This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Ghofrani, H. A. Articles by Rubin, L. J. Search for Related Content PubMed PubMed Citation Articles by Ghofrani, H. A. Articles by Rubin, L. J.

STATE-OF-THE-ART PAPER

Future Perspectives for the Treatment of Pulmonary Arterial Hypertension

Hossein A. Ghofrani, MD^_*,_*,_*, Robyn J. Barst, MD, Raymond L. Benza, MD, Hunter C. Champion, MD, PhD, Karen A. Fagan, MD^||, Friedrich Grimminger, MD, PhD^_*,_*, Marc Humbert, MD, PhD^¶, Gérald Simonneau, MD^¶, Duncan J. Stewart, MD^#, Carlo Ventura, MD, PhD^_** and Lewis J. Rubin, MD

^_* University of Giessen Lung Center, University Hospital Giessen and Marburg GmbH, Giessen, Germany
Columbia University, New York, New York
Drexel University College of Medicine, Section of Heart Failure, Transplantation, and Pulmonary Hypertension Program, Allegheny General Hospital, Pittsburgh, Pennsylvania
Division of Cardiology, Johns Hopkins University, Baltimore, Maryland
^|| Division of Pulmonary and Critical Care Medicine, University of South Alabama, Mobile, Alabama
^¶ Université Paris-Sud, Service de Pneumologie, Hôpital Antoine-Béclère, Clamart, France
^# Ottawa Health Research Institute, Ottawa, Ontario, Canada
^_** University of Bologna, Laboratory of Molecular Biology and Stem Cell Engineering, National Institute of Biostructures and Biosystems, Institute of Cardiology, S.Orsola-Malpighi Hospital, Bologna, Italy
University of California, San Diego, Medical Center, La Jolla, California
^_* Medical Clinic IV and V, University Hospital Giessen and Marburg GmbH, Giessen, Germany

Manuscript received February 6, 2009; accepted April 16, 2009.

^_* Reprint requests and correspondence: Dr. Hossein A. Ghofrani, Pulmonary Hypertension Division, Medical Clinic II/V, University of Giessen Lung Center, University Hospital Giessen and Marburg GmbH, Klinikstrasse 36, 35392 Giessen, Germany (Email: ardeschir.ghofrani{at}uglc.de).

Over the past 2 decades, pulmonary arterial hypertension has evolved from a uniformly fatal condition to a chronic, manageable disease in many cases, the result of unparalleled development of new therapies and advances in early diagnosis. However, none of the currently available therapies is curative, so the search for new treatment strategies continues. With a deeper understanding of the genetics and the molecular mechanisms of pulmonary vascular disorders, we are now at the threshold of entering a new therapeutic era. Our working group addressed what can be expected in the near future. The topics span the understanding of genetic variations, novel antiproliferative treatments, the role of stem cells, the right ventricle as a therapeutic target, and strategies and challenges for the translation of novel experimental findings into clinical practice.

Key Words: treatment • pulmonary arterial hypertension • PAH

Abbreviations and Acronyms   ANP = atrial natriuretic peptide   BMP = bone morphogenetic protein   BMPR2 = bone morphogenetic protein receptor type 2   BNP = brain natriuretic peptide   EGFR = epidermal growth factor receptor   EPC = endothelial progenitor cell   eNOS = endothelial nitric oxide synthase   ET = endothelin   ETA = ET-1 receptor A   ETB = ET-1 receptor B   Gs = G subunit   GPCR = G-protein–coupled receptor   5-HT = serotonin   IPAH = idiopathic pulmonary arterial hypertension   Kv = voltage-dependent potassium channel   MCT = monocrotaline   MI = myocardial infarction   NADPH = nicotinamide adenine dinucleotide phosphate   NO = nitric oxide   PAH = pulmonary arterial hypertension   PASMC = pulmonary artery smooth muscle cell   PDGF = platelet-derived growth factor   PET = positron emission tomography   PGI2 = prostacyclin   PH = pulmonary hypertension   ROS = reactive oxygen species   RV = right ventricular   SERT = serotonin transporter   SMC = smooth muscle cell   TGF = transforming growth factor   VEGF = vascular endothelial growth factor

