Pulmonary arterial hypertension in congenital heart disease

Cardiology Clinics

Volumn 20, Issue 3, 2002, Pages 441-457

  Granton, John T ^a   Rabinovitch, Marlene ^b  

^a UNIVERSITY HEALTH NETWORK   (Canada)

^b HOSPITAL FOR SICK CHILDREN   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

CONGENITAL HEART DISEASE; HEART SEPTUM DEFECT; HUMAN; PATHOGENESIS; PATHOPHYSIOLOGY; PRIORITY JOURNAL; PROGNOSIS; PULMONARY HYPERTENSION; REVIEW; RISK FACTOR;

EID: 0036696142     PISSN: 07338651     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0733-8651(02)00017-6     Document Type: Article

References (95)

1. Eisenmenger V. Die angeborenen Defects des Kammerscheidewand des Herzen. Z Klin Med 1897;32:1-28.
2. Vongpatanasin W, Brickner ME, Hillis LD, et al. The Eisenmenger syndrome in adults. Ann Intern Med 1998;128:745-55.
3. Wood P. The Eisenmenger syndrome or pulmonary hypertension with reverses central shunt. BMJ 1958;2:701-9.
4. Rich S. Primary pulmonary hypertension: executive summary from the world symposium - primary pulmonary hypertension. Available from the World Health Organization via the intranet (http://www.who.int/ncd/cvd/pph.html); 1998.
5. Young D. Mark H. Fate of the patient with the Eisenmenger syndrome. Am J Cardiol 1971;28:658-9.
6. Kidd L, Driscoll D, Gersony W, et al. Second natural history study of congenital heart defects. Results of treatment of patients with ventricular septal defects. Circulation 1993;87:138-51.
7. Steele P, Fuster V, Cohen M, et al. Isolated atrial septal defect with pulmonary vascular obstructive disease-long-term follow-up and prediction of outcome after surgical correction. Circulation 1987;76:1037-42.
8. Vogel M, Berger F, Kramer A, et al. Incidence of secondary pulmonary hypertension in adults with atrial septal or sinus venosus defects. Heart 1999;82:30-3.
9. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A nationally prospective study. Ann Intern Med 1987;107:216-23.
10. Hopkins W, Ochoa L, Richardson G, et al. Comparison of the hemodynamics and survival of adults with severe pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant 1996;15:100-5.
11. Cantor WJ, Harrison DA, Moussadji JS, et al. Determinants of survival and length of survival in adults with Eisenmenger syndrome. Am J Cardiol 1999;84:677-81.
12. Clarkson P, Frye R, Dushane J, et al. Prognosis of patients with ventricular septal defect and severe pulmonary vascular obstructive disease. Circulation 1968;38:129-35.
13. Hallidie-Smith K, Goodwin J. The Eisenmenger syndrome. Prog Cardiol 1974;3:211-25.
14. Saha A, Balakrishnan K, Jaiswal P, et al. Prognosis for patients with Eisenmenger syndrome of various aetiology. Int J Cardiol 1994;45:199-207.
15. Daliento L, Somerville J, Presbitero P, et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J 1998;19:1845-55.
16. Hopkins WE, Waggoner AD. Severe pulmonary hypertension without right ventricular failure: The unique hearts of patients with Eisenmenger syndrome. Am J Cardiol 2002;89:34-8.
17. Glanville A, Burke C, Theodore J, et al. Primary pulmonary hypertension. Length of survival in patients referred for heart-lung transplantation. Chest 1987;91:675-81.
18. Rozkovec A, Montanes P, Oakley C. Factors that influence the outcome of primary pulmonary hypertension. Br Heart J 1997;55:449-58.
19. Barst R. Role of atrial septostomy in the treatment of pulmonary vascular disease. Thorax 2000;55:95.
20. Sandoval J, Gaspar J, Pulido T, et al. Graded balloon dilation atrial septostomy in severe primary pulmonary hypertension: A therapeutic alternative for patients nonresponsive to vasodilator treatment. J Am Coll Cardiol 1998;32:297-304.
21. Berman W Jr, Wood W, Yabek S, et al. Systemic oxygen transport in patients with congenital heart disease. Circulation 1987;75:360-8.
22. Rabinovitch M. Pathobiology of pulmonary hypertension: impact on clinical management. Sem in Thorac CV Surg 2000;3:63-81.
23. Rabinovitch M, Haworth S, Castaneda A, et al. Lung biopsy in congenital heart disease: a morphometric approach to pulmonary vascular disease. Circulation 1978;58:1107-22.
