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J Am Coll Cardiol, 2004; 43:13-24
© 2004 by the American College of Cardiology Foundation
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Cellular and molecular pathobiology of pulmonary arterial hypertension

Marc Humbert, MD, PhD*,*, Nicholas W. Morrell, MD{dagger}, Stephen L. Archer, MD{ddagger}, Kurt R. Stenmark, MD§, Margaret R. MacLean, Bsc, PhD||, Irene M. Lang, MD, Brian W. Christman, MD, E. Kenneth Weir, MD**, Oliver Eickelberg, MD{dagger}{dagger}, Norbert F. Voelkel, MD{ddagger}{ddagger} and Marlene Rabinovitch, MD§§

* Service de Pneumologie et Réanimation Respiratoire, Centre des Maladies Vasculaires Pulmonaires, UPRES EA2705, Hôpital Antoine-Béclère, Université Paris-Sud, Clamart, France
{dagger} Respiratory Medicine Unit, Department of Medicine, University of Cambridge School of Clinical Medicine, Addenbrooke's Hospital, Cambridge, United Kingdom
{ddagger} Vascular Biology Group and Pulmonary Hypertension Program, Department of Medicine (Cardiology), University of Alberta, Edmonton, Alberta, Canada
§ Development Lung Biology Research, University of Colorado Health Sciences Center, Denver, Colorado, USA
|| Division of Biomedical and Life Sciences, Institute of Biomedical and Life Sciences, Glasgow University, Glasgow, United Kingdom
Department of Cardiology, University of Vienna, Vienna, Austria
Center for Lung Research, Department of Pharmacology, Vanderbilt University School of Medicine, Nashville, Tennessee, USA
** Department of Medicine, Veterans Affairs Medical Center, Minneapolis, Minnesota, USA
{dagger}{dagger} Department of Internal Medicine, Justus-Liebig University, Giessen, Germany
{ddagger}{ddagger} Pulmonary Hypertension Center, University of Colorado Health Sciences Center, Denver, Colorado, USA
§§ Department of Pediatrics, Stanford University School of Medicine, Stanford, California, USA

Manuscript received January 7, 2004; accepted February 3, 2004.

* Reprint requests and correspondence: Dr. Marc Humbert, Service de Pneumologie, Hôpital Antoine-Béclère, 157, Rue de la Porte de Trivaux, 92140 Clamart, France.
humbert{at}ipsc.u-psud.fr

Pulmonary arterial hypertension (PAH) has a multifactorial pathobiology. Vasoconstriction, remodeling of the pulmonary vessel wall, and thrombosis contribute to increased pulmonary vascular resistance in PAH. The process of pulmonary vascular remodeling involves all layers of the vessel wall and is complicated by cellular heterogeneity within each compartment of the pulmonary arterial wall. Indeed, each cell type (endothelial, smooth muscle, and fibroblast), as well as inflammatory cells and platelets, may play a significant role in PAH. Pulmonary vasoconstriction is believed to be an early component of the pulmonary hypertensive process. Excessive vasoconstriction has been related to abnormal function or expression of potassium channels and to endothelial dysfunction. Endothelial dysfunction leads to chronically impaired production of vasodilators such as nitric oxide and prostacyclin along with overexpression of vasoconstrictors such as endothelin (ET)-1. Many of these abnormalities not only elevate vascular tone and promote vascular remodeling but also represent logical pharmacological targets. Recent genetic and pathophysiologic studies have emphasized the relevance of several mediators in this condition, including prostacyclin, nitric oxide, ET-1, angiopoietin-1, serotonin, cytokines, chemokines, and members of the transforming-growth-factor-beta superfamily. 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.

