title: Basic science of pulmonary arterial hypertension for clinicians: new concepts and experimental therapies.
authors.affiliation: University of Chicago, IL 60637, USA. sarcher@medicine.bsd.uchicago.edu
authors.forename: Stephen L
authors.initials: SL
authors.lastname: Archer
authors.forename: E Kenneth
authors.initials: EK
authors.lastname: Weir
authors.forename: Martin R
authors.initials: MR
authors.lastname: Wilkins
mesh_terms: D000818:Animals; D002309:Cardiology; D006333:Heart Failure / Q000473:pathology / Q000503:physiopathology* / Q000628:therapy*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000473:pathology / Q000503:physiopathology* / Q000628:therapy*
references.citation: Am J Physiol Lung Cell Mol Physiol. 2007 Apr;292(4):L885-97
references.pmid: 17189321
references.citation: Circulation. 2009 May 5;119(17):2313-22
references.pmid: 19380626
references.citation: N Engl J Med. 1992 Jul 9;327(2):70-5
references.pmid: 1603138
references.citation: Eur Respir J. 2008 Jan;31(1):126-34
references.pmid: 17898019
references.citation: Am J Physiol Lung Cell Mol Physiol. 2007 Sep;293(3):L583-90
references.pmid: 17586694
references.citation: Circulation. 2008 Aug 12;118(7):722-30
references.pmid: 18663089
references.citation: Eur Respir J. 2007 Dec;30(6):1103-10
references.pmid: 17804449
references.citation: Curr Opin Cell Biol. 1999 Apr;11(2):211-8
references.pmid: 10209154
references.citation: Am J Physiol. 1994 Jan;266(1 Pt 2):H365-70
references.pmid: 8304521
references.citation: Am J Respir Crit Care Med. 2009 Jan 15;179(2):151-7
references.pmid: 18931333
references.citation: Proc Natl Acad Sci U S A. 2007 Jan 23;104(4):1342-7
references.pmid: 17227868
references.citation: Circ J. 2006 Feb;70(2):174-8
references.pmid: 16434811
references.citation: Am J Physiol Lung Cell Mol Physiol. 2001 Apr;280(4):L801-12
references.pmid: 11238022
references.citation: Circ Res. 2006 Feb 3;98(2):209-17
references.pmid: 16357305
references.citation: Am J Physiol Lung Cell Mol Physiol. 2004 Jan;286(1):L49-67
references.pmid: 14656699
references.citation: FASEB J. 2009 Jul;23(7):2299-306
references.pmid: 19244313
references.citation: Eur Respir J. 2007 Jul;30(1):104-9
references.pmid: 17360728
references.citation: Pulm Pharmacol Ther. 2006;19(5):370-4
references.pmid: 16483811
references.citation: Am J Respir Crit Care Med. 2003 May 15;167(10):1433-9
references.pmid: 12615632
references.citation: News Physiol Sci. 2004 Apr;19:44-50
references.pmid: 15016901
references.citation: ChemMedChem. 2009 May;4(5):853-65
references.pmid: 19263460
references.citation: Am J Physiol Lung Cell Mol Physiol. 2007 May;292(5):L1202-10
references.pmid: 17189322
references.citation: Am J Respir Crit Care Med. 1999 Nov;160(5 Pt 1):1500-6
references.pmid: 10556112
references.citation: Am J Respir Cell Mol Biol. 2006 Aug;35(2):182-9
references.pmid: 16543611
references.citation: N Engl J Med. 1993 Jun 17;328(24):1732-9
references.pmid: 8497283
references.citation: J Am Coll Cardiol. 1998 Jan;31(1):202-8
references.pmid: 9426041
references.citation: Science. 2002 May 31;296(5573):1646-7
references.pmid: 12040180
references.citation: Circulation. 2005 Jun 14;111(23):3105-11
references.pmid: 15939821
references.citation: Am J Respir Crit Care Med. 2007 Jun 15;175(12):1280-9
references.pmid: 17413127
references.citation: J Pharmacol Exp Ther. 2008 May;325(2):370-9
references.pmid: 18252813
references.citation: Circ Res. 2004 Apr 30;94(8):1109-14
references.pmid: 15031260
references.citation: J Appl Physiol (1985). 1993 Oct;75(4):1748-57
references.pmid: 7506706
references.citation: Cancer Cell. 2007 Jan;11(1):37-51