This article has been cited by other articles:

				F. S. de Man, M. L. Handoko, J. J. M. van Ballegoij, I. Schalij, S. J. P. Bogaards, P. E. Postmus, J. van der Velden, N. Westerhof, W. J. Paulus, and A. Vonk-Noordegraaf Bisoprolol Delays Progression Towards Right Heart Failure in Experimental Pulmonary Hypertension Circ Heart Fail, January 1, 2012; 5(1): 97 - 105. [Abstract] [Full Text] [PDF]

				L. Ikonomou, A. R. Hemnes, G. Bilousova, R. Hamid, J. E. Loyd, A. K. Hatzopoulos, D. N. Kotton, S. M. Majka, and E. D. Austin Programmatic change: lung disease research in the era of induced pluripotency Am J Physiol Lung Cell Mol Physiol, December 1, 2011; 301(6): L830 - L835. [Abstract] [Full Text] [PDF]

				C. Weber, M. Silver, D. Cromer, S. Kaminski, T. Wirick, and J. Vallejo Under Pressure: Pulmonary Arterial Hypertension . . . A Mother's Struggle Crit. Care Nurse, August 1, 2011; 31(4): 87 - 94. [Abstract] [Full Text] [PDF]

				F. S. de Man, H. W. H. van Hees, M. L. Handoko, H. W. Niessen, I. Schalij, M. Humbert, P. Dorfmuller, O. Mercier, H.-J. Bogaard, P. E. Postmus, et al. Diaphragm Muscle Fiber Weakness in Pulmonary Hypertension Am. J. Respir. Crit. Care Med., May 15, 2011; 183(10): 1411 - 1418. [Abstract] [Full Text] [PDF]

				C. Jardim, S. Hoette, and R. Souza Contemporary issues in pulmonary hypertension Eur. Respir. Rev., December 1, 2010; 19(118): 266 - 271. [Full Text] [PDF]

				R. L. Cordina and D. S. Celermajer Therapeutic approaches in adults with congenital heart disease-associated pulmonary arterial hypertension Eur. Respir. Rev., December 1, 2010; 19(118): 300 - 307. [Abstract] [Full Text] [PDF]

				S. Sakao, K. Tatsumi, and N. F. Voelkel Reversible or Irreversible Remodeling in Pulmonary Arterial Hypertension Am. J. Respir. Cell Mol. Biol., December 1, 2010; 43(6): 629 - 634. [Abstract] [Full Text] [PDF]

				M. A. Matthay, B. T. Thompson, E. J. Read, D. H. McKenna Jr, K. D. Liu, C. S. Calfee, and J. W. Lee Therapeutic Potential of Mesenchymal Stem Cells for Severe Acute Lung Injury Chest, October 1, 2010; 138(4): 965 - 972. [Abstract] [Full Text] [PDF]

				C. G. Elliott, R. J. Barst, W. Seeger, M. Porres-Aguilar, L. M. Brown, R. T. Zamanian, and L. J. Rubin Worldwide Physician Education and Training in Pulmonary Hypertension: Pulmonary Vascular Disease: The Global Perspective Chest, June 1, 2010; 137(6_suppl): 85S - 94S. [Abstract] [Full Text] [PDF]

				M. L. Handoko, F. S. de Man, C. P. Allaart, W. J. Paulus, N. Westerhof, and A. Vonk-Noordegraaf Perspectives on novel therapeutic strategies for right heart failure in pulmonary arterial hypertension: lessons from the left heart Eur. Respir. Rev., March 1, 2010; 19(115): 72 - 82. [Abstract] [Full Text] [PDF]