Abbreviations and Acronyms
  BMP = bone morphogenetic proteins
  ET = endothelin
  5-HT = 5-hydroxytryptamine
  NO = nitric oxide
  PAH = pulmonary arterial hypertension
  TGF-ß = transforming growth factor-beta
  TGF-ßR2 = transforming growth factor-beta type-2 receptor
  VEGF = vascular endothelial growth factor
  VIP = vasoactive intestinal peptide




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Procoagulant Membrane Microparticles Correlate with the Severity of Pulmonary Arterial Hypertension
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[Abstract] [Full Text] [PDF]

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 HeartHome page
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]

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 Am. J. Physiol. Heart Circ. Physiol.Home page
C. Morin, C. Guibert, M. Sirois, V. Echave, M. M. Gomes, and E. Rousseau
Effects of {omega}-hydroxylase product on distal human pulmonary arteries
Am J Physiol Heart Circ Physiol, March 1, 2008; 294(3): H1435 - H1443.
[Abstract] [Full Text] [PDF]

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 ThoraxHome page
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.
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 Circ. Res.Home page
N. El-Bizri, L. Wang, S. L. Merklinger, C. Guignabert, T. Desai, T. Urashima, A. Y. Sheikh, R. H. Knutsen, R. P. Mecham, Y. Mishina, et al.
Smooth Muscle Protein 22{alpha}-Mediated Patchy Deletion of Bmpr1a Impairs Cardiac Contractility but Protects Against Pulmonary Vascular Remodeling
Circ. Res., February 15, 2008; 102(3): 380 - 388.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
K. Otterdal, A. K. Andreassen, A. Yndestad, E. Oie, W. J. Sandberg, C. P. Dahl, T. M. Pedersen, T. Ueland, L. Gullestad, F. R. Brosstad, et al.
Raised LIGHT Levels in Pulmonary Arterial Hypertension: Potential Role in Thrombus Formation
Am. J. Respir. Crit. Care Med., January 15, 2008; 177(2): 202 - 207.
[Abstract] [Full Text] [PDF]

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 Cardiovasc ResHome page
V. Leblais, E. Delannoy, F. Fresquet, H. Begueret, N. Bellance, S. Banquet, C. Allieres, L. Leroux, C. Desgranges, A. Gadeau, et al.
{beta}-adrenergic relaxation in pulmonary arteries: preservation of the endothelial nitric oxide-dependent {beta}2 component in pulmonary hypertension
Cardiovasc Res, January 1, 2008; 77(1): 202 - 210.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
O. Sanchez, E. Marcos, F. Perros, E. Fadel, L. Tu, M. Humbert, P. Dartevelle, G. Simonneau, S. Adnot, and S. Eddahibi
Role of Endothelium-derived CC Chemokine Ligand 2 in Idiopathic Pulmonary Arterial Hypertension
Am. J. Respir. Crit. Care Med., November 15, 2007; 176(10): 1041 - 1047.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Cell Mol. Bio.Home page
C. M. Carlin, A. J. Peacock, and D. J. Welsh
Fluvastatin Inhibits Hypoxic Proliferation and p38 MAPK Activity in Pulmonary Artery Fibroblasts
Am. J. Respir. Cell Mol. Biol., October 1, 2007; 37(4): 447 - 456.
[Abstract] [Full Text] [PDF]

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 Am. J. Physiol. Lung Cell. Mol. Physiol.Home page
M. S. McMurtry, S. Bonnet, E. D. Michelakis, S. Bonnet, A. Haromy, and S. L. Archer
Statin therapy, alone or with rapamycin, does not reverse monocrotaline pulmonary arterial hypertension: the rapamcyin-atorvastatin-simvastatin study
Am J Physiol Lung Cell Mol Physiol, October 1, 2007; 293(4): L933 - L940.
[Abstract] [Full Text] [PDF]

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 Am. J. Physiol. Heart Circ. Physiol.Home page
I. R. Henkens, K. T. B. Mouchaers, H. W. Vliegen, W. J. van der Laarse, C. A. Swenne, A. C. Maan, H. H. M. Draisma, I. Schalij, E. E. van der Wall, M. J. Schalij, et al.
Early changes in rat hearts with developing pulmonary arterial hypertension can be detected with three-dimensional electrocardiography
Am J Physiol Heart Circ Physiol, August 1, 2007; 293(2): H1300 - H1307.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Cell Mol. Bio.Home page
P. B. Sehgal and S. Mukhopadhyay
Dysfunctional Intracellular Trafficking in the Pathobiology of Pulmonary Arterial Hypertension
Am. J. Respir. Cell Mol. Biol., July 1, 2007; 37(1): 31 - 37.
[Abstract] [Full Text] [PDF]

