Pulmonary hypertension in childhood

In the presence of pulmonary hypertension, the pulmonary vasculature fails to remodel after birth. Secondary changes then develop, and do so more rapidly than in the adult lung because the cells are exposed to the insult whilst still relatively undifferentiated. The commonest causes of pulmonary hypertension in newborn and young infants are alveolar hypoxia, and congenital heart disease causing a left-to-right shunt and increased pulmonary blood flow. The initial response of an increase in pulmonary arterial muscularity is common to both, but intimal proliferation can develop rapidly in those with complex congenital heart disease. The structural abnormalities are accompanied by abnormalities in the control of pulmonary vascular reactivity, a problem which is the focus of much current research activity.

This article has been cited by other articles:

				B Wojciak-Stothard New drug targets for pulmonary hypertension: Rho GTPases in pulmonary vascular remodelling Postgrad. Med. J., July 1, 2008; 84(993): 348 - 353. [Abstract] [Full Text] [PDF]

				P. J. McNamara, P. Murthy, C. Kantores, L. Teixeira, D. Engelberts, T. van Vliet, B. P. Kavanagh, and R. P. Jankov Acute vasodilator effects of Rho-kinase inhibitors in neonatal rats with pulmonary hypertension unresponsive to nitric oxide Am J Physiol Lung Cell Mol Physiol, February 1, 2008; 294(2): L205 - L213. [Abstract] [Full Text] [PDF]

				M. Gorenflo Open lung biopsy in patients with congenital heart disease. Ann. Thorac. Surg., April 1, 2002; 73(4): 1357 - 1358. [Full Text] [PDF]

				R.M.F. Berger Possibilities and impossibilities in the evaluation of pulmonary vascular disease in congenital heart defects Eur. Heart J., January 1, 2000; 21(1): 17 - 27. [PDF]

				M. Das, E. C. Dempsey, D. Bouchey, M. E. Reyland, and K. R. Stenmark Chronic Hypoxia Induces Exaggerated Growth Responses in Pulmonary Artery Adventitial Fibroblasts . Potential Contribution of Specific Protein Kinase C Isozymes Am. J. Respir. Cell Mol. Biol., January 1, 2000; 22(1): 15 - 25. [Abstract] [Full Text]

				I. Schulze-Neick, D. J. Penny, M. L. Rigby, C. Morgan, A. Kelleher, P. Collins, J. Li, A. Bush, E. A. Shinebourne, and A. N. Redington L-Arginine and Substance P Reverse the Pulmonary Endothelial Dysfunction Caused by Congenital Heart Surgery Circulation, August 17, 1999; 100(7): 749 - 755. [Abstract] [Full Text] [PDF]

				A. M. Evans, O. N. Osipenko, S. G. Haworth, and A. M. Gurney Resting potentials and potassium currents during development of pulmonary artery smooth muscle cells Am J Physiol Heart Circ Physiol, September 1, 1998; 275(3): H887 - H899. [Abstract] [Full Text] [PDF]

				M. Das, K. R. Stenmark, L. J. Ruff, and E. C. Dempsey Selected isozymes of PKC contribute to augmented growth of fetal and neonatal bovine PA adventitial fibroblasts Am J Physiol Lung Cell Mol Physiol, December 1, 1997; 273(6): L1276 - L1284. [Abstract] [Full Text] [PDF]

				M. Das, D. M. Bouchey, M. J. Moore, D. C. Hopkins, R. A. Nemenoff, and K. R. Stenmark Hypoxia-induced Proliferative Response of Vascular Adventitial Fibroblasts Is Dependent on G Protein-mediated Activation of Mitogen-activated Protein Kinases J. Biol. Chem., May 4, 2001; 276(19): 15631 - 15640. [Abstract] [Full Text] [PDF]