references.pmid: 17222789
references.citation: Proc Natl Acad Sci U S A. 2003 Aug 5;100(16):9488-93
references.pmid: 12878719
references.citation: Am J Physiol Lung Cell Mol Physiol. 2005 Apr;288(4):L727-33
references.pmid: 15608146
references.citation: Am J Physiol Heart Circ Physiol. 2001 Feb;280(2):H746-55
references.pmid: 11158974
references.citation: Circulation. 1994 May;89(5):2035-40
references.pmid: 7514109
references.citation: Circulation. 2009 Feb 3;119(4):566-76
references.pmid: 19153267
references.citation: J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl S):33S-39S
references.pmid: 15194176
references.citation: Physiol Res. 2000;49(5):539-48
references.pmid: 11191358
references.citation: Annu Rev Biochem. 2007;76:481-511
references.pmid: 17376027
references.citation: J Mol Med (Berl). 2010 Jan;88(1):47-60
references.pmid: 19949938
references.citation: Circulation. 2001 Aug 14;104(7):790-5
references.pmid: 11502704
references.citation: Am J Respir Crit Care Med. 2004 Mar 15;169(6):764-9
references.pmid: 14701708
references.citation: Am J Physiol Lung Cell Mol Physiol. 2008 Feb;294(2):L276-89
references.pmid: 18083765
references.citation: Am J Respir Crit Care Med. 2006 Sep 15;174(6):699-705
references.pmid: 16799071
references.citation: J Pathol. 2001 Oct;195(3):367-74
references.pmid: 11673836
references.citation: Respir Med. 2008 Jul;102(7):1073-9
references.pmid: 18394873
references.citation: Circulation. 2003 Oct 14;108(15):1839-44
references.pmid: 14530202
references.citation: Circulation. 2007 Mar 13;115(10):1260-8
references.pmid: 17309917
references.citation: N Engl J Med. 1996 Feb 1;334(5):296-301
references.pmid: 8532025
references.citation: J Heart Lung Transplant. 2001 Mar;20(3):381-4
references.pmid: 11257567
references.citation: Biochem Biophys Res Commun. 2006 Oct 20;349(2):781-8
references.pmid: 16956581
references.citation: Br J Pharmacol. 2002 Dec;137(8):1187-94
references.pmid: 12466227
references.citation: Am J Physiol Lung Cell Mol Physiol. 2007 Jan;292(1):L18-24
references.pmid: 16891395
references.citation: Am J Pathol. 2004 Jan;164(1):253-62
references.pmid: 14695338
references.citation: Hypertension. 2004 Nov;44(5):662-7
references.pmid: 15466668
references.citation: Circulation. 2004 Aug 10;110(6):718-23
references.pmid: 15289381
references.citation: BioDrugs. 2009;23(3):201-2
references.pmid: 19627172
references.citation: Circulation. 2005 Jul 19;112(3):423-31
references.pmid: 16027270
references.citation: Circ Res. 2006 May 26;98(10):1323-30
references.pmid: 16614302
references.citation: Circulation. 2007 Jul 17;116(3):238-48
references.pmid: 17606845
references.citation: Circulation. 2002 May 21;105(20):2398-403
references.pmid: 12021227
references.citation: Am J Physiol Lung Cell Mol Physiol. 2005 Nov;289(5):L798-806
references.pmid: 15964898
references.citation: Am J Respir Crit Care Med. 1994 Jun;149(6):1512-7
references.pmid: 8004307
references.citation: Circulation. 2009 Jan 27;119(3):374-81
references.pmid: 19139384
references.citation: N Engl J Med. 1995 Jul 27;333(4):214-21
references.pmid: 7540722
references.citation: Eur Respir J. 2004 Sep;24(3):371-4
references.pmid: 15358693
references.citation: Circulation. 2003 Apr 22;107(15):2037-44
references.pmid: 12695303
references.citation: Am J Respir Crit Care Med. 2006 May 1;173(9):1023-30
references.pmid: 16456139
references.citation: Circulation. 2007 Mar 13;115(10):1275-84
references.pmid: 17339547
references.citation: Physiol Genomics. 2008 Apr 22;33(2):278-91
references.pmid: 18303084
references.citation: J Lab Clin Med. 2002 Apr;139(4):194-201
