Therapy for Pulmonary Arterial Hypertension: The More, the Merrier?
Authors: Darren B. Taichman
Journal: Annals of Internal Medicine (2008)

Editorials21 October 2008Therapy for Pulmonary Arterial Hypertension: The More, the Merrier?Darren B. Taichman, MD, PhDDarren B. Taichman, MD, PhDFrom University of Pennsylvania, Philadelphia, PA 19104.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-149-8-200810210-00010 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Without effective therapy, patients with pulmonary arterial hypertension (PAH) have inexorably worsening dyspnea and declining exercise capacity and can die, most often of right heart failure. The disease involves dysregulated endothelial and vascular smooth-muscle cell growth, in situ thrombosis, and an imbalance of vasodilators and constrictors. The result is a noncompliant, markedly narrowed blood vessel that resists blood flow from a progressively weakening right ventricle. Less than 20 years ago, the mean survival of patients with idiopathic PAH (formerly called primary) was only 2.8 years. There was little to offer beyond supplemental oxygen, anticoagulants, and diuretics (1).Since then, the ...References1. D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991;115:343-9. [PMID: 1863023] LinkGoogle Scholar2. Badesch DB, Abman SH, Simonneau G, Rubin LJ, McLaughlin VV. Medical therapy for pulmonary arterial hypertension: updated ACCP evidence-based clinical practice guidelines. Chest. 2007;131:1917-28. [PMID: 17565025] CrossrefMedlineGoogle Scholar3. Chin KM, Rubin LJ. Pulmonary arterial hypertension. J Am Coll Cardiol. 2008;51:1527-38. [PMID: 18420094] CrossrefMedlineGoogle Scholar4. Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med. 1996;334:296-302. [PMID: 8532025] CrossrefMedlineGoogle Scholar5. McLaughlin VV, Presberg KW, Doyle RL, Abman SH, McCrory DC, Fortin T, et al; American College of Chest Physicians. Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126:78S-92S. [PMID: 15249497] CrossrefMedlineGoogle Scholar6. Chen H, Taichman DB, Doyle RL. Health-related quality of life and patient-reported outcomes in pulmonary arterial hypertension. Proc Am Thorac Soc. 2008;5:623-30. [PMID: 18625755] CrossrefMedlineGoogle Scholar7. Snow JL, Jones PL, Taichman DB. Pathobiologic mechanisms of pulmonary arterial hypertension.. In: Mandel J, Taichman DB, eds. Pulmonary Vascular Disease. Philadelphia: Elsevier; 2006:33-49. Google Scholar8. Fisher RI, Gaynor ER, Dahlberg S, Oken MM, Grogan TM, Mize EM, et al. Comparison of a standard regimen (CHOP) with three intensive chemotherapy regimens for advanced non-Hodgkin's lymphoma. N Engl J Med. 1993;328:1002-6. [PMID: 7680764] CrossrefMedlineGoogle Scholar9. Anand S, Yusuf S, Xie C, Pogue J, Eikelboom J, Budaj A, et al; Warfarin Antiplatelet Vascular Evaluation Trial Investigators. Oral anticoagulant and antiplatelet therapy and peripheral arterial disease. N Engl J Med. 2007;357:217-27. [PMID: 17634457] CrossrefMedlineGoogle Scholar10. McGoon M, Barst RJ, Doyle RL, Liou TG, Miller D, Feldkircher K. REVEAL Registry: Treatment history and treatment at baseline. Chest. 2007;132:631S. CrossrefGoogle Scholar11. McLaughlin VV, Oudiz RJ, Frost A, Tapson VF, Murali S, Channick RN, et al. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med. 2006;174:1257-63. [PMID: 16946127] CrossrefMedlineGoogle Scholar12. Humbert M, Barst RJ, Robbins IM, Channick RN, Galiè N, Boonstra A, et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J. 2004;24:353-9. [PMID: 15358690] CrossrefMedlineGoogle Scholar13. Simonneau G, Rubin LJ, Galiè N, Barst RJ, Fleming TR, Frost AE, et al. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension. A randomized trial. Ann Intern Med. 2008;149:521-30. LinkGoogle Scholar14. Robbins IM, Christman BW, Newman JH, Matlock R, Loyd JE. A survey of diagnostic practices and the use of epoprostenol in patients with primary pulmonary hypertension. Chest. 1998;114:1269-75. [PMID: 9824000] CrossrefMedlineGoogle Scholar15. McLaughlin VV, Genthner DE, Panella MM, Rich S. Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med. 1998;338:273-7. [PMID: 9445406] CrossrefMedlineGoogle Scholar16. Schermuly RT, Pullamsetti SS, Breitenbach SC, Weissmann N, Ghofrani HA, Grimminger F, et al. Iloprost-induced desensitization of the prostacyclin receptor in isolated rabbit lungs. Respir Res. 2007;8:4. [PMID: 17257398] CrossrefMedlineGoogle Scholar17. Sitbon O, Humbert M, Nunes H, Parent F, Garcia G, Hervé P, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol. 2002;40:780-8. [PMID: 12204511] CrossrefMedlineGoogle Scholar18. McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation. 2002;106:1477-82. [PMID: 12234951] CrossrefMedlineGoogle Scholar19. Rich S. The current treatment of pulmonary arterial hypertension: time to redefine success. Chest. 2006;130:1198-202. [PMID: 17035456] CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAffiliations: From University of Pennsylvania, Philadelphia, PA 19104.Disclosures:Grants received: Actelion.Corresponding Author: Darren B. Taichman, MD, PhD, University of Pennsylvania Medical Center, Pulmonary, Allergy & Critical Care Division, Suite 120, PHI Building, Penn-Presbyterian Medical Center, 51 North 39th Street, Philadelphia, PA 19104; e-mail, darren.[email protected]upenn.edu. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetailsSee AlsoAddition of Sildenafil to Long-Term Intravenous Epoprostenol Therapy in Patients with Pulmonary Arterial Hypertension Gérald Simonneau , Lewis J. Rubin , Nazzareno Galiè , Robyn J. Barst , Thomas R. Fleming , Adaani E. Frost , Peter J. Engel , Mordechai R. Kramer , Gary Burgess , Lorraine Collings , Nandini Cossons , Olivier Sitbon , David B. 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