Diagnostic power of aortic elastic properties in young patients with Marfan syndrome

J Thorac Cardiovasc Surg. 2005 Apr;129(4):730-9. doi: 10.1016/j.jtcvs.2004.07.019.


Background: In patients with Marfan syndrome, progressive aortic dilation implicates a still-unpredictable risk of life-threatening aortic dissection and rupture. We sought to quantify aortic wall dysfunction noninvasively, determine the diagnostic power of various aortic parameters, and establish a diagnostic model for the early detection of aortic abnormalities associated with Marfan syndrome.

Methods: In 19 patients with Marfan syndrome (age, 17.7 +/- 9.5 years) and 19 age- and sex-matched healthy control subjects, computerized ascending and abdominal aortic wall contour analysis with continuous determination of aortic diameters was performed out of transthoracic M-mode echocardiographic tracings. After simultaneous oscillometric blood pressure measurement, aortic elastic properties were determined automatically.

Results: The following ascending aortic elastic parameters showed statistically significant differences between the Marfan group and the control group: (1) decreased aortic distensibility ( P < .001), (2) increased wall stiffness index ( P < .01), (3) decreased systolic diameter increase ( P < .01), and (4) decreased maximum systolic area increase ( P < .001). The diagnostic power of all investigated parameters was tested by single logistic regression models. A multiple logistic regression model including solely aortic parameters yielded a sensitivity of 95% and a specificity of 100%.

Conclusions: In young patients with Marfan syndrome, a computerized image-analyzing technique revealed decreased aortic elastic properties expressed by parameters showing high diagnostic power. A multiple logistic regression model including merely aortic parameters can serve as useful predictor for Marfan syndrome.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aorta / diagnostic imaging
  • Aorta / physiopathology*
  • Aorta, Abdominal / diagnostic imaging
  • Aorta, Abdominal / physiopathology
  • Aortic Diseases / diagnosis*
  • Blood Pressure / physiology
  • Case-Control Studies
  • Child
  • Child, Preschool
  • Diastole
  • Echocardiography
  • Elasticity
  • Female
  • Humans
  • Image Processing, Computer-Assisted
  • Male
  • Marfan Syndrome / diagnostic imaging
  • Marfan Syndrome / genetics
  • Marfan Syndrome / physiopathology*
  • Mutation / genetics
  • Predictive Value of Tests
  • Systole
  • Vectorcardiography