references.pmid: 12024106
references.citation: Circulation. 1994 May;89(5):2041-4
references.pmid: 8181127
references.citation: Eur Respir J. 2005 Feb;25(2):244-9
references.pmid: 15684287
references.citation: J Am Coll Cardiol. 2009 Jun 30;54(1 Suppl):S43-S54
references.pmid: 19555858
references.citation: Am J Physiol Lung Cell Mol Physiol. 2003 Dec;285(6):L1233-45
references.pmid: 12909593
references.citation: Chest. 1998 Sep;114(3 Suppl):225S-230S
references.pmid: 9741573
references.citation: Chest. 2009 Jul;136(1):31-36
references.pmid: 19188551
references.citation: J Clin Invest. 2000 Jan;105(1):21-34
references.pmid: 10619858
references.citation: Lancet. 1998 Mar 7;351(9104):726-7
references.pmid: 9504523
references.citation: Am J Physiol Heart Circ Physiol. 2007 Jan;292(1):H140-8
references.pmid: 16920812
references.citation: Nat Genet. 2000 Sep;26(1):81-4
references.pmid: 10973254
references.citation: J Biol Chem. 1998 Aug 7;273(32):20156-61
references.pmid: 9685360
references.citation: Circ Res. 2001 Mar 30;88(6):555-62
references.pmid: 11282888
references.citation: Eur J Heart Fail. 2006 Nov;8(7):712-5
references.pmid: 16716659
references.citation: Am J Med. 1995 Sep;99(3):249-54
references.pmid: 7653484
references.citation: FASEB J. 2005 Jul;19(9):1175-7
references.pmid: 15827267
references.citation: Lancet. 2001 Oct 6;358(9288):1119-23
references.pmid: 11597664
references.citation: J Am Coll Cardiol. 2007 Apr 10;49(14):1566-71
references.pmid: 17418297
references.citation: Am J Physiol Lung Cell Mol Physiol. 2004 Apr;286(4):L668-78
references.pmid: 12754186
references.citation: Am J Pathol. 2008 Mar;172(3):615-27
references.pmid: 18258847
references.citation: Trends Cardiovasc Med. 2005 Apr;15(3):110-8
references.pmid: 16039971
references.citation: Am J Respir Crit Care Med. 2005 Jun 1;171(11):1292-7
references.pmid: 15750042
references.citation: Am J Physiol Lung Cell Mol Physiol. 2007 Apr;292(4):L872-8
references.pmid: 17142350
references.citation: Am J Hum Genet. 2000 Sep;67(3):737-44
references.pmid: 10903931
references.citation: Nat Med. 2007 Feb;13(2):198-203
references.pmid: 17273169
references.citation: J Biol Chem. 2001 Dec 14;276(50):47650-7
references.pmid: 11598137
references.citation: Eur Respir J. 2008 Jan;31(1):135-9
references.pmid: 18166594
references.citation: Circulation. 2007 Aug 21;116(8):936-43
references.pmid: 17679618
references.citation: Circulation. 1984 Oct;70(4):580-7
references.pmid: 6148159
references.citation: Eur Respir J. 2008 Oct;32(4):881-91
references.pmid: 18550612
references.citation: N Engl J Med. 2005 Sep 29;353(13):1412-3
references.pmid: 16192491
references.citation: Cardiovasc Res. 1996 Jan;31(1):55-62
references.pmid: 8849589
references.citation: Circulation. 2006 Jun 6;113(22):2630-41
references.pmid: 16735674
references.citation: Am J Physiol Heart Circ Physiol. 2008 Feb;294(2):H570-8
references.pmid: 18083891
references.citation: J Clin Invest. 2005 Oct;115(10):2811-21
references.pmid: 16200212
references.citation: J Crit Care. 2004 Mar;19(1):48-53
references.pmid: 15101006
references.citation: Circulation. 2006 Oct 24;114(17):1883-91
references.pmid: 17060398
references.citation: J Clin Invest. 2008 May;118(5):1846-57
references.pmid: 18382765
references.citation: Circulation. 2002 Jan 15;105(2):244-50
references.pmid: 11790708
references.citation: Am J Respir Crit Care Med. 2006 Apr 1;173(7):798-802
references.pmid: 16339917
references.citation: Circulation. 2007 May 1;115(17):2331-9
references.pmid: 17438150
references.citation: Am J Respir Crit Care Med. 2003 Feb 15;167(4):580-6
references.pmid: 12446266
