Primary pulmonary hypertension: An overview of epidemiology and pathogenesis

Cleveland Clinic Journal of Medicine

Volumn 70, Issue SUPPL.1, 2003, Pages

  Ghamra, Ziad W ^a   Dweik, Raed A ^a  

^a LERNER RESEARCH INSTITUTE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ENDOTHELIAL CELL GROWTH FACTOR; ENDOTHELIN 1; NITRIC OXIDE; PROSTAGLANDIN;

FEMALE; GENETICS; HEALTH; HUMAN; MALE; METABOLISM; PATHOPHYSIOLOGY; PULMONARY HYPERTENSION; RETROSPECTIVE STUDY; REVIEW; SEX DIFFERENCE; THROMBOSIS; VASCULAR ENDOTHELIUM; VASCULAR SMOOTH MUSCLE;

ENDOTHELIAL GROWTH FACTORS; ENDOTHELIN-1; ENDOTHELIUM, VASCULAR; FEMALE; HUMANS; HYPERTENSION, PULMONARY; MALE; MUSCLE, SMOOTH, VASCULAR; NITRIC OXIDE; PROSTAGLANDINS; RETROSPECTIVE STUDIES; SEX FACTORS; THROMBOSIS; WORLD HEALTH;

EID: 2342667629     PISSN: 08911150     EISSN: None     Source Type: Journal    
DOI: 10.3949/ccjm.70.Suppl_1.S2     Document Type: Article

References (71)

