Diagnostic power of aortic elastic properties in young patients with Marfan syndrome.
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.
Baumgartner
Daniela
D
Department of Pediatric Cardiology, Innsbruck Medical University, Innsbruck, Austria. Daniela.baumgartner@aon.at
Baumgartner
Christian
C
Mátyás
Gabor
G
Steinmann
Beat
B
Löffler-Ragg
Judith
J
Schermer
Elisabeth
E
Schweigmann
Ulrich
U
Baldissera
Ivo
I
Frischhut
Bernhard
B
Hess
John
J
Hammerer
Ignaz
I
D000293:Adolescent; D000328:Adult; D001011:Aorta / Q000000981:diagnostic imaging / Q000503:physiopathology*; D001012:Aorta, Abdominal / Q000000981:diagnostic imaging / Q000503:physiopathology; D001018:Aortic Diseases / Q000175:diagnosis*; D001794:Blood Pressure / Q000502:physiology; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003971:Diastole; D004452:Echocardiography; D004548:Elasticity; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008297:Male; D008382:Marfan Syndrome / Q000000981:diagnostic imaging / Q000235:genetics / Q000503:physiopathology*; D009154:Mutation / Q000235:genetics; D011237:Predictive Value of Tests; D013599:Systole; D014672:Vectorcardiography