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E. Arciniegas, M. G. Frid, I. S. Douglas, and K. R. Stenmark
Perspectives on endothelial-to-mesenchymal transition: potential contribution to vascular remodeling in chronic pulmonary hypertension
Am J Physiol Lung Cell Mol Physiol, July 1, 2007; 293(1): L1 - L8.
[Abstract] [Full Text] [PDF]

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 Am. J. Physiol. Heart Circ. Physiol.Home page
P. B. Sehgal and S. Mukhopadhyay
Pulmonary arterial hypertension: a disease of tethers, SNAREs and SNAPs?
Am J Physiol Heart Circ Physiol, July 1, 2007; 293(1): H77 - H85.
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L. Taraseviciene-Stewart, M. R. Nicolls, D. Kraskauskas, R. Scerbavicius, N. Burns, C. Cool, K. Wood, J. E. Parr, S. A. Boackle, and N. F. Voelkel
Absence of T Cells Confers Increased Pulmonary Arterial Hypertension and Vascular Remodeling
Am. J. Respir. Crit. Care Med., June 15, 2007; 175(12): 1280 - 1289.
[Abstract] [Full Text] [PDF]

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 Eur Respir JHome page
F. Perros, P. Dorfmuller, R. Souza, I. Durand-Gasselin, V. Godot, F. Capel, S. Adnot, S. Eddahibi, M. Mazmanian, E. Fadel, et al.
Fractalkine-induced smooth muscle cell proliferation in pulmonary hypertension
Eur. Respir. J., May 1, 2007; 29(5): 937 - 943.
[Abstract] [Full Text] [PDF]

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 Arterioscler. Thromb. Vasc. Bio.Home page
R. E. Morty, B. Nejman, G. Kwapiszewska, M. Hecker, A. Zakrzewicz, F. M. Kouri, D. M. Peters, R. Dumitrascu, W. Seeger, P. Knaus, et al.
Dysregulated Bone Morphogenetic Protein Signaling in Monocrotaline-Induced Pulmonary Arterial Hypertension
Arterioscler Thromb Vasc Biol, May 1, 2007; 27(5): 1072 - 1078.
[Abstract] [Full Text] [PDF]

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D. Merkus, B. Houweling, V. J. de Beer, Z. Everon, and D. J. Duncker
Alterations in endothelial control of the pulmonary circulation in exercising swine with secondary pulmonary hypertension after myocardial infarction
J. Physiol., May 1, 2007; 580(3): 907 - 923.
[Abstract] [Full Text] [PDF]

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 Am. J. Physiol. Heart Circ. Physiol.Home page
J. E. Faber, C. L. Szymeczek, S. Cotecchia, S. A. Thomas, A. Tanoue, G. Tsujimoto, and H. Zhang
{alpha}1-Adrenoceptor-dependent vascular hypertrophy and remodeling in murine hypoxic pulmonary hypertension
Am J Physiol Heart Circ Physiol, May 1, 2007; 292(5): H2316 - H2323.
[Abstract] [Full Text] [PDF]

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 CirculationHome page
R. T. Schermuly, S. S. Pullamsetti, G. Kwapiszewska, R. Dumitrascu, X. Tian, N. Weissmann, H. A. Ghofrani, C. Kaulen, T. Dunkern, C. Schudt, et al.
Phosphodiesterase 1 Upregulation in Pulmonary Arterial Hypertension: Target for Reverse-Remodeling Therapy
Circulation, May 1, 2007; 115(17): 2331 - 2339.
[Abstract] [Full Text] [PDF]