1. Rich S, ed. Primary pulmonary hypertension: executive summary from the World Symposium on Primary Pulmonary Hypertension. Geneva: World Health Organization, 1998.
2. Pietra GG, Edwards WD, Kay M, et al. Histopathology of primary pulmonary hypertension. A qualitative and quantitative study of pulmonary blood vessels from 58 patients in the National Heart, Lung, and Blood Institute Primary Pulmonary Hypertension Registry. Circulation 1989; 80:1198-1206.
3. Pietra GG. Histopathology of primary pulmonary hypertension. Chest 1994; 105(2 suppl):2S-6S.
4. Loyd JE, Atkinson JB, Pietra GG, et al. Heterogeneity of pathologic lesions in familial primary pulmonary hypertension. Am Rev Respir Dis 1988; 138:952-957.
5. Bjornsson J, Edwards WD, Kay JM, et al. Primary pulmonary hypertension: a histopathologic study of 80 cases. Mayo Clin Proc 1985; 60:16-25.
6. Dresdale DT, Schultz M, Michtom RJ. Primary pulmonary hypertension: clinical and hemodynamic study. Am J Med 1951; 11:686-694.
7. Wood P. Pulmonary hypertension with special reference to the vasoconstrictive factor. Br Heart J 1959; 21:557-570.
8. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension: a national prospective study. Ann Intern Med 1987; 107:216-223.
9. Brenot F. Primary pulmonary hypertension. Case series from France. Chest 1994; 105(suppl):33S-36S.
10. Appelbaum L, Yigla M, Bendayan D, et al. Primary pulmonary hypertension in Israel: a national survey. Chest 2001; 119:1801-1806.
11. Kuriyama T. Epidemiology of primary pulmonary hypertension in Japan. Nippon Rinsho 2001; 59:1047-1052.
12. Loyd JE, Primm RK, Newman JH. Familial primary pulmonary hypertension: clinical patterns. Am Rev Respir Dis 1984; 129:194-197.
13. Loyd JE, Butler MG, Foroud TM, et al. Genetic anticipation and abnormal gender ratio at birth in familial primary pulmonary hypertension. Am J Respir Crit Care Med 1995; 152:93-97.
14. Nichols WC, Koller DL, Slovis B, et al. Localization of the gene for familial primary pulmonary hypertension to chromosome 2q31-32. Nat Genet 1997; 15:277-280.
15. Morse JH, Jones AC, Barst RJ, et al. Mapping of familial primary pulmonary hypertension locus (PPH1) to chromosome 2q31-32. Circulation 1997; 95:2603-2606.
16. Deng Z, Morse JH, Slager SL, et al. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet 2000; 67:737-744.
17. Lane KB, Machado RD, Pauciulo MW, et al. Heterozygous germline mutations in BMPR2, encoding a TGF-b receptor, cause familial primary pulmonary hypertension. The International PPH Consortium. Nat Genet 2000; 26:81-84.
18. Thomson JR, Machado RD, Pauciulo MW, et al. Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-b family. J Med Genet 2000; 37:741-745.
19. Gurtner HP. Pulmonary hypertension, "plexogenic pulmonary arteriopathy" and the appetite depressant drug aminorex: post or propter? Bull Physiopathol Res 1979; 15:897-923.
20. Rothman RB, Ayestas MA, Dersch CM, Baumann MH. Aminorex, fenfluramine, and chlorphentermine are serotonin transporter substrates. Implications for primary pulmonary hypertension. Circulation 1999; 100:869-875.
21. Eddahibi S, Humbert M, Fadel E, et al. Hyperplasia of pulmonary artery smooth muscle cells is causally related to overexpression of the serotonin transporter in primary pulmonary hypertension. Chest 2002; 121(3 suppl):97S-98S.
22. Mehta NJ, Khan IA, Mehta RN, Sepkowitz DA. HIV-related pulmonary hypertension: analytic review of 131 cases. Chest 2000; 118:1133-1141.
23. Ascheri G, Hohendl C, Schatz O, et al. Infection with human immunodeficiency virus-1 increases expression of vascular endothelial cell growth factor in T cells: implications for acquired immunodeficiency syndrome-associated vasculopathy. Blood 1999; 93:4232-4241.
24. Gurubhagavatula I, Palevsky HI. Pulmonary hypertension in systemic autoimmune disease. Rheum Dis Clin North Am 1997; 23:365-394.
25. Yanai-Landau H, Amital H, Bar-Dayan Y, et al. Autoimmune aspects of primary pulmonary hypertension. Pathobiology 1995; 63:71-75.
26. Barst RJ, Flaster ER, Menon A, et al. Evidence for the association of unexplained pulmonary hypertension in children with the major histocompatibility complex. Circulation 1992; 85:249-258.
27. Curnock AL, Dweik RA, Higgins BH, et al. High prevalence of hypothyroidism in patients with primary pulmonary hypertension. Am J Med Sci 1999; 318:289-292.
28. Dweik RA. Pulmonary hypertension and the search for the selective pulmonary vasodilator. Lancet 2002; 360:886.
29. Dweik RA. The promise and reality of nitric oxide in the diagnosisand treatment of lung disease. Cleve Clin J Med 2001; 68:486-493.
30. Kaneko FT, Arroliga AC, Dweik RA, et al. Biochemical reaction products of nitric oxide as quantitative markers of primary pulmonary hypertension. Am J Respir Crit Care Med 1998; 158:917-923.
31. Ozkan M, Dweik RA, Laskowski D, et al. High levels of nitric oxide in individuals with pulmonary hypertension receiving epoprostenol therapy. Lung 2001; 179:233-243.
32. Ozkan M, Dweik RA, Ahmad M. Drug-induced lung disease. Cleve Clin J Med 2001; 68:782-795.
33. Arroliga AC, Dweik RA, Kaneko FJ, Erzurum SC. Primary pulmonary hypertension: update on pathogenesis and novel therapies. Cleve Clin J Med 2000; 67:175-190.
34. Dweik RA, Erzurum SC. Effects of nitric oxide and cyclic GMP on smooth muscle proliferation. In: Moss J, ed. LAM and Other Diseases Characterized by Smooth Muscle Proliferation (Lung Biology in Health and Disease series). New York: Marcel Dekker, 1999; 131:333-349.
35. Dweik RA, Laskowski D, Abu-Soud HM, et al. Nitric oxide synthesis in the lung. Regulation by oxygen through a kinetic mechanism. J Clin Invest 1998; 101:660-666.
36. Moncada S, Higgs A. The L-arginine-nitric oxide pathway. N Engl J Med 1993; 329:2002-2012.