references.citation: Circulation. 2008 Jun 3;117(22):2928-37
references.pmid: 18506000
references.citation: Mol Ther. 2002 Jul;6(1):119-26
references.pmid: 12095312
references.citation: Chest. 1998 Sep;114(3 Suppl):213S-224S
references.pmid: 9741572
references.citation: J Heart Lung Transplant. 2008 Dec;27(12):1326-32
references.pmid: 19059113
references.citation: Am J Physiol Lung Cell Mol Physiol. 2007 May;292(5):L1182-92
references.pmid: 17277049
references.citation: Circulation. 1987 Jul;76(1):135-41
references.pmid: 2954725
references.citation: Circ Res. 2007 Mar 30;100(6):923-9
references.pmid: 17332430
references.citation: Am J Pathol. 2009 Feb;174(2):380-9
references.pmid: 19116361
references.citation: Arterioscler Thromb Vasc Biol. 2004 Mar;24(3):413-20
references.pmid: 14656731
references.citation: Am J Physiol Heart Circ Physiol. 2004 Mar;286(3):H1185-92
references.pmid: 14630626
references.citation: Peptides. 2007 Apr;28(4):910-9
references.pmid: 17267073
references.citation: Circ Res. 2004 Oct 15;95(8):830-40
references.pmid: 15375007
references.citation: J Heart Lung Transplant. 1996 Jan;15(1 Pt 1):100-5
references.pmid: 8820089
references.citation: Ann Intern Med. 2002 Apr 2;136(7):515-22
references.pmid: 11926786
references.citation: Am J Physiol Lung Cell Mol Physiol. 2003 Sep;285(3):L740-54
references.pmid: 12740218
references.citation: Proc Natl Acad Sci U S A. 2004 Sep 21;101(38):13861-6
references.pmid: 15358862
references.citation: Eur Respir J. 2003 Aug;22(2):358-63
references.pmid: 12952274
references.citation: Circulation. 1998 Oct 6;98(14):1400-6
references.pmid: 9760294
references.citation: Clin Sci (Lond). 1991 Sep;81(3):379-85
references.pmid: 1655340
references.citation: Am J Physiol. 1991 Oct;261(4 Pt 2):H1255-67
references.pmid: 1928407
references.citation: FASEB J. 2009 Dec;23(12):4135-47
references.pmid: 19679640
references.citation: Circulation. 2005 Apr 26;111(16):2126-33
references.pmid: 15824200
references.citation: Br J Pharmacol. 2003 Jul;139(5):899-910
references.pmid: 12839863
references.citation: Circulation. 2006 Jan 17;113(2):286-95
references.pmid: 16391154
references.citation: J Appl Physiol (1985). 2002 Nov;93(5):1770-8
references.pmid: 12381765
references.citation: Am J Respir Crit Care Med. 2004 Aug 1;170(3):252-9
references.pmid: 15087293
references.citation: Respirology. 2008 Nov;13(6):825-31
references.pmid: 18811881
references.citation: Am J Respir Crit Care Med. 2006 Aug 15;174(4):437-45
references.pmid: 16728715
references.citation: Circ Res. 2009 May 22;104(10):1184-91
references.pmid: 19390056
references.citation: Circulation. 2003 Aug 19;108(7):889-95
references.pmid: 12835224
references.citation: Circ Res. 2008 May 23;102(10):1212-21
references.pmid: 18436795
references.citation: Am J Physiol. 1984 Oct;247(4 Pt 2):H682-5
references.pmid: 6437245
references.citation: Circ Res. 2005 Mar 4;96(4):442-50
references.pmid: 15692087
references.citation: Eur Heart J. 2008 Jan;29(1):120-7
references.pmid: 18065750
references.citation: Respiration. 2008;75(3):272-80
references.pmid: 18025812
references.citation: J Am Coll Cardiol. 2006 May 16;47(10):2049-56
references.pmid: 16697324
references.citation: Ann Intern Med. 1991 Mar 15;114(6):464-9
references.pmid: 1994793
references.citation: J Clin Invest. 2005 Jun;115(6):1479-91
references.pmid: 15931388
references.citation: J Pharmacol Exp Ther. 2000 Feb;292(2):512-20
references.pmid: 10640287
references.citation: Ann Intern Med. 1990 Apr 1;112(7):485-91
references.pmid: 2107780
references.citation: N Engl J Med. 1992 Jul 9;327(2):76-81
references.pmid: 1603139
