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The Cellular Basis of the Pathophysiology and Treatment of Pulmonary Hypertension

  • Stuart Rich
Part of the NATO ASI Series book series (NSSA, volume 281)

Abstract

Pulmonary hypertension has traditionally been categorized into primary and secondary types. Although primary pulmonary hypertension (PPH) is felt to be relatively unique as a cardiopulmonary disease, its distinction from secondary forms of pulmonary hypertension often solely depends on whether or not an identifiable etiology can be ascertained. Histologically it can be impossible to distinguish primary from secondary forms of pulmonary hypertension (Pietra et al, 1989), suggesting that the distinction may be in the mechanism(s) that produce the pulmonary vascular disease with pulmonary hypertension as a common end point.

Keywords

Pulmonary Hypertension Congenital Heart Disease Pulmonary Blood Flow Primary Pulmonary Hypertension Secondary Form 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. Belardinelli, I., Under, J. and Berne, R.M., 1989, The cardiac effects of adenosine. Prog. Cardiovas. Dis. 32: 73.CrossRefGoogle Scholar
  2. Celemajer, D.S., Cullen, S. and Deanfield, J.E., 1993, Impairment of endothelium-dependent pulmonary artery relaxation in children with congenital heart disease and abnormal pulmonary hemodynamics. Circulation 87: 440.Google Scholar
  3. Christman, B.W., McPherson, C.D., Newman, J.H., King, J.A., Bernard, G.R., Groves, B.M., and Loyd, J.E., 1992, An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. N. Engl. J. Med. 327: 70.PubMedCrossRefGoogle Scholar
  4. Cohen, M.D., Edwards, W.D., and Fuster, V., 1986, Regression in thromboembolic type of primary pulmonary hypertension during 2–1/2 years of antithrombotic therapy. J. Am. Coll. Cardiol. 7: 172.PubMedCrossRefGoogle Scholar
  5. Cremona, G., Higenbottam, T.W., and Scott, J.P., 1991, Continuous intravenous infusion of prostacyclin (PGI2) improves survival in primary pulmonary hypertension [abstract]. Am. Rev. Respir. Dis. 143: A180.Google Scholar
  6. Eisenburg, P.R., Lucore, C., Kaufinann, L., Sobel, B.E., Jafife, A.S. and Rich, S., 1990, Fibrinopeptide A levels indicative of pulmonary vascular thrombosis in patients with primary pulmonary hypertension. Circulation 82: 841.CrossRefGoogle Scholar
  7. Fuster, V., Steele, P.M., Edwards, W.D., Gersh, B.J., McGooon, M.D. and Frye, R.L., 1984, Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 70: 580.PubMedCrossRefGoogle Scholar
  8. Gomez-Sanchez, M.A., Mestre, de Juan, M., Gomez-Pajuelo, C. et al, 1989, Pulmonary hypertension due to toxic oil syndrome. Chest. 95: 325.Google Scholar
  9. Gurtner, H.P., 1985, Aminorex and pulmonary hypertension. Cor. Vasa. 27: 160.PubMedGoogle Scholar
  10. Hoflman, J.I.E., Rudolf, A.M. and Heymann, M.A., 1981, Pulmonar vascular disease with congenital heart lesions: Pathologic features and causes. Circulation 64: 873.CrossRefGoogle Scholar
  11. Houston, D.S. and Vanhoutte, P.M., 1988, Comparison of serotonergic receptor subtypes on the smooth muscle and endothelin of the canine coronary artery. J. Pharmacol Exp. Ther. 244: 1.PubMedGoogle Scholar
  12. Loyd, J.E., Atkinson, J.B., Pietra, G.G., Virmani, R. and Newman, J.H., 1988, Heterogeneity of pathologic lesions in familial primary pulmonary hypertension. Am. Rev. Respir. Dis. 138: 952.PubMedCrossRefGoogle Scholar
  13. Loyd, J.E., Primm, R.K. and Newmann, J.H., 1984, Familial primary pulmonary hypertension: clinical patterns. Am. Rev. Respir. Dis. 129: 194.PubMedGoogle Scholar
  14. Luscher, T.F., 1992, Endothelin: Systemic arterial and pulmonary effects of a new peptide with potent biologic properties. Am. Rev. Respir. Dis. 146: 556.Google Scholar
  15. McDonnell, P. J., Toye, P. A. and Hutchins, G.M., 1983, Primary pulmonary hypertension and cirrhosis: Are they related? Am. Rev. REspir. Dis. 127: 437.PubMedGoogle Scholar
  16. Meyrick, B. and Reid, L., 1979, Development of pulmonary arterial changes in rats fed Crotalaria Spectabilis. Am. J. Pathol 94: 37.PubMedGoogle Scholar
  17. Miller, V.M., Aahus, L.L. and Vanhoutte, P.M., 1986, Modulation of endothelium-dependent responses by chronic alterations of blood flow. Am. J. Physiol 251: H520.PubMedGoogle Scholar
