Abstract
M-mode echocardiograms obtained from 31 patients with types I and IV osteogenesis imperfecta (OI) were evaluated for increased aortic root dilatation and wall stiffness. Four patients were observed to have dilated aortic roots. The pressure-strain elastic moduli (Ep) of the majority of OI patients studied were significantly different from those of age-matched controls; the observed values of Ep were both greater and less than that of controls. At high strain in the circumferential direction, nine of 31 OI patients had aortic roots that were significantly stiffer than those of controls. Increased stiffness in the circumferential direction was associated with decreased aortic pumping efficiency. The increased stiffness observed in the circumferential direction is consistent with increased accumulation and crosslinking of collagen within the aortic wall and may reflect premature aging of OI patients.
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Acar, J., B. Breil, J. Lavabre, G. Chomette, J. Roland, J. Dewidle and S. Hospitel. Osteogenese imparfaite et insuffisance aortique. Une observation avec documenta anatomiques. Revue de la literature.Ann. Med. Interne 131:514–518, 1980.
Arndt, J.O., H.F. Stegall and H.J. Wicke. Mechanics of the aortain vivo. A radiographic approach.Circ. Res. 28:693–704, 1971.
Bader, H. Dependence of wall stress in the human thoracic aorta on age and pressure.Circ. Res. 20:354–361, 1967.
Bergel, D.H. The static elastic properties of the arterial wall.J. Physiology 156:445–457, 1961.
Birk, D.E. and F.H. Silver. Collagen fibrillogenesisin vitro: comparison of types I, II and III.Arch. Biochem. Biophys. 235:178–185, 1984.
Boucek, R.J., N.L. Noble, Z. Gunja-Smith, W.T. Bulter. The Marfan syndrome: a deficiency in chemically stable cross-links.N. Engl. J. Med. 305:988–991, 1981.
Browell J.N. and E.H. Drake. Aortic valve lesions associated with osteogenesis imperfecta.Henry Ford Hosp. Med. Bull. 14:245–247, 1966.
Fujii, K. and M.L. Tanzer. Osteogenesis imperfecta: biochemical studies of bone and collagen.Clin. Orthop. 124:271–277, 1977.
Gow, B.S. and M.G. Taylor. Measurement of viscoelastic properties of arteries in the living dog.Circ. Res. 23:111–122, 1968.
Greenfield, J.C., Jr. and D.J. Patel. Relation between pressure and diameter in the ascending aorta of man.Circ. Res. 10:778–781, 1962.
Haas, G. Relations between the structure of the aging aorta and the properties of the isolated aortic elastic tissue.Arch. Pathology 35:29–45, 1943.
Heckman, B.A. and I. Steinberg. Congenital heart disease (mitral regurgitation) in osteogenesis imperfecta.Am. J. Roentgenol. 103:601–607, 1968.
Henry, W.L., J.M. Gardin and J.H. Ware. Echocardiographic measurements in normal subjects from infancy to old age.Circulation 62:1055–1069, 1980.
Heppner, R.L., H.I. Babitt, J.W. Bianchine and J.R. Warbasse. Aortic regurgitation and aneurysm of sinus of Valsalva associated with osteogenesis imperfecta.Am. J. Cardiol. 31:654–657, 1973.
Hortop, J., P. Taipouras, J.A. Hanley, B.J. Maron and J.R. Shapiro. Cardiovascular involvement in osteogenesis imperfecta.Circulation, in press.
Kalath, S., P. Tsipouras and F.H. Silver. Non-invasive assessment of aortic mechanical properties,Annals Biomedical Engineering, 14:513–524, 1986.
Krafka, J. Jr. Comparative study of the histo-physics of the aorta.Am. J. Physiology 125:1–14, 1939.
Lai, W.C. Experimental investigation and analysis of aortic mechanics. M.S. thesis in mechanical and aerospace engineering. Rutgers University, New Brunswick, NJ, 1985.
McKusick, V.A.Heritable Disorders of Connective Tissue. St. Louis: Mosby, 1982.
Patel, D.J., J.S. Janicki and T.E. Carew. Static anisotropic elastic properties of the aorta in living dogs.Circ. Res. 25:765–775, 1969.
Pease, D.C. and W.J. Paule. Electron microscopy of elastic arteries; the thoracic aorta of the rat.J. Ultrastructure Res. 3:469–484, 1960.
Pyeritz, R.E. and L.S. Levin, Aortic root dilatation and valvular dysfunction in osteogenesis imperfecta. (abstr).Circulation 64, supp. IV-311, 1981.
Pyeritz, R.E. and V.A. McKusick. The Marfan syndrome: diagnosis and management.N. Engl. J. Med. 300:772–777, 1979.
Ramirez, F., F.O. Sangiorgi and P. Tsipouras. Human collagens: Biochemical molecular and genetic features in normal and diseased states. In:Human genes and diseases, edited by F. Blasi. New York: Wiley, 1985.
Sillence, D.O., A. Senn and D.M. Danks. Genetic heterogeneity in osteogenesis imperfecta,J. Med. Genetics 16:101–116, 1979.
Smith, R., M.J.O. Francis and G.R. Houghton.The Brittle Bone Syndrome. London: Butterworth, 1983.
Stein, D. and F.E. Kloster. Valvular heart disease in osteogenesis imperfecta.Am. Heart J. 94: 637–641, 1977.
Trelstad R.L. and F.H. Silver. Matrix polymerization. In:Cell Biology of the Extracellular Matrix, edited by E.D. Hay. New York: Plenum, 179–215, 1981.
White, N.J., C.G. Winearls and R. Smith. Cardiovascular abnormalities in osteogenesis imperfecta.Am. Heart J. 106:1416–1420, 1983.
Wolinsky, H. and S. Glagov. Structural basis for the static mechanical properties of the aortic media.Circ. Res. 14:400–413, 1964.
Wolinsky, H. and S. Glagov. A lamellar unit of aortic medial structure and function in mammals.Circ. Res. 20:99–111, 1967.
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Kalath, S., Tsipouras, P. & Silver, F.H. Increased aortic root stiffness associated with osteogenesis imperfecta. Ann Biomed Eng 15, 91–99 (1987). https://doi.org/10.1007/BF02364170
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DOI: https://doi.org/10.1007/BF02364170