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Increased aortic root stiffness associated with osteogenesis imperfecta

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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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References

  1. 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.

    CAS  Google Scholar 

  2. Arndt, J.O., H.F. Stegall and H.J. Wicke. Mechanics of the aortain vivo. A radiographic approach.Circ. Res. 28:693–704, 1971.

    CAS  PubMed  Google Scholar 

  3. Bader, H. Dependence of wall stress in the human thoracic aorta on age and pressure.Circ. Res. 20:354–361, 1967.

    CAS  PubMed  Google Scholar 

  4. Bergel, D.H. The static elastic properties of the arterial wall.J. Physiology 156:445–457, 1961.

    Google Scholar 

  5. Birk, D.E. and F.H. Silver. Collagen fibrillogenesisin vitro: comparison of types I, II and III.Arch. Biochem. Biophys. 235:178–185, 1984.

    Article  CAS  PubMed  Google Scholar 

  6. 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.

    CAS  PubMed  Google Scholar 

  7. Browell J.N. and E.H. Drake. Aortic valve lesions associated with osteogenesis imperfecta.Henry Ford Hosp. Med. Bull. 14:245–247, 1966.

    Google Scholar 

  8. Fujii, K. and M.L. Tanzer. Osteogenesis imperfecta: biochemical studies of bone and collagen.Clin. Orthop. 124:271–277, 1977.

    CAS  PubMed  Google Scholar 

  9. Gow, B.S. and M.G. Taylor. Measurement of viscoelastic properties of arteries in the living dog.Circ. Res. 23:111–122, 1968.

    CAS  PubMed  Google Scholar 

  10. 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.

    PubMed  Google Scholar 

  11. 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.

    Google Scholar 

  12. Heckman, B.A. and I. Steinberg. Congenital heart disease (mitral regurgitation) in osteogenesis imperfecta.Am. J. Roentgenol. 103:601–607, 1968.

    CAS  Google Scholar 

  13. 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.

    Google Scholar 

  14. 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.

    Article  CAS  PubMed  Google Scholar 

  15. Hortop, J., P. Taipouras, J.A. Hanley, B.J. Maron and J.R. Shapiro. Cardiovascular involvement in osteogenesis imperfecta.Circulation, in press.

  16. Kalath, S., P. Tsipouras and F.H. Silver. Non-invasive assessment of aortic mechanical properties,Annals Biomedical Engineering, 14:513–524, 1986.

    CAS  Google Scholar 

  17. Krafka, J. Jr. Comparative study of the histo-physics of the aorta.Am. J. Physiology 125:1–14, 1939.

    Google Scholar 

  18. Lai, W.C. Experimental investigation and analysis of aortic mechanics. M.S. thesis in mechanical and aerospace engineering. Rutgers University, New Brunswick, NJ, 1985.

    Google Scholar 

  19. McKusick, V.A.Heritable Disorders of Connective Tissue. St. Louis: Mosby, 1982.

    Google Scholar 

  20. 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.

    CAS  PubMed  Google Scholar 

  21. 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.

    CAS  Google Scholar 

  22. Pyeritz, R.E. and L.S. Levin, Aortic root dilatation and valvular dysfunction in osteogenesis imperfecta. (abstr).Circulation 64, supp. IV-311, 1981.

    Google Scholar 

  23. Pyeritz, R.E. and V.A. McKusick. The Marfan syndrome: diagnosis and management.N. Engl. J. Med. 300:772–777, 1979.

    CAS  PubMed  Google Scholar 

  24. 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.

    Google Scholar 

  25. Sillence, D.O., A. Senn and D.M. Danks. Genetic heterogeneity in osteogenesis imperfecta,J. Med. Genetics 16:101–116, 1979.

    CAS  Google Scholar 

  26. Smith, R., M.J.O. Francis and G.R. Houghton.The Brittle Bone Syndrome. London: Butterworth, 1983.

    Google Scholar 

  27. Stein, D. and F.E. Kloster. Valvular heart disease in osteogenesis imperfecta.Am. Heart J. 94: 637–641, 1977.

    CAS  PubMed  Google Scholar 

  28. 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.

    Google Scholar 

  29. White, N.J., C.G. Winearls and R. Smith. Cardiovascular abnormalities in osteogenesis imperfecta.Am. Heart J. 106:1416–1420, 1983.

    Article  CAS  PubMed  Google Scholar 

  30. Wolinsky, H. and S. Glagov. Structural basis for the static mechanical properties of the aortic media.Circ. Res. 14:400–413, 1964.

    CAS  PubMed  Google Scholar 

  31. Wolinsky, H. and S. Glagov. A lamellar unit of aortic medial structure and function in mammals.Circ. Res. 20:99–111, 1967.

    CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Biomaterials Center, Department of Pediatrics and Biochemistry, UMDNJ-Rutgers Medical School, 08854, Piscataway, New Jersey

    Petros Tsipouras

  2. Biomaterials Center, Department of Pathology, UMDNJ-Rutgers Medical School, 08854, Piscataway, New Jersey

    Satish Kalath & Frederick H. Silver

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  1. Satish Kalath
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  2. Petros Tsipouras
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  3. Frederick H. Silver
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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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