  18. Nootens, M., Kaufinann, E. and Rich, S., 1993, Short-term effectiveness of nifedipine in secondary pulmonary hypertension. Am. J. Cardiol 72: 1475.CrossRefGoogle Scholar
  19. Palevsky, H.I., Schloo, B.L., Pietra, G.G., Weber, K.T., Janicki, J.S., Rubin, E., et al, 1989, Primary pulmonary hypertension: vascular structure, morphometry and responsiveness to vasodilator agents. Circulation 80: 1207.PubMedCrossRefGoogle Scholar
  20. Pepke-Zaba, J., Higgenbottam, W., Tuan Ding-Xuan, A., Strong, D. and Wallwork, J., 1991, Inhaled nitric oxide as a cause of selective pulmonary vasodilation in pulmonary hypertension. Lancet 338: 447.Google Scholar
  21. Pietra, G.G., Edwards, W.D. and Kay, J.M., et al, 1989, Histopathology of pulmonary hypertension: a qualitative and quantitative study of pulmonary blood vessels from 58 patients in National Heart, Lung, and Blood Institute, Primary Pulmonary Hypertension Registry. Circulation 80: 1198.PubMedCrossRefGoogle Scholar
  22. Pietra, G.G., and Ruttner, J.R., 1987, Specificity of pulmonary vascular lesions in primary pulmonary hypertension. Respiration 52: 81.PubMedCrossRefGoogle Scholar
  23. Polos, P.G., Wolfe, D., Harley, R.A., Strange, C. and Sahn, S.A., 1992, Pulmonary hypertension and immunodeficiency virus infection. Chest 101: 474.PubMedCrossRefGoogle Scholar
  24. Rabinovitch, M., Andrew, M. and Thom, H., et al, 1987, Abnormal endothelial factor VIII associated pulmonary hypertension and congenital heart defects. Circulation 76: 1043.PubMedCrossRefGoogle Scholar
  25. Rabinovitch, M., Bothwell, T. and Hayakawa, B.N., et al, 1986, Pulmonary artery endothelial abnormalities in patients with congenital heart defects and pulmonary hypertension. Lab. Invest. 55: 632.PubMedGoogle Scholar
  26. Reeves, J.T., Groves, B.M. and Turkevich, D., 1986, The case for treatment of selected patients with primary pulmonary hypertension. Am. Rev. Respir. Dis. 134: 342.PubMedGoogle Scholar
  27. Rich, S., Kaufinann, E. and Levy, P.S., 1992, The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N. Engl J. Med. 327: 76.PubMedCrossRefGoogle Scholar
  28. Roberts, J.D., Lang, P., Bigatello, L.M., Vlahakes, G.J. and Zapol, W.M., 1993, Inhaled nitric oxide in congenital heart disease. Circulation 87: 447.PubMedGoogle Scholar
  29. Rubin, L.J., Mendoza, J., Hood, M., McGoon, M., Barst, R. and Williams, W.B., et al, 1990, Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Ann. Intern. Med. 112: 485.PubMedGoogle Scholar
  30. Ryan, U.S., 1986, The endothelial surface and responses to injury. Fed. Proc. 45: 101.PubMedGoogle Scholar
  31. Samet, P. and Bernstein, W.H., 1963, Loss of reactivity of the pulmonary vascular bed in primary pulmonary hypertension. Am. Heart J. 66: 197.PubMedCrossRefGoogle Scholar
  32. Schrader, B.J., Inbar, S., Kaufinann, L., Vestal, R.E. and Rich, S., 1992, Comparison of the effects of adenosine and nifedipine in pulmonary hypertension. J. Am. Coll Cardiol 19: 1060.PubMedCrossRefGoogle Scholar
  33. Stelzner, T.J., O’Brien, R.F., Yanagisawa, M., Sakurai, T., Sato, K., Webb, S., Zamora, M., McMurtry, I.F. and Fisher, J.H., 1992, Increased lung endothelin-1 production in rats with idiopathic pulmonary hypertension. Am. J. Physiol L614.Google Scholar
  34. Stewart, D.J., Levy, R.D., Cernacek, P. and Langleben, D., 1991, Increased plasma endothelin-1 in pulmonary hypertension: Marker or mediator of disease. Ann. Intern. Med. 114: 464.PubMedGoogle Scholar
  35. Tracy, P.B., 1991, The role of endothelium in the expression of procoagulant activity. Coronary Artery Disease 2: 146.CrossRefGoogle Scholar
  36. Ungerer, R.G., Tashkin, D.P., Furst, D., et al, 1983, Prevalence and clinical correlates of pulmonary arterial hypertension in progressive systemic sclerosis. Am. J. Med. 75: 65.PubMedCrossRefGoogle Scholar
  37. Wood, P., 1958 Pulmonary hypertension with special reference to the vasoconstrictive factor. Br. Heart J. 20: 557.PubMedCrossRefGoogle Scholar
  38. Yamaki, S., Horiuchi, T., Miura, M., et al, 1986, Pulmonary vascular disease in secundum atrial septal defect with pulmonary hypertension. Chest. 89: 694.PubMedCrossRefGoogle Scholar

Copyright information

© Plenum Press, New York 1996

Authors and Affiliations

  • Stuart Rich
    • 1
  1. 1.University of Illinois at Chicago Section of Cardiology (M/C 787)ChicagoUSA

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