24. Meyrick B, Reid L. Ultrastructural findings in lung biopsy material from children with congenital heart defects. Am J Pathol 1980;101:527-37.
25. Heath D, Edwards J. A description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation 1958;18:533-47.
26. Rabinovitch M, Keane J, Norwood W, et al. Vascular structure in lung tissue obtained at biopsy correlated with pulmonary hemodynamic findings after repair of congenital heart defects. Circulation 1984;69:655-67.
27. Rabinovitch M, Bothwell T, Hayakawa B, et al. Pulmonary artery endothelial abnormalities in patients with congenital heart defects and pulmonary hypertension: a correlation of light with scanning electron microscopy and transmission electron microscopy. Lab Invest 1986; 55:632-53.
28. Sakesla O, Rifkin D. Release of basic fibroblast growth factor-heparin sulfate complexes from endothelial cells by plasminogen activator-mediated proteolytic degradation. J Cell Biol 1990;107:743-52.
29. Taipale J, Lohi J, Saarinen J, et al. Human mast cell chymase and leukocyte elastase release latent transforming growth factor-B1 from the extracellular matrix of cultured human epithelial and endothelial cells. J Biol Chem 1995;270:4689-96.
30. Jones P, Cowan K, Rabinovitch M. Induction of tenascin and fibronectin are features associated with increased smooth muscle cell proliferation during the development of progressive pulmonary hypertension in children. Am J Pathol 1997;150:1349-60.
31. Jones P, Rabinovitch M. Tenascin-C is induced with progressive pulmonary vascular disease in rats is functionally related to increased smooth muscle cell proliferation. Circ Res 1996;79:1131-42.
32. Boudreau N, Turley E, Rabinovitch M. Fibronectin, hyaluronan binding protein contribute to increased ductus arteriosus smooth muscle cell migration. Dev Biol 1991;43:235-47.
33. Zhou B, Boudreau N, Coulber C, et al. Microtubule-associated protein 1 light chain 3 is a fibronectin mRNA-binding protein linked to mRNA translation in lamb vascular smooth muscle cells. J Clin Invest 1997;100:3070-82.
34. Cowan KN, Heilbut A, Humpl T, et al. Complete reversal of fatal pulmonary hypertension in rats by a serine elastase inhibitor. Nat Med 2000;6:698-702.
35. Geiger R, Berger RM, Hess J, et al. Enhanced expression of vascular endothelial growth factor in pulmonary plexogenic arteriopathy due to congenital heart disease. J Pathol 2000;191:202-7.
36. Tuder RM, Cool CD, Yeager M, et al. The pathobiology of pulmonary hypertension. Endothelium. Clin Chest Med 2001;22:405-18.
37. Adatia I, Haworth S. Circulating endothelin levels in children with congenital heart disease. Br Heart J 1993;69:233-6.
38. 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;327:70-5.
39. 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;328:1732-9.
40. Giaid A. Nitric oxide and endothelin-1 in pulmonary hypertension. Chest 1998;114:2085-125.
41. Giaid A, Saleh D. Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension. N Engl J Med 1995;333:214-21.
42. Davie N, Haleen SJ, Upton PD, et al. ET(A) and ET(B) receptors modulate the proliferation of human pulmonary artery smooth muscle cells. Am J Respir Crit Care Med 2002;165:398-405.
43. Stewart DJ, Levy RD, Cernacek P, et al. Increased plasma endothelin-1 in pulmonary hypertension: marker or mediator of disease? Ann Intern Med 1991;114:464-9.
44. Gries A, Bode C, Peter K, et al. Inhaled nitric oxide inhibits human platelet aggregation, P-selectin expression, and fibrinogen binding in vitro and in vivo. Circulation 1998;97:1481-7.
45. Kharitonov SA, Cailes JB, Black CM, et al. Decreased nitric oxide in the exhaled air of patients with systemic sclerosis with pulmonary hypertension. Thorax 1997;52:1051-5.
46. Rolla G, Colagrande P, Brussino L, et al. Exhaled nitric oxide and pulmonary response to iloprost in systemic sclerosis with pulmonary hypertension. [letter] [see comments]. Lancet 1998;351:1491-2.