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 Am. J. Physiol. Lung Cell. Mol. Physiol.Home page
M. S. McMurtry, R. Moudgil, K. Hashimoto, S. Bonnet, E. D. Michelakis, and S. L. Archer
Overexpression of human bone morphogenetic protein receptor 2 does not ameliorate monocrotaline pulmonary arterial hypertension
Am J Physiol Lung Cell Mol Physiol, April 1, 2007; 292(4): L872 - L878.
[Abstract] [Full Text] [PDF]

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 Circ. Res.Home page
M. Oka, N. Homma, L. Taraseviciene-Stewart, K. G. Morris, D. Kraskauskas, N. Burns, N. F. Voelkel, and I. F. McMurtry
Rho Kinase-Mediated Vasoconstriction Is Important in Severe Occlusive Pulmonary Arterial Hypertension in Rats
Circ. Res., March 30, 2007; 100(6): 923 - 929.
[Abstract] [Full Text] [PDF]

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 Mayo Clin Proc.Home page
J. H. Ryu, M. J. Krowka, K. L. Swanson, P. A. Pellikka, and M. D. McGoon
Pulmonary Hypertension in Patients With Interstitial Lung Diseases
Mayo Clin. Proc., March 1, 2007; 82(3): 342 - 350.
[Abstract] [Full Text] [PDF]

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 CirculationHome page
G.-P. Diller and M. A. Gatzoulis
Pulmonary Vascular Disease in Adults With Congenital Heart Disease
Circulation, February 27, 2007; 115(8): 1039 - 1050.
[Abstract] [Full Text] [PDF]

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 J Am Coll CardiolHome page
M. Levy, C. Maurey, D. S. Celermajer, P. R. Vouhe, C. Danel, D. Bonnet, and D. Israel-Biet
Impaired Apoptosis of Pulmonary Endothelial Cells Is Associated With Intimal Proliferation and Irreversibility of Pulmonary Hypertension in Congenital Heart Disease
J. Am. Coll. Cardiol., February 20, 2007; 49(7): 803 - 810.
[Abstract] [Full Text] [PDF]

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 ChestHome page
D. Montani, R. Souza, C. Binkert, W. Fischli, G. Simonneau, M. Clozel, and M. Humbert
Endothelin-1/Endothelin-3 Ratio: A Potential Prognostic Factor of Pulmonary Arterial Hypertension
Chest, January 1, 2007; 131(1): 101 - 108.
[Abstract] [Full Text] [PDF]

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 Am. J. Physiol. Lung Cell. Mol. Physiol.Home page
J. L. Wright, H. Tai, R. Wang, X. Wang, and A. Churg
Cigarette smoke upregulates pulmonary vascular matrix metalloproteinases via TNF-{alpha} signaling
Am J Physiol Lung Cell Mol Physiol, January 1, 2007; 292(1): L125 - L133.
[Abstract] [Full Text] [PDF]

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 Am. J. Physiol. Lung Cell. Mol. Physiol.Home page
D. Case, D. Irwin, C. Ivester, J. Harral, K. Morris, M. Imamura, M. Roedersheimer, A. Patterson, M. Carr, M. Hagen, et al.
Mice deficient in galectin-1 exhibit attenuated physiological responses to chronic hypoxia-induced pulmonary hypertension
Am J Physiol Lung Cell Mol Physiol, January 1, 2007; 292(1): L154 - L164.
[Abstract] [Full Text] [PDF]

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 Am. J. Physiol. Lung Cell. Mol. Physiol.Home page
F. Murray, H. H. Patel, R. Y. S. Suda, S. Zhang, P. A. Thistlethwaite, J. X.-J. Yuan, and P. A. Insel
Expression and activity of cAMP phosphodiesterase isoforms in pulmonary artery smooth muscle cells from patients with pulmonary hypertension: role for PDE1
Am J Physiol Lung Cell Mol Physiol, January 1, 2007; 292(1): L294 - L303.
[Abstract] [Full Text] [PDF]