references.citation: J Biol Chem. 2007 May 18;282(20):15081-9
references.pmid: 17403661
references.citation: N Engl J Med. 2009 Nov 5;361(19):1864-71
references.pmid: 19890129
references.citation: Arterioscler Thromb Vasc Biol. 2007 Sep;27(9):1960-7
references.pmid: 17569879
references.citation: FASEB J. 2005 Jul;19(9):1178-80
references.pmid: 15897232
references.citation: J Appl Physiol (1985). 2001 Jun;90(6):2249-56
references.pmid: 11356790
references.citation: Cardiovasc Res. 2007 Jul 15;75(2):339-48
references.pmid: 17544387
references.citation: Nat Med. 2002 Oct;8(10):1129-35
references.pmid: 12244304
references.citation: Circulation. 2008 Jun 10;117(23):3020-30
references.pmid: 18519847
references.citation: J Med Genet. 2000 Oct;37(10):741-5
references.pmid: 11015450
references.citation: Cell Metab. 2006 Mar;3(3):177-85
references.pmid: 16517405
references.citation: Am Rev Respir Dis. 1992 Apr;145(4 Pt 1):793-7
references.pmid: 1554204
references.citation: Ann Intern Med. 2008 Oct 21;149(8):521-30
references.pmid: 18936500
references.citation: Circulation. 2008 Oct 21;118(17):1758-66
references.pmid: 18936338
references.citation: Endocr Rev. 2006 Feb;27(1):47-72
references.pmid: 16291870
references.citation: Am J Physiol Cell Physiol. 2007 May;292(5):C1837-53
references.pmid: 17267549
references.citation: Circ Res. 1997 Jul;81(1):34-41
references.pmid: 9201025
references.citation: Pediatr Res. 2006 Jun;59(6):767-72
references.pmid: 16641207
references.citation: J Clin Invest. 2001 Oct;108(8):1141-50
references.pmid: 11602621
references.citation: Prog Nucleic Acid Res Mol Biol. 2001;70:33-75
references.pmid: 11642366
references.citation: J Physiol. 2006 Jul 1;574(Pt 1):113-23
references.pmid: 16709639
references.citation: Am J Respir Crit Care Med. 1998 Oct;158(4):1061-7
references.pmid: 9769261
references.citation: Microvasc Res. 2004 Sep;68(2):75-103
references.pmid: 15313118
references.citation: N Engl J Med. 2005 Nov 17;353(20):2148-57
references.pmid: 16291984
references.citation: Circulation. 2004 Jan 27;109(3):351-6
references.pmid: 14718403
references.citation: J Clin Invest. 2003 May;111(9):1339-46
references.pmid: 12727925
references.citation: Br J Pharmacol. 2008 Oct;155(4):606-16
references.pmid: 18695640
references.citation: Am J Physiol Heart Circ Physiol. 2003 Nov;285(5):H2125-31
references.pmid: 14561683
references.citation: Am J Respir Crit Care Med. 2008 Oct 15;178(8):861-9
references.pmid: 18689467
references.citation: Am J Physiol Lung Cell Mol Physiol. 2006 May;290(5):L841-8
references.pmid: 16339782
references.citation: Proc Natl Acad Sci U S A. 2007 Jul 3;104(27):11418-23
references.pmid: 17596340
references.citation: Pediatr Transplant. 2008 Sep;12(6):650-5
references.pmid: 18466198
references.citation: Hum Mol Genet. 2008 Oct 15;17(20):3180-90
references.pmid: 18647753
references.citation: Am J Respir Crit Care Med. 2002 Nov 15;166(10):1403-8
references.pmid: 12406854
references.citation: Circ Res. 2006 Mar 31;98(6):818-27
references.pmid: 16497988
references.citation: Nat Rev Drug Discov. 2006 Sep;5(9):755-68
references.pmid: 16955067
references.citation: Circulation. 2001 Sep 25;104(13):1557-63
references.pmid: 11571252
references.citation: Circulation. 2002 Jan 29;105(4):516-21
references.pmid: 11815437
references.citation: N Engl J Med. 2005 Nov 10;353(19):2042-55
references.pmid: 16282179
references.citation: Hypertension. 2007 Jan;49(1):232-6
references.pmid: 17130306
references.citation: N Engl J Med. 1996 Aug 29;335(9):609-16
references.pmid: 8692238
references.citation: Am J Respir Crit Care Med. 2006 Apr 1;173(7):793-7
references.pmid: 16399993