47. Mason N, Springall D, Burke M, et al. High expression of endothelial nitric oxide synthase in plexiform lesions of pulmonary hypertension. J Pathol 1998;185:313-8.
48. Black SM, Fineman JR, Steinhorn RH, et al. Increased endothelial NOS in lambs with increased pulmonary blood flow and pulmonary hypertension. Am J Physiol 1998;275:H1643-51.
49. Berger RMF, Geiger R, Hess J, et al. Altered arterial expression patterns of inducible and endothelial nitric oxide synthase in pulmonary plexogenic arteriopathy caused by congenital heart disease. Am J Respir Crit Care Med 2001;163:1493-99.
50. Nadaud S, Philippe M, Arnal J, et al. Sustained increase in aortic endothelial nitric oxide synthase expression in vivo in a model of chronic high blood flow. Circ Res 1997;79:857-63.
51. Shaul P. Ontogeny of nitric oxide in the pulmonary vasculature. Sem in Perinatol 1997;21:381-92.
52. Dinh-Xuan AT, Higenbottam TW, Clelland CA, et al. Impairment of endothelium-dependent pulmonary-artery relaxation in chronic obstructive lung disease. N Engl J Med 1991;324:1539-47.
53. Michelakis ED, Weir EK. The pathobiology of pulmonary hypertension. Smooth muscle cells and ion channels. Clin Chest Med 2001;22:419-32.
54. 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;98:1400-6.
55. Golovina VA, Platoshyn O, Bailey CL, et al. Upregulated TRP and enhanced capacitative Ca(2+) entry in human pulmonary artery myocytes during proliferation. Am J Physiol Heart Circ Physiol 2001;280:H746-55.
56. Platoshyn O, Golovina VA, Bailey CL, et al. Sustained membrane depolarization and pulmonary artery smooth muscle cell proliferation. Am J Physiol Cell Physiol 2000;279:C1540-9.
57. Seiden JE, Platoshyn O, Bakst AE, et al. High K(+)-induced membrane depolarization attenuates endothelium-dependent pulmonary vasodilation. Am J Physiol Lung Cell Mol Physiol 2000;278:L261-7.
58. Limsuwan A, Platoshyn O, Yu Y, et al. Inhibition of K(+) channel activity in human pulmonary artery smooth muscle cells by serum from patients with pulmonary hypertension secondary to congenital heart disease. Pediatr Res 2001;50:23-8.
59. 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;101:927-34.
60. Tuder RM, Chacon M, Alger L, et al. Expression of angiogenesis-related molecules in plexiform lesions in severe pulmonary hypertension: evidence for a process of disordered angiogenesis. J Pathol 2001;195:367-74.
61. Yeager ME, Halley GR, Golpon HA, et al. Microsatellite instability of endothelial cell growth and apoptosis genes within plexiform lesions in primary pulmonary hypertension. Circ Res 2001;88:E2-E11.
62. Deng Z, Haghighi F, Helleby L, et al. Fine mapping of PPH1, a gene for familial primary pulmonary hypertension, to a 3-cM region on chromosome 2q33. Am J Respir Crit Care Med 2000;161:1055-9.
63. 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;67:737-44.
64. Lane K, Machado R, Pauciulo M, et al. Heterozygous germline mutations in BMPR2 encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. The international PPH consortium. Nat Genet 2000;26:81-4.
65. Thomson J, Machado R, Pauciulo M, et al. Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-beta family. J Med Genet 2000;37:741-5.
66. De Caestecker M, Meyrick B. Bone morphogenic proteins, genetics and the pathophysiology of primary pulmonary hypertension. Respir Res 2001;2:193-7.
67. Newman JH, Wheeler L, Lane KB, et al. Mutation in the gene for bone morphogenetic protein receptor II as a cause of primary pulmonary hypertension in a large kindred. N Engl J Med 2001;345:319-24.
68. Morrell 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-β1 and bone morphogenetic proteins. Circulation 2001;104:790-5.
69. Geraci MW, Gao BG, Hoshikawa Y, et al. Genomic approaches to research in pulmonary hypertension. Respir Res 2001;2:210-5.
70. Geraci MW, Moore M, Gesell T, et al. Gene expression patterns in the lungs of patients with primary pulmonary hypertension: a gene microarray analysis. Circ Res 2001;88:555-62.
71. Roberts DH, Lepore JJ, Maroo A, et al. Oxygen therapy improves cardiac index and pulmonary vascular resistance in patients with pulmonary hypertension. Chest 2001;120:1547-55.