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 Arterioscler. Thromb. Vasc. Bio.Home page
P. C. Schulze, H. Liu, E. Choe, J. Yoshioka, A. Shalev, K. D. Bloch, and R. T. Lee
Nitric Oxide-Dependent Suppression of Thioredoxin-Interacting Protein Expression Enhances Thioredoxin Activity
Arterioscler Thromb Vasc Biol, December 1, 2006; 26(12): 2666 - 2672.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
L. Dewachter, S. Adnot, E. Fadel, M. Humbert, B. Maitre, A.-M. Barlier-Mur, G. Simonneau, M. Hamon, R. Naeije, and S. Eddahibi
Angiopoietin/Tie2 Pathway Influences Smooth Muscle Hyperplasia in Idiopathic Pulmonary Hypertension
Am. J. Respir. Crit. Care Med., November 1, 2006; 174(9): 1025 - 1033.
[Abstract] [Full Text] [PDF]

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 Am. J. Physiol. Heart Circ. Physiol.Home page
J. E. Faber, C. L. Szymeczek, S. S. Salvi, and H. Zhang
Enhanced {alpha}1-adrenergic trophic activity in pulmonary artery of hypoxic pulmonary hypertensive rats
Am J Physiol Heart Circ Physiol, November 1, 2006; 291(5): H2272 - H2281.
[Abstract] [Full Text] [PDF]

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 CirculationHome page
S. Mukhopadhyay, M. Sharma, S. Ramakrishnan, J. Yusuf, M. D. Gupta, N. Bhamri, V. Trehan, and S. Tyagi
Phosphodiesterase-5 Inhibitor in Eisenmenger Syndrome: A Preliminary Observational Study
Circulation, October 24, 2006; 114(17): 1807 - 1810.
[Abstract] [Full Text] [PDF]

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 Eur Respir JHome page
M. C. Tamby, M. Humbert, P. Guilpain, A. Servettaz, N. Dupin, J. J. Christner, G. Simonneau, J. Fermanian, B. Weill, L. Guillevin, et al.
Antibodies to fibroblasts in idiopathic and scleroderma-associated pulmonary hypertension
Eur. Respir. J., October 1, 2006; 28(4): 799 - 807.
[Abstract] [Full Text] [PDF]

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 Am. J. Physiol. Lung Cell. Mol. Physiol.Home page
K. Ihida-Stansbury, D. M. McKean, K. B. Lane, J. E. Loyd, L. A. Wheeler, N. W. Morrell, and P. L. Jones
Tenascin-C is induced by mutated BMP type II receptors in familial forms of pulmonary arterial hypertension
Am J Physiol Lung Cell Mol Physiol, October 1, 2006; 291(4): L694 - L702.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
N. Koulmann, V. Novel-Chate, A. Peinnequin, R. Chapot, B. Serrurier, N. Simler, H. Richard, R. Ventura-Clapier, and X. Bigard
Cyclosporin A Inhibits Hypoxia-induced Pulmonary Hypertension and Right Ventricle Hypertrophy
Am. J. Respir. Crit. Care Med., September 15, 2006; 174(6): 699 - 705.
[Abstract] [Full Text] [PDF]

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 JEMHome page
B. S. Zuckerbraun, B. Y. Chin, B. Wegiel, T. R. Billiar, E. Czsimadia, J. Rao, L. Shimoda, E. Ifedigbo, S. Kanno, and L. E. Otterbein
Carbon monoxide reverses established pulmonary hypertension
J. Exp. Med., September 4, 2006; 203(9): 2109 - 2119.
[Abstract] [Full Text] [PDF]

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 ChestHome page
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]

Home page
 Eur Respir JHome page
M. Witzenrath, B. Ahrens, S. M. Kube, A. C. Hocke, S. Rosseau, E. Hamelmann, N. Suttorp, and H. Schutte
Allergic lung inflammation induces pulmonary vascular hyperresponsiveness
Eur. Respir. J., August 1, 2006; 28(2): 370 - 377.
[Abstract] [Full Text] [PDF]


 
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