37. Nathan C, Xie QW. Nitric oxide synthases: roles, tolls, and controls. Cell 1994; 78:915-918.
38. Kobzik L, Bredt DS, Lowenstein CJ, et al. Nitric oxide synthase in human and rat lung: immunocytochemical and histochemical localization. Am J Respir Cell Mol Biol 1993; 9:371-377.
39. Gustafsson LE, Leone AM, Persson MG, et al. Endogenous nitric oxide is present in the exhaled air of rabbits, guinea pigs and humans. Biochem Biophys Res Commun 1991; 181:852-857.
40. Lundberg JO, Farkas-Szallasi T, Weitzberg E, et al. High nitric oxide production in human paranasal sinuses. Nat Med 1995; 1:370-373.
41. Channick RN, Newhart JW, Johnson FW, et al. Pulsed delivery of inhaled nitric oxide to patients with primary pulmonary hypertension: an ambulatory delivery system and initial clinical tests. Chest 1996; 109:1545-1549.
42. Rubin LJ. Primary pulmonary hypertension. N Engl J Med 1997; 336:111-117.
43. Yanagisawa M, Kurihara H, Kimura S, et al. A novel potent vasoconstrictor peptide produced by vascular endothelial cells. Nature 1988; 332:411-415.
44. Dupuis J, Duncan JS, Cernacek P, Gosselin G. Human pulmonary circulation is an important site for both clearance and production of endothelin-1. Circulation 1996; 94:1578-1584.
45. Giaid A, Yanagisawa M, Langleben D, et al. Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N Engl J Med 1993; 328:1732-1739.
46. Stewart DJ, Levy RD, Cernacek P, Langleben D. Increased plasma endothelin-1 in pulmonary hypertension: marker or mediator of disease? Ann Intern Med 1991; 114:464-469.
47. Moncada S, Vane JR. Pharmacology and endogenous roles of prostaglandin endoperoxides, thromboxane A2, and prostacyclin. Pharmacol Rev 1979; 30:293-331.
48. Gorman RR, Bunting S, Miller OV. Modulation of human platelet adenylate cyclase by prostacyclin. Prostaglandins 1977; 273:767-768.
49. Geraci MW, Gao B, Shepherd DC, et al. Pulmonary prostacyclin synthase overexpression in transgenic mice protects against development of hypoxic pulmonary hypertension. J Clin Invest 1999; 103:1509-1515.
50. Tuder RM, Cool CD, Geraci MW, et al. Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension. Am J Respir Crit Care Med 1999; 159:1925-1932.
51. Christman BW, McPherson CD, Newman JH, et al. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. N Engl J Med 1992; 327:70-75.
52. Tuder RM, Groves B, Badesh DB, Voelkel NF. Exuberant endothelial cell growth and elements of inflammation are present in plexiform lesions of pulmonary hypertension. Am J Pathol 1994; 144:275-285.
53. Lee SD, Shroyer KR, Markham NE, et al. Monoclonal endothelial cell proliferation is present in primary but not secondary pulmonary hypertension. J Clin Invest 1998; 101:927-934.
54. Tuder RM, Chacon M, Wang J, et al. Expression of angiogenesis- related molecules in plexiform lesions in severe pulmonary hypertension: evidence for a process of disordered angiogenesis. J Pathol 2001; 195:367-374.
55. Newby AC, Southgate KM, Assender JW. Inhibition of vascular smooth muscle cell proliferation by endothelium-dependent vasodilators. Herz 1992; 17:291-299.
56. Pukac LA, Ottlinger ME, Karnovsky MJ. Heparin suppresses specific second messenger pathways for protooncogene expression in rat vascular smooth muscle cells. J Biol Chem 1992; 267:3707-3711.
57. Hirata Y, Takagi Y, Fukunda Y, Marumo F. Endothelin is a potent mitogen for rat vascular smooth muscle cells. Atherosclerosis 1989; 78:225-228.
58. Morell NW, Yang X, Upton PD, et al. Altered growth responses of pulmonary artery smooth muscle cells from patients with primary pulmonary hypertension to transforming growth factor-b1 and bone morphogenetic proteins. Circulation 2001; 104:790-795.
59. Lee S, Wang W, Lanzillo J, Fanburg B. Serotonin produces both hyperplasia and hypertrophy of bovine pulmonary artery smooth muscle cells in culture. Am J Physiol 1994; 266(1 Pt 1):L46-L52.
60. Fanburg B, Sheu-Ling L. A new role for an old molecule: serotonin as a mitogen. Am J Physiol 1997; 272(1 Pt 1):L795-L806.
61. Herve P, Launay J-M, Scrobohaci M-L, et al. Increased plasma serotonin in primary pulmonary hypertension. Am J Med 1995; 99:249-254.
62. Herve P, Drouet L, Dosquet C, et al. Primary pulmonary hypertension in a patient with a familial platelet storage pool disease: role of serotonin. Am J Med 1990; 89:117-120.
63. Eddahibi S, Humbert M, Fadel E, et al. Serotonin transporter overexpression is responsible for pulmonary artery smooth muscle hyperplasia in primary pulmonary hypertension. J Clin Invest 2001; 108:1141-1150.
64. Yuan JX, Aldinger AM, Juhaszova M, et al. Dysfunctional voltage- gated K+ channels in pulmonary artery smooth muscle cells of patients with primary pulmonary hypertension. Circulation 1998; 98:1400-1406.
65. Yuan XJ, Wang J, Juhaszova M, et al. Attenuated K+ channel gene transcription in primary pulmonary hypertension. Lancet 1998; 351:726-727.
66. Rabinovitch M. Pathobiology of pulmonary arterial hypertension. Extracellular matrix. Clin Chest Med 2001; 22:433-449.
67. Fuster V, Steele PM, Edwards WD, et al. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984; 70:580-587.
68. Farber HW, Loscalzo J. Prothrombotic mechanisms in primary pulmonary hypertension. J Lab Clin Med 1999; 134:561-566.
69. Chaouat A, Weitzenblum E, Higenbottam T. The role of thrombosis in severe pulmonary hypertension. Eur Respir J 1996; 9:356-363.
70. Welsh CH, Hassell KL, Badesch DB, et al. Coagulation and fibrinolytic profiles in patients with severe pulmonary hypertension. Chest 1996; 110:710-717.
71. Lopes AA, Maeda NY, Almeida A, et al. Circulating platelet aggregates indicative of in vivo platelet activation in pulmonary hypertension. Angiology 1993; 44:701-706.