72. Sandoval J, Alvarado P, Martinez-Guerra ML, et al. Effect of body position changes on pulmonary gas exchange in Eisenmenger's syndrome. Am J Respir Crit Care Med 1999;159:1070-3.
73. Bowyer J, Busst C, Denison D, et al. Effect of long term oxygen treatment at home in children with pulmonary vascular disease. BMJ 1986;55:385-90.
74. Sandoval J, Aguirre JS, Pulido T, et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med 2001;164:1682-7.
75. Higenbottam T, Wheeldon D, Wells F, et al. Treatment of pulmonary hypertension with the continuous intravenous epoprostenol (prostacyclin). Lancet 1984;1:1046-7.
76. Rubin L, Mendoza J, Hood M, et al. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med 1990;112:485-91.
77. Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996;334:296-301.
78. Badesch DB, Tapson V, McGoon M, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized controlled trial. Ann Intern Med 2000;132:489-98.
79. Clapp LH, Finney P, Turcato S, et al. Differential effects of stable prostacyclin analogs on smooth muscle proliferation and cyclic AMP generation in human pulmonary artery. Am J Respir Cell Mol Biol 2002;26:194-201.
80. Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999;99:1858-65.
81. McLaughlin VV, Genthner DE, Panella MM, et al. Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: as case series. Ann Intern Med 1999;130:740-3.
82. Nagaya N, Uematsu M, Okano Y, et al. Effect of orally active prostacylin analogue on survival of outpatients with primary pulmonary hypertension. J Am Call Cardiol 1999;34:1188-92.
83. Zwissler B, Rank N, Jaenicke U, et al. Selective pulmonary vasodilation by inhaled prostacyclin in a newborn with congenital heart disease and cardiopulmonary bypass. Anesthesiology 1995;82:1512-6.
84. Hoeper MM, Schwarze M, Ehlerding S, et al. Long-term treatment of primary pulmonary hypertension with aerosolized iloprost, a prostacyclin analogue. N Engl J Med 2000;342:1866-70.
85. The neonatal inhaled nitric oxide study group. Inhaled nitric oxide in full-term and nearly full-term infants with hypoxic respiratory failure. N Engl J Med 1997;336:597-604.
86. Homer K, Wanstall J. In vitro comparison of two NONOates (novel nitric oxide donors) on rat pulmonary arteries. Eur J Pharmacol 1998;356:49-57.
87. Jacobs BR, Brilli RJ, Ballard ET, et al. Aerosolized soluble nitric oxide donor improves oxygenation and pulmonary hypertension in acute lung injury. Am J Respir Crit Care Med 1998;158:1536-42.
88. Lopez-Lopez JG, Perez-Vizcaino F, Cogolludo AL, et al. Nitric oxide- and nitric oxide donors-induced relaxation and its modulation by oxidative stress in piglet pulmonary arteries. Br J Pharmacol 2001;133:615-24.
89. Channick RN, Simonneau G, Sitbon O, et al. Effects of the dual endothelin-receptor antagonist bosental in patients with pulmonary hypertension: a randomized placebo controlled study. Lancet 2001;358:1119-23.
90. Campbell AIM, Kuliszewski MA, Stewart DJ. Cell-based gene transfer to the pulmonary vasculature. Endothelial nitric oxide synthase overexpression inhibits monocrotaline-induced pulmonary hypertension. Am J Respir Cell Mol Biol 1999;21:567-75.
91. Campbell AIM, Zhao Y, Sandhu R, et al. Cell-based gene transfer of vascular endothelial growth factor attenuates monocrotaline-induced pulmonary hypertension. Circulation 2001;104:2242-8.
92. Hosenpud JD, Bennett LE, Keck BM, et al. The registry of the International Society for Heart and Lung Transplantation: eighteenth official report 2001. J Heart Lung Transplant 2001;20:805-15.
93. Waddell T, Bennett L, Kennedy R, et al. Heart-lung or lung transplantation for Eisenmenger syndrome. J Heart Lung Transpl 2002;21:731-7.
94. Pielstickler EJ, Martinez FJ, Rubenfire M. Lung and heart-lung transplant practice patterns in pulmonary hypertension centres. J Heart Lung Transplant 2001;20:1297-304.
95. Charman SC, Sharples LD, McNeil KD, et al. Assessment of survival benefit after lung transplantation by patient diagnosis. J Heart Lung Transplant 2002;21:226-32.