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Increased aortic root stiffness associated with osteogenesis imperfecta.

S Kalath, P Tsipouras, F H Silver

    Annals of Biomedical Engineering
    |January 1, 1987
    PubMed
    Summary
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    Osteogenesis imperfecta (OI) patients show aortic root stiffness and altered elasticity, potentially due to collagen changes. This may indicate premature aging and reduced aortic pumping efficiency in individuals with OI.

    Area of Science:

    • Cardiovascular Medicine
    • Genetics
    • Biomaterials Science

    Background:

    • Osteogenesis imperfecta (OI) is a genetic disorder characterized by bone fragility.
    • Aortic complications, including dilatation and dissection, are known risks in OI patients.
    • The biomechanical properties of the aortic wall in OI are not fully understood.

    Purpose of the Study:

    • To evaluate aortic root dilatation and wall stiffness in patients with Osteogenesis Imperfecta (OI) types I and IV.
    • To assess the pressure-strain elastic moduli (Ep) and identify differences compared to controls.
    • To investigate the relationship between aortic wall stiffness and aortic pumping efficiency in OI.

    Main Methods:

    • M-mode echocardiography was performed on 31 patients with OI types I and IV.

    Related Experiment Videos

  • Aortic root dimensions and wall stiffness were analyzed.
  • Pressure-strain elastic moduli (Ep) were calculated and compared to age-matched controls.

    • Main Results:

    • Four out of 31 OI patients exhibited dilated aortic roots.
    • The majority of OI patients showed significantly different pressure-strain elastic moduli (Ep) compared to controls.
    • Nine OI patients displayed significantly stiffer aortic roots in the circumferential direction at high strain.
    • Increased circumferential stiffness correlated with decreased aortic pumping efficiency.

    Conclusions:

    • OI patients can present with aortic root dilatation and altered elastic properties.
    • Increased aortic wall stiffness, particularly in the circumferential direction, is observed in a subset of OI patients.
    • These findings suggest potential premature aging of the aortic wall due to collagen alterations and may impact cardiovascular function.