramuru, R. D. P. S. Soares, N. Y. Maeda, and A. A. Lopes Hypoxia and Altered Platelet Behavior Influence von Willebrand Factor Multimeric Composition in Secondary Pulmonary Hypertension Clinical and Applied Thrombosis/Hemostasis, July 1, 2003; 9(3): 251 - 258. [Abstract] [PDF]
|
|
|
|
|
|
O. Sitbon, D. B. Badesch, R. N. Channick, A. Frost, I. M. Robbins, G. Simonneau, V. F. Tapson, and L. J. Rubin Effects of the Dual Endothelin Receptor Antagonist Bosentan in Patients With Pulmonary Arterial Hypertension: A 1-Year Follow-up Study Chest, July 1, 2003; 124(1): 247 - 254. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. M Murphey and E. H Hood Bosentan and Warfarin Interaction Ann. Pharmacother., July 1, 2003; 37(7): 1028 - 1031. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Widlitz and R.J. Barst Pulmonary arterial hypertension in children Eur. Respir. J., January 1, 2003; 21(1): 155 - 176. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Eddahibi, N. Morrell, M-P. d'Ortho, R. Naeije, and S. Adnot Pathobiology of pulmonary arterial hypertension Eur. Respir. J., December 1, 2002; 20(6): 1559 - 1572. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. Chemla, V. Castelain, P. Herve, Y. Lecarpentier, and S. Brimioulle Haemodynamic evaluation of pulmonary hypertension Eur. Respir. J., November 1, 2002; 20(5): 1314 - 1331. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. Shitrit, D. Bendayan, A. Bar-Gil-Shitrit, M. Huerta, B. Rudensky, G. Fink, and M. R. Kramer Significance of a Plasma D-dimer Test in Patients With Primary Pulmonary Hypertension Chest, November 1, 2002; 122(5): 1674 - 1678. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
N. Galie, A. Manes, and A. Branzi The new clinical trials on pharmacological treatment in pulmonary arterial hypertension Eur. Respir. J., October 1, 2002; 20(4): 1037 - 1049. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. Chatterjee, T. De Marco, and J. S. Alpert Pulmonary Hypertension: Hemodynamic Diagnosis and Management Arch Intern Med, September 23, 2002; 162(17): 1925 - 1933. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Ueno, T. Miyauchi, S. Sakai, R. Yamauchi-Kohno, K. Goto, and I. Yamaguchi A combination of oral endothelin-areceptor antagonist and oral prostacyclinanalogue is superior to each drug alone inameliorating pulmonary hypertension in rats J. Am. Coll. Cardiol., July 3, 2002; 40(1): 175 - 181. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Humbert, P. Labrune, O. Sitbon, C. Le Gall, J. Callebert, P. Herve, D. Samuel, R. Machado, R. Trembath, L. Drouet, et al. Pulmonary arterial hypertension and type-I glycogen-storage disease: the serotonin hypothesis Eur. Respir. J., July 1, 2002; 20(1): 59 - 65. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. J. Raymond, A. L. Hinderliter, P. W. Willis IV, D. Ralph, E. J. Caldwell, W. Williams, N. A. Ettinger, N. S. Hill, W. R. Summer, B. de Boisblanc, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension J. Am. Coll. Cardiol., April 3, 2002; 39(7): 1214 - 1219. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. J. Rubin, D. B. Badesch, R. J. Barst, N. Galie, C. M. Black, A. Keogh, T. Pulido, A. Frost, S. Roux, I. Leconte, et al. Bosentan Therapy for Pulmonary Arterial Hypertension N. Engl. J. Med., March 21, 2002; 346(12): 896 - 903. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 N. H.S. Kim and L. J. Rubin Endothelin in Health and Disease: Endothelin Receptor Antagonists in the Management of Pulmonary Artery Hypertension Journal of Cardiovascular Pharmacology and Therapeutics, March 1, 2002; 7(1): 9 - 19. [Abstract] [PDF]
|
|
|
|
|
|
M. Beghetti, G. Reber, P. de Moerloose, L. Vadas, A. Chiappe, I. Spahr-Schopfer, and P.C. Rimensberger Aerosolized iloprost induces a mild but sustained inhibition of platelet aggregation Eur. Respir. J., March 1, 2002; 19(3): 518 - 524. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M.M. Hoeper Pulmonary hypertension in collagen vascular disease Eur. Respir. J., March 1, 2002; 19(3): 571 - 576. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. DORFMULLER, V. ZARKA, I. DURAND-GASSELIN, G. MONTI, K. BALABANIAN, G. GARCIA, F. CAPRON, A. COULOMB-LHERMINE, A. MARFAING-KOKA, G. SIMONNEAU, et al. Chemokine RANTES in Severe Pulmonary Arterial Hypertension Am. J. Respir. Crit. Care Med., February 15, 2002; 165(4): 534 - 539. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Wilkens, A. Guth, J. Konig, N. Forestier, B. Cremers, B. Hennen, M. Bohm, and G. W. Sybrecht Effect of Inhaled Iloprost Plus Oral Sildenafil in Patients With Primary Pulmonary Hypertension Circulation, September 11, 2001; 104(11): 1218 - 1222. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
British Cardiac Society Guidelines and Medical Pra Recommendations on the management of pulmonary hypertension in clinical practice Heart, September 1, 2001; 86(90001): i1 - 13. [Full Text] [PDF]
|
|
|
|
 |
|
 L. Monnery, J. Nanson, and G. Charlton Primary pulmonary hypertension in pregnancy; a role for novel vasodilators Br. J. Anaesth., August 1, 2001; 87(2): 295 - 298. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. J. MacGregor, R. Canavan, C. Knight, C. P. Denton, J. Davar, J. Coghlan, and C. M. Black Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival Rheumatology, April 1, 2001; 40(4): 453 - 459. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
N. Galie Do we need controlled clinical trials in pulmonary arterial hypertension? Eur. Respir. J., January 1, 2001; 17(1): 1 - 3. [Full Text] [PDF]
|
|
|
|
|
|
T. Higenbottam and T. Siddons Trials of inhaled Iloprost and other new vasodilating prostaglandins Eur. Respir. J., January 1, 2001; 17(1): 6 - 7. [Full Text] [PDF]
|
|
|
|
|
|
T. M. Hyers, G. Agnelli, R. D. Hull, T. A. Morris, M. Samama, V. Tapson, and J. G. Weg Antithrombotic Therapy for Venous Thromboembolic Disease Chest, January 1, 2001; 119 (2009): 176S - 193S. [Full Text] [PDF]
|
|
|
|
 |
|
 S. Gaine Pulmonary Hypertension JAMA, December 27, 2000; 284(24): 3160 - 3168. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. Sakamaki, S. Kyotani, N. Nagaya, N. Sato, H. Oya, T. Satoh, and N. Nakanishi Increased Plasma P-Selectin and Decreased Thrombomodulin in Pulmonary Arterial Hypertension Were Improved by Continuous Prostacyclin Therapy Circulation, November 28, 2000; 102(22): 2720 - 2725. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. J. Williamson, L. L. Wallman, R. Jones, A. M. Keogh, F. Scroope, BEd, R. Penny, C. Weber, and P. S. Macdonald Hemodynamic Effects of Bosentan, an Endothelin Receptor Antagonist, in Patients With Pulmonary Hypertension Circulation, July 25, 2000; 102(4): 411 - 418. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A J Peacock Rare diseases bullet 5: Primary pulmonary hypertension Thorax, December 1, 1999; 54(12): 1107 - 1118. [Full Text]
|
|
|
|
|
|
R. J. Barst Do No Harm Chest, November 1, 1999; 116(5): 1147 - 1148. [Full Text] [PDF]
|
|
|
|
|
|
N. Nagaya, M. Uematsu, Y. Okano, T. Satoh, S. Kyotani, F. Sakamaki, N. Nakanishi, K. Miyatake, and T. Kunieda Effect of orally active prostacyclin analogue on survival of outpatients with primary pulmonary hypertension J. Am. Coll. Cardiol., October 1, 1999; 34(4): 1188 - 1192. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. Wax, R. Garofano, and R. J. Barst Effects of Long-term Infusion of Prostacyclin on Exercise Performance in Patients With Primary Pulmonary Hypertension Chest, October 1, 1999; 116(4): 914 - 920. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Kesten, J. Dainauskas, V. McLaughlin, and S. Rich Development of Nonspecific Interstitial Pneumonitis Associated With Long-term Treatment of Primary Pulmonary Hypertension With Prostacyclin Chest, August 1, 1999; 116(2): 566 - 569. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. D. Cool, J. S. Stewart, P. Werahera, G. J. Miller, R. L. Williams, N. F. Voelkel, and R. M. Tuder Three-Dimensional Reconstruction of Pulmonary Arteries in Plexiform Pulmonary Hypertension Using Cell-Specific Markers : Evidence for a Dynamic and Heterogeneous Process of PulmonaryEndothelial Cell Growth Am. J. Pathol., August 1, 1999; 155(2): 411 - 419. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
V. V. McLaughlin, D. E. Genthner, M. M. Panella, D. M. Hess, and S. Rich Compassionate Use of Continuous Prostacyclin in the Management of Secondary Pulmonary Hypertension: A Case Series Ann Intern Med, May 4, 1999; 130(9): 740 - 743. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. B. Rosenzweig, D. Kerstein, and R. J Barst Long-Term Prostacyclin for Pulmonary Hypertension With Associated Congenital Heart Defects Circulation, April 13, 1999; 99(14): 1858 - 1865. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. J. Barst, G. Maislin, and A. P. Fishman Vasodilator Therapy for Primary Pulmonary Hypertension in Children Circulation, March 9, 1999; 99(9): 1197 - 1208. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
O. Sanchez, M. Humbert, O. Sitbon, and G. Simonneau Treatment of pulmonary hypertension secondary to connective tissue diseases Thorax, March 1, 1999; 54(3): 273 - 277. [Full Text]
|
|
|
|
 |
|
 S. G Haworth Primary pulmonary hypertension in childhood Arch. Dis. Child., November 1, 1998; 79(5): 452 - 455. [Full Text]
|
|
|
|
|
|
M. J. Ricciardi, B. P. Knight, F. J. Martinez, and M. Rubenfire Inhaled nitric oxide in primary pulmonary hypertension: A safe and effective agent for predicting response to nifedipine J. Am. Coll. Cardiol., October 1, 1998; 32(4): 1068 - 1073. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L-S Tam and E K Li Successful treatment with immunosuppression, anticoagulation and vasodilator therapy of pulmonary hypertension in SLE associated with secondary antiphospholipid syndrome Lupus, September 1, 1998; 7(7): 495 - 497. [Abstract] [PDF]
|
|
|
|
|
|
T Higenbottam, A Y Butt, A McMahon, R Westerbeck, and L Sharples Long term intravenous prostaglandin (epoprostenol or iloprost) for treatment of severe pulmonary hypertension Heart, August 1, 1998; 80(2): 151 - 155. [Abstract] [